Eclampsia

Definition

Eclampsia, which is considered a complication of severe preeclampsia, is commonly defined as new onset of grand mal seizure activity and/or unexplained coma during pregnancy or postpartum in a woman with signs or symptoms of preeclampsia. It typically occurs during or after the 20th week of gestation or in the postpartum period. Nonetheless, eclampsia in the absence of hypertension with proteinuria has been demonstrated to occur in 38% of cases reported in the United Kingdom. Similarly, hypertension was absent in 16% of cases reviewed in the United States.

The clinical manifestations of maternal preeclampsia are hypertension and proteinuria with or without coexisting systemic abnormalities involving the kidneys, liver, or blood. There is also a fetal manifestation of preeclampsia involving fetal growth restriction, reduced amniotic fluid, and abnormal fetal oxygenation.HELLP syndrome is a severe form of preeclampsia and involves hemolytic anemia, elevated liver function tests (LFTs), and low platelet count.

Most cases of eclampsia present in the third trimester of pregnancy, with about 80% of eclamptic seizures occurring intrapartum or within the first 48 hours following delivery. Rare cases have been reported before 20 weeks' gestation or as late as 23 days’ postpartum. Other than early detection of preeclampsia, no reliable test or symptom complex predicts the development of eclampsia. In developed countries, many reported cases have been classified as unpreventable.

Course of eclamptic seizures

Eclampsia manifests as 1 seizure or more, with each seizure generally lasting 60-75 seconds. The patient’s face initially may become distorted, with protrusion of the eyes, and foaming at the mouth may occur. Respiration ceases for the duration of the seizure.

Eclamptic seizures may be divided into 2 phases. Phase 1 lasts 15-20 seconds and begins with facial twitching. The body becomes rigid, leading to generalized muscular contractions.

Phase 2 lasts about 60 seconds. It starts in the jaw, moves to the muscles of the face and eyelids, and then spreads throughout the body. The muscles begin alternating between contracting and relaxing in rapid sequence.

A coma or period of unconsciousness, lasting for a variable period, follows phase 2. After the coma phase, the patient may regain some consciousness, and she may become combative and very agitated. However, the patient will have no recollection of the seizure.

A period of hyperventilation occurs after the tonic-clonic seizure. This compensates for the respiratory and lactic acidosis that develops during the apneic phase.

Seizure-induced complications can include tongue biting, head trauma, broken bones, and aspiration.

Etiologic and Risk Factors for Preeclampsia/Eclampsia

Genetic predisposition, immunology, endocrinology, nutrition, abnormal trophoblastic invasion, coagulation abnormalities, vascular endothelial damage, cardiovascular maladaptation, dietary deficiencies or excess, and infection have been proposed as etiologic factors for preeclampsia/eclampsia. Imbalanced prostanoid production and increased plasma antiphospholipids have also been implicated in eclampsia.
Risk factors for eclampsia

The following are considered risk factors for eclampsia:




Nulliparity



Family history of preeclampsia, previous preeclampsia and eclampsia



Poor outcome of previous pregnancy, including intrauterine growth retardation, abruptio placentae, or fetal death



Multifetal gestations, hydatid mole, fetal hydrops, primigravida



Teen pregnancy



Primigravida



Patient older than 35 years



Lower socioeconomic status

The following preexisting medical conditions are also considered risk factors :




Obesity



Chronic hypertension



Renal disease



Thrombophilias-antiphospholipid antibody syndrome



Protein C deficiency and protein S deficiency



Antithrombin deficiency



Vascular and connective tissue disorders



Gestational diabetes



Systemic lupus erythematosus

Multiorgan System Effects

Preeclampsia/eclampsia produces multiple systemic derangements that can involve a diversity of organ systems including hematologic, hepatic, renal, and cardiovascular systems as well as the central nervous system. The severity of these derangements often correlates with maternal medical (eg, preexisting renal or vascular pathology) or obstetric factors (eg, multifetal gestations or molar pregnancy).
Cardiovascular concerns

Eclampsia is associated with cardiovascular derangements such as generalized vasospasm, increased peripheral vascular resistance, increased left ventricular stroke work index, decreased central venous pressure, and decreased pulmonary wedge pressure.
Hematologic concerns

Hematologic problems associated with eclampsia can include decreased plasma volume, increased blood viscosity, hemoconcentration, and coagulopathy.
Renal concerns

Eclampsia-associated renal abnormalities can include decreases in glomerular filtration rate, renal plasma flow, and uric acid clearance.
Hepatic concerns

Hepatic derangements associated with eclampsia can include periportal necrosis, hepatocellular damage, and subcapsular hematoma.
Central nervous system concerns

Eclampsia can result in central nervous system (CNS) abnormalities such as cerebral overperfusion due to loss of autoregulation, cerebral edema, and cerebral hemorrhage.

Pathophysiology of Eclampsia

 
Inhibition of uterovascular development

Many uterovascular changes occur when a woman is pregnant. It is believed that these changes are due to the interaction between fetal and maternal allografts and result in systemic and local vascular changes. It has been shown that in patients with eclampsia, the development of uteroplacental arteries is hindered.
Hindrance of cerebral blood flow regulation

It is believed that in eclampsia there is abnormal cerebral blood flow in the setting of extreme hypertension. The regulation of cerebral perfusion is inhibited, vessels become dilated with increased permeability, and cerebral edema occurs, resulting in ischemia and encephalopathy. In extreme hypertension, normal compensatory vasoconstriction may become defective. Several autopsy findings support this model and consistently reveal swelling and fibrinoid necrosis of vessel walls.
Endothelial dysfunction

Factors associated with endothelial dysfunction have been shown to be increased in the systemic circulation of women suffering from eclampsia. These include the following :




Cellular fibronectin



Von Willebrand factor



Cell adhesion molecules (ie, P-selectin, vascular endothelial adhesion molecule-1 [VCAM-1]



Intercellular adhesion molecule-1 [ICAM-1])



Cytokines (ie, interleukin-6 [IL-6])



Tumor necrosis factor-α [TNF-α]

In addition, it is believed that antiangiogenic factors, such as placental protein fms-like tyrosine kinase 1 (sFlt-1) and activin A, antagonize vascular endothelial growth factor (VEGF). Elevated levels of these proteins cause a reduction of VEGF and induce systemic and local endothelial cell dysfunction.

Leakage of proteins from the circulation and generalized edema are sequelae of the endothelial dysfunction and thus a defining factor associated with preeclampsia and eclampsia.
Oxidative stress

Evidence indicates that leptin molecules increase in the circulation of women with eclampsia, inducing oxidative stress, another factor in eclampsia, on cells. (The leptin increase also results in platelet aggregation, most likely contributing to the coagulopathy associated with eclampsia.)

Oxidative stress has been found to stimulate the production and secretion of the antiangiogenic factor activin A from placental and endothelial cells, Studies in pregnant mouse models have proposed that there is a dysregulation in the reactive oxygen species (ROS) signaling pathway.

Studies also suggest that increased systemic leukocyte activity plays a role in the mediation of oxidative stress, inflammation, and endothelial cell dysfunction. Histochemistry studies indicate that there is predominantly an increase in neutrophil infiltration of vasculature in patients with eclampsia.

Evaluation

Eclampsia always should be considered in a pregnant patient with a seizure episode. A pregnant patient who has been involved in an unexplained trauma (such as a single-vehicle auto accident) and has exhibited seizure activity should be evaluated for eclampsia. Eclampsia can occur during the antepartum, intrapartum, and postpartum periods. Ninety percent of eclampsia cases occur after 28 weeks' gestation.[2]

Preeclampsia can quickly develop into eclampsia. The natural progression of the disease is from symptomatic severe preeclampsia (differentiated from preeclampsia by specific vital signs, symptoms, and laboratory abnormalities) to seizures.

Features of eclampsia include the following:




Seizure or postictal state (100%)



Headache (80%), usually frontal



Generalized edema (50%)



Vision disturbance (40%), such as blurred vision and photophobia



Right upper quadrant abdominal pain with nausea (20%)



Amnesia and other mental status changes

The incidences of signs or symptoms before seizure include the following:




Headache (83%)



Hyperactive reflexes (80%)



Marked proteinuria (52%)



Generalized edema (49%)



Visual disturbances (44%)



Right upper quadrant pain or epigastric pain (19%)

The absence of signs or symptoms before seizure include the following:




Lack of edema (39%)



Absence of proteinuria (21%)



Normal reflexes (20%)

The relation of seizure to delivery is as follows:




Before delivery (>70%)



Before labor (antepartum) (25%)



During labor (intrapartum) (50%)



After delivery (postpartum) (25%)

Although patients with severe preeclampsia are at greater risk for seizures, 25% of patients have symptoms consistent with mild preeclampsia before the seizures.

A study by Cooray et al found that the most common symptoms that immediately precede eclamptic seizures are neurologic symptoms (ie, headache, with or without visual disturbance), regardless of degree of hypertension. This suggests that closely monitoring patients with these symptoms may provide an early warning for eclampsia.
Physical findings

Most patients with eclampsia present with hypertension and seizures, along with some combination of proteinuria and edema. Findings at physical examination may include the following:




Sustained systolic BP greater than 160 mm Hg or diastolic BP greater than 110 mm Hg



Tachycardia



Tachypnea



Rales



Mental status changes



Hyperreflexia



Clonus



Papilledema



Oliguria or anuria



Localizing neurologic deficits



Right upper quadrant or epigastric abdominal tenderness



Generalized edema



Small fundal height for the estimated gestational age



Apprehension

Cervical examination of the patient with eclampsia should not be overlooked, because the delivery mode may largely depend upon the patient’s cervical status.

Differential Diagnosis

Adrenal Insufficiency and Adrenal Crisis

Cerebellar Hemorrhage

Cerebral Aneurysms

Cerebral Venous Thrombosis

Encephalopathy, Hypertensive

Encephalitis

Gestational Trophoblastic Neoplasia

Head Trauma

Hyperaldosteronism, Primary

Hypertensive Emergencies

Hypoglycemia

Meningitis

Neoplasms, Brain

Pregnancy, Preeclampsia

Seizures and Epilepsy: Overview and Classification

Shock, Septic

Stroke, Hemorrhagic

Stroke, Ischemic

Subarachnoid Hemorrhage

Systemic Lupus Erythematosus

Thrombotic Thrombocytopenic Purpura

Withdrawal Syndromes

Angiomas

Cerebral Vasculitis

Drug Overdose

Metabolic Disorders

Undiagnosed Brain Tumors

Diagnostic Overview

Seizures in the first trimester or well into the postpartum period probably are due to CNS pathology and warrant full evaluation, including computed tomography (CT) scanning of the head, lumbar puncture (if clinical evidence of meningitis or concern for hemorrhage exists), determination of electrolyte levels, and urine or serum toxicologic screening. Do not overlook other neurologic causes of seizure, particularly if the seizure occurs more than 24 hours after delivery. In addition, rule out hypoglycemia as cause of seizure or result of seizure, and rule out hyperglycemia as cause of mental status changes.

When preeclampsia occurs in the early second trimester (ie, 14-20 weeks' gestation), the diagnosis of hydatiform mole or choriocarcinoma should be considered.

Ruling out eclampsia in an obstetric patient who has been involved in an unexplained trauma is important. Immediately consult an obstetrician/gynecologist when the diagnosis of eclampsia is being considered.

No single laboratory test or set of laboratory determinations is useful in predicting maternal or neonatal outcome in women with eclampsia. Imaging studies may be indicated after initial stabilization, especially if there is doubt about the diagnosis or possible injuries secondary to seizure activity.

Urinalysis and Uric Acid levels

Proteinuria is typically one of the presenting symptoms in patients with eclampsia. A timed collection has been the criterion standard for urinalysis to detect proteinuria (>300 mg/24 h or >1 g/L). Protein per unit time measured over 24 hours has been used traditionally; however, 12-hour collections have proved to be as accurate.

Although investigational, Baweja et al suggest that when measuring intact urinary albumin levels using high-performance liquid chromatography in an early and uncomplicated pregnancy, spot urinary albumin:creatinine ratio (ACR) values are higher. If measured early in the second trimester, an ACR of 35.5 mg/mmol or higher may predict preeclampsia before symptoms arise.

Uric acid levels may be mildly increased.

Hematologic Studies

A complete blood cell (CBC) count may reveal the following:




Anemia due to microangiopathic hemolysis, hemoconcentration due to third spacing, or physiologic hemodilution of pregnancy



Peripheral smear (schistocytes, burr cells, echinocytes)



Increased bilirubin (>1.2 mg/dL)



Thrombocytopenia (< 100,000) due to hemolysis and low platelet count associated with HELLP syndrome (seen in 20-25% of patients with eclampsia)



Low serum haptoglobin levels



Elevated lactate dehydrogenase (LDH) levels (threshold of 180–600 U/L)

The coagulation profile may reveal normal prothrombin (PT) and activated partial thromboplastin (aPTT) times, fibrin split products, and fibrinogen levels. Rule out associated disseminated intravascular coagulation (DIC).

Serum Creatinine level

The serum creatinine level is elevated in eclampsia because of a decreased intravascular volume and a reduced glomerular filtration rate (GFR). Creatinine clearance (CrCl) may be less than 90 mL/min/1.73 m2.

Liver Function Tests

Liver function test results may reveal the following (20-25% of patients with eclampsia):




Aspartate aminotransferase (SGOT) level higher than 72 IU/L



Total bilirubin levels higher than 1.2 mg/dL



LDH level higher than 600 IU/L



Elevated levels due to hepatocellular injury and HELLP syndrome

CT Scanning

CT scanning of the head, with or without contrast, can exclude cerebral venous thrombosis, intracranial hemorrhage, and central nervous system lesions, all of which can occur in pregnancy and present with seizures.

Consider CT scanning in patients who have been involved in trauma, are refractory to magnesium sulfate therapy, or have atypical presentations (eg, seizures >24 h after delivery).

Although obtaining a CT scan in eclampsia is not routine, abnormalities have been observed in up to 50% of women imaged.

Characteristic CT scan findings include cortical hypodense areas, particularly in the occipital lobes, and diffuse cerebral edema, which is believed to correspond to petechial hemorrhages and diffuse edema noted in postmortem studies.

CT scan findings may include the following:




Cerebral edema



Diffuse white matter low-density areas



Patchy area of low density



Occipital white matter edema



Loss of normal cortical sulci



Reduced ventricular size



Cerebral hemorrhage



Intraventricular hemorrhage



Parenchymal hemorrhage (high density)



Cerebral infarction



Low attenuation areas



Basal ganglia infarctions

Magnetic Resonance Imaging of the Head

Abnormal magnetic resonance imaging (MRI) findings of the head have been reported in up to 90% of women imaged. These include an increased signal at the gray-white matter junction on T2-weighted images, as well as cortical edema and hemorrhage.

Transabdominal Ultrasonography

Transabdominal ultrasonography is used to estimate gestational age. This may also be used to rule out abruptio placentae, which can complicate eclampsia.

Electroencephalography and CSF Studies

Electroencephalograms and cerebrospinal fluid studies are rarely useful in management; however, they may be indicated if epilepsy or meningitis is considered in the diagnosis.

Medical Therapy

Eclamptic convulsions are life-threatening emergencies and require the proper treatment to decrease maternal morbidity and mortality. Delivery is the only definitive treatment for eclampsia.

The patient should be advised and educated on the course of the disease and any residual problems. She should also be educated on the importance of adequate prenatal care in subsequent pregnancies.

Several organizations have developed screening, treatment, and prevention guidelines for preeclampsia and eclampsia.
Consultations and/or Transfer

An experienced obstetrician or maternal-fetal medicine specialist may be consulted. Patients with eclampsia require immediate obstetric consultation and admission to an intensive care setting for supportive care and treatment until delivery of the neonate. In the event of premature delivery or fetal compromise, a pediatrician or neonatologist should be consulted.

When initially evaluating a patient with eclampsia, become familiar with the level of care that the medical center can offer the patient, as eclampsia clearly poses a risk of considerable maternal and neonatal morbidity and mortality. Patients with eclampsia may benefit from management at a tertiary care center, a high-risk obstetric facility that provides neonatal and maternal intensive care.

Supportive care

Emergency medical services personnel should (1) secure an intravenous (IV) line with a large-bore catheter, (2) initiate cardiac monitoring and administer oxygen, and (3) transport the patient in the left lateral decubitus position. Supportive care for eclamptic convulsions includes the following:




Close monitoring (invasive, if clinically indicated)



Airway support



Adequate oxygenation



Anticonvulsant therapy



Blood pressure (BP) control

Place the patient in the left lateral position. This positioning decreases the risk of aspiration and will help to improve uterine blood flow by relieving obstruction of the vena cava by the gravid uterus. Protect the patient against injury during the seizure by padding and raising guardrails, using a padded tongue blade between the teeth, and suctioning the oral secretions as needed.

After the seizure has ended, a 16- to 18-gauge IV line should be established for drawing specimens and administering fluids and medications. (Fluid management is critical in patients with eclampsia.) IV fluids should be limited to isotonic solutions to replace urine output plus about 700 mL/d to replace insensible losses.
Pharmacologic considerations for convulsions and hypertension

Pharmacotherapy goals are to reduce morbidity, prevent complications, and correct eclampsia. The drug of choice to treat and prevent eclampsia is magnesium sulfate. Familiarity with second-line medications phenytoin and diazepam/lorazepam is required for cases in which magnesium sulfate may be contraindicated (eg, myasthenia gravis) or ineffective. Control of hypertension is essential to prevent further morbidity or possible mortality. The most commonly used antihypertensive agents are hydralazine, labetalol, and nifedipine.

IV magnesium sulfate is the initial drug administered to terminate seizures and lower BP. Seizures usually terminate after the loading dose of magnesium. A loading dose of 6 g (15-20 min) and a maintenance dose of 2 g per hour as a continuous IV solution should be administered. Once the seizures terminate, 85% of patients note improved BP control. Note: Magnesium toxicity can cause coma, and, if mental status changes with these infusion rates, this should be considered.

Benzodiazepine or phenytoin can be used for seizures that are not responsive to magnesium sulfate. Avoid the use of multiple agents to abate eclamptic seizures, unless necessary.

Severe hypertension must be addressed after magnesium infusions. Hydralazine or labetalol can then be administered IV for BP control. The goal is to maintain systolic BP between 140 and 160 mm Hg and diastolic BP between 90 and 110 mm Hg. Recommended IV bolus of hydralazine (5-10 mg) or labetalol (20-40 mg q15min prn). Other potent antihypertensive medications, such as sodium nitroprusside or nitroglycerin, can be used but are rarely required.

Diuretics are used only in the setting of pulmonary edema.

Care must be taken not to decrease the BP too drastically; an excessive decrease can cause inadequate uteroplacental perfusion and fetal distress.

A dose of antenatal steroids may be administered in anticipation of emergent delivery when gestational age is less than 32 weeks. Betamethasone (12 mg IM q24h × 2 doses) or dexamethasone (6 mg IM q12h × 4 doses) is recommended.

About 10% of women with eclampsia will have an additional seizure after receiving magnesium sulfate. Another 2 g bolus of magnesium may be given in these cases. For the rare patient who continues to have seizure activity while receiving adequate magnesium therapy, seizures may be treated with sodium amobarbital, 250 mg IV over 3-5 minutes. More commonly, lorazepam (Ativan) 4 mg IV over 2-5 minutes (may repeat in 5-15 min to maximum of 8 mg in 12 hours or diazepam (Valium), 5-10 mg IV slowly (may be repeated every 15 min up to 30 mg) both as per protocol for status epilepticus.

BP should be assessed with the goal of maintaining the diastolic BP at less than 110 mm Hg with administration of antihypertensive medications as needed (eg, hydralazine, labetalol).

Keep nothing by mouth (including medications) until the patient is medically stabilized or delivered, because she is at risk for aspiration when postictal and may have recurrent seizures.
Maternal monitoring

Depending on the clinical course, regularly check the patient’s neurologic status for signs of increased intracranial pressure or bleeding (eg, funduscopic examination, cranial nerves)

Monitor fluid intake and urine output, maternal respiratory rate, and oxygenation, as indicated, and continuously monitor fetal status. Pulmonary arterial pressure monitoring is rarely indicated but may be helpful in patients who have evidence of pulmonary edema or oliguria/anuria.

Once the seizure is controlled and the patient has regained consciousness, the patient’s general medical condition should be assessed to identify any other causes for seizures.

Induction of labor may be initiated when the patient is stable.
Fetal monitoring

Fetal heart rate and uterine contractions should be continuously monitored. Fetal bradycardia is common following the eclamptic seizure and has been reported to last from 30 seconds to 9 minutes. The interval from the onset of the seizure to the fall in the fetal heart rate is typically 5 minutes or less. Transitory fetal tachycardia may occur following the bradycardia.

After the initial bradycardia, during the recovery phase, the fetal heart rate tracing may reveal a loss of short- and long-term variability and the presence of late decelerations. These abnormalities are most likely due to the decrease in uterine blood flow caused by the intense vasospasm and uterine hyperactivity during the convulsion. If the fetal heart tracing does not improve following a seizure, further evaluation should be undertaken. Growth-restricted and preterm fetuses may take longer to recover following a seizure. Placental abruption may be present if uterine hyperactivity remains and fetal bradycardia persists.
Delivery (antepartum or intrapartum eclampsia)

Delivery is the treatment for eclampsia after the patient has been stabilized. No attempt should be made to deliver the infant either vaginally or by cesarean delivery until the acute phase of the seizure or coma has passed. The mode of delivery should be based on obstetric indications but should be chosen with an awareness that vaginal delivery is preferable from a maternal standpoint.

Adequate maternal pain relief for labor and delivery is vital and may be provided with either systemic opioids or epidural anesthesia.

In the absence of fetal malpresentation or fetal distress, oxytocin or prostaglandins may be initiated to induce labor.

Cesarean delivery may be considered in patients with an unfavorable cervix and a gestational age of 30 weeks or less, as induction under these circumstances may result in a prolonged intrapartum course and is frequently unsuccessful in avoiding cesarean section, given the high rate of intrapartum complications. When emergent cesarean delivery is indicated, substantiating the absence of coagulopathy before the procedure is important. (See Surgical Therapy.)

Intrapartum complications include the following:




Fetal growth retardation (30%)



Nonreassuring fetal heart rate patterns (30%)



Placental abruption (23%)

Irrespective of gestational age, a prolonged induction with clinically significant worsening of maternal cardiovascular, hematologic, renal, hepatic, and/or neural status is generally an indication for cesarean delivery when the anticipated delivery time is remote.

Surgical Therapy

Cesarean delivery may be necessary for obstetric indications or a deteriorating maternal condition. The patient should be stabilized with respect to seizures, oxygenation, and hemodynamic status before the initiation of cesarean delivery. BP should be controlled and coagulopathies monitored or corrected.
Anesthesia

An anesthesiology consultation may be obtained. Early evaluation is recommended to assist with cardiopulmonary stabilization and to prepare for a possible operative delivery or endotracheal intubation.

For nonemergency cesarean delivery, epidural or combined techniques of regional anesthesia are preferred. Regional anesthesia is contraindicated in the presence of coagulopathy or severe thrombocytopenia (< 50,000 platelets/µL). General anesthesia in women with eclampsia increases the risk of aspiration, and airway edema may make intubation difficult. It also can produce significant increases in systemic and cerebral pressures during intubation and extubation.

The use of spinal anesthesia requires caution because of the possibility of total sympathetic blockade, resulting in maternal hypotension and uteroplacental insufficiency.

Postpartum Outpatient Monitoring

Follow up 1-2 weeks after delivery to evaluate the patient for BP control and any residual deficits from the eclamptic seizure. Patients with persistent hypertension past 8 weeks' puerperium or neurologic changes may need medical referral.

Al-Safi et al suggest that the first week after discharge is the most critical period for the development of postpartum eclampsia. Discussing the risks and educating patients about the possibility of delayed postpartum preeclampsia is important, regardless of whether they develop hypertensive disease prior to discharge.

Prevention of Preeclampsia/Eclampsia

Preventing the development of preeclampsia in high-risk patients could theoretically decrease the risk of eclampsia and its complications later in pregnancy. Aspirin blocks platelet aggregation and vasospasm in preeclampsia, and it may be effective in preventing preeclampsia. Studies have shown that low-dose aspirin in women at high risk for preeclampsia can contribute to a decreased risk of preeclampsia, a reduction in preterm delivery rates, and a reduction in fetal death rates, without increasing the risk of placental abruption. An obstetrician should directly supervise aspirin therapy in high-risk patients.

If the patient has preexisting hypertension, she should have good control before conception and throughout her pregnancy. Her case should be followed for recognition and treatment of preeclampsia.

A study by Vadillo-Ortega et al suggests that in a high-risk population (eg, previous pregnancy complicated by preeclampsia, preeclampsia in a first-degree relative), supplementation during pregnancy with a special food (eg, bars) containing L-arginine and antioxidant vitamins may reduce the risk of preeclampsia. Notably, the beneficial effect was greatest when supplementation was started prior to 24 weeks' gestation. Antioxidant vitamin supplementation alone did not protect against preeclampsia. More studies performed on low-risk populations are needed

Complications of Eclampsia

As many as 56% of patients with eclampsia may have transient deficits, including cortical blindness. However, studies have failed to demonstrate evidence of persisting neurologic deficits after uncomplicated eclamptic seizures during the follow-up period. Studies suggest that there is an increased risk for cerebrovascular accidents (CVAs) and coronary artery disease (CAD) in eclamptic mothers later in life.




Other potential complications of eclampsia include the following:



Permanent neurologic damage from recurrent seizures or intracranial bleeding



Renal insufficiency and acute renal failure



Fetal changes – IUGR, abruptio placentae, oligohydramnios



Hepatic damage and rarely hepatic rupture



Hematologic compromise and DIC



Increased risk of recurrent preeclampsia/eclampsia with subsequent pregnancy



Maternal or fetal death

Outcome

Although the incidence of eclampsia has declined in recent years, mainly due to the improvement of healthcare, serious adverse outcomes still exist.Five percent of patients with hypertension develop severe preeclampsia, and about 25% of women with eclampsia have hypertension in subsequent pregnancies. About 2% of women with eclampsia develop eclampsia with future pregnancies.

Multiparous women with eclampsia have a higher risk for the development of essential hypertension; they also have a higher mortality rate in subsequent pregnancies than do primiparous women
Maternal morbidity

Maternal complications from eclampsia include the following:




Permanent CNS damage from recurrent seizures or intracranial bleeds



Disseminated intravascular coagulopathy



Renal insufficiency



Pulmonary edema



Cardiopulmonary arrest

The most significant maternal complication of eclampsia is permanent CNS damage secondary to recurrent seizures or intracranial bleeding. The maternal mortality rate is 8-36% in these cases.
Maternal mortality

Eclampsia and preeclampsia account for approximately 63,000 maternal deaths annually worldwide. In developed countries, the maternal death rate is reportedly 0-1.8%. The perinatal mortality rate from eclampsia in the United States and Great Britain ranges from 5.6% to 11.8%. The maternal mortality rate is as high as 14% in developing countries.

A study from the US Centers for Disease Control and Prevention (CDC) found an overall preeclampsia/eclampsia case-fatality rate of 6.4 per 10,000 cases at delivery. The study also found a particularly high risk of maternal death at 20-28 weeks’ gestation.

Black woman have twice the risk that white women have for mortality associated with preeclampsia/eclampsia. This is most likely due to inadequate access to prenatal care among black women, as well as to increased incidences in black women of genetic diseases associated with circulating antiphospholipids. It has been proven that patients with elevated antiphospholipid plasma levels have a higher incidence of preeclampsia and eclampsia. However, whether this is due to the antiphospholipids themselves or to some other underlying process is not clear.

A majority of women who suffer eclampsia-associated death have concurrent HELLP syndrome.

A report of an international study demonstrated that serious complications among patients with eclampsia (including maternal mortality) may be predicted by the use of a model that incorporates gestational age, chest pain or dyspnea, oxygen saturation, platelet count, and creatinine and aspartate transaminase concentrations. Although clinical use of the model awaits future validation, the identification of the predictive variables may aid in management decisions.
Fetal/neonatal mortality

The fetal mortality rate varies from 13-30% due to premature delivery and its complications. Placental infarcts, abruptio placentae, intrauterine growth retardation, and fetal hypoxia also contribute to fetal demise.

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Proteus syndrome

Proteus syndrome, also known as Wiedemann's syndrome (named after the German paediatrician Hans-Rudolf Wiedemann), is a congenital disorder that causes skin overgrowth and atypical bone development, often accompanied by tumors over half the body.

Proteus syndrome is highly variable, and is named after the Greek sea-god Proteus, who could change his shape.

The condition appears to have been first described in the American medical literature by Drs. Samia Temtamy and John Rogers in 1976 Dr.Michael Cohen described it in 1979, only a few more than 200 cases have been confirmed worldwide, with estimates that about 120 people are currently alive with the condition. As attenuated forms of the disease may exist, there could be many people with Proteus syndrome who remain undiagnosed. Those most readily diagnosed are also the most severely disfigured.

Presentation

Proteus syndrome causes an overgrowth of skin, bones, muscles, fatty tissues, and blood and lymphatic vessels.

Proteus syndrome is a progressive condition wherein children are usually born without any obvious deformities. Tumors and skin and bone growths appear as they age. The severity and locations of these various asymmetrical growths vary greatly but typically the skull, one or more limbs, and soles of the feet will be affected. There is a risk of premature death in affected individuals due to deep vein thrombosis and pulmonary embolism caused by the vessel malformations that are associated with this disorder. Because of carrying excess weight and enlarged limbs, arthritis and muscle pain may also be symptoms — as is the case for Mandy Sellars, a woman living with a form of Proteus syndrome. Further risks may occur due to the mass of extra tissue — Joseph Merrick, the most famous sufferer of Proteus syndrome, himself died when the weight of his head dislocated his neck while he was sleeping.

The disorder itself does not uniformly cause learning impairments: the distribution of intelligence deficits among sufferers of Proteus syndrome appears higher than that of the general population, although this is difficult to determine with statistical significance. In addition, the presence of visible deformity may have a negative effect on the social experiences of the sufferer, causing cognitive and social deficits.

Afflicted individuals are at increased risk for developing certain tumors including unilateral ovarian cystadenomas, testicular tumors, meningiomas, and monomorphic adenomas of the parotid gland.

Genetics

In 2011 researchers determined the cause of Proteus syndrome. In 26 of 29 patients who met strict clinical criteria for the disorder Lindhurst et al. identified an activating mutation in AKT1 kinase in a mosaic state gene. This mutation was not present in more than 1,000 persons who were unaffected by this disorder. Previous research had suggested the condition linked to PTEN on chromosome 10, while other research pointed to chromosome 16. Prior to the determination of the cause of the disease in 2011, other researchers expressed doubt regarding the involvement of PTEN or GPC3.

Treatment

A team of doctors in Australia have trial tested the drug Rapamycin in the treatment of a patient said to have Proteus syndrome and have found it to be an effective remedy. However, the diagnosis of Proteus syndrome in this patient has been questioned by others.

Classification

Many sources classify Proteus syndrome to be a type of nevus syndrome. The lesions appear to be distributed in a mosaic manner. It has been confirmed that the disorder is an example of genetic mosaicism.

Notable cases

This extremely rare condition has been proposed as the condition that affected Joseph Merrick, the 'Elephant Man'.

Mandy Sellars has been diagnosed by some doctors as suffering from this condition. Her legs and feet have grown at a disproportionate rate since birth.

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Rare and Unusual Psychiatric Syndromes

Rare Psychiatric Syndromes: Introduction

Psychiatric disorders affect many lives. When assessing patients with a mental disorder, clinicians draw on a set of diagnoses that are common and that they frequently consider in the differential diagnosis when encountering a new patient. Likewise, when devising a treatment plan, clinicians select from common management options tailored to these conditions.

However, there are a number of rare psychiatric syndromes that are often taught in medical school or are asked about in standardized tests, but that clinicians rarely encounter. Some syndromes are neurologic, some are psychiatric in origin, which is relevant for the management. Some syndromes are merely a specific form of more general psychopathology and are managed similarly as the underlying disorder. The fact that these unusual mental phenomena carry the names of cities, historical events, and people indicates that the specific phenomenology is considered noteworthy and unusual, being a testimony of how complex the human brain is.

This 2-part series about rare psychiatric conditions and culture-bound syndromes is intended to refresh clinicians' memories of clinical syndromes they encounter rarely, but that they should be able to recognize, diagnose, and manage appropriately. The included syndromes span delusional misinterpretations, trauma-induced mental phenomena that range from anxiety to dissociative phenomena and psychosis; reactions to stressful, unexpected, overwhelming or experiences; and distinct phenomena following neurologic damage.

Capgras syndrome, named after a French psychiatrist who described the illusion of doubles, is a delusion of misidentification. It is characterized by a person's delusional belief that an acquaintance, usually a spouse or other close family member, has been replaced by an identical looking impostor or several doubles.

Capgras syndrome occurs most often in patients with schizophrenia, although it has also been reported in patients with dementia or epilepsy and after traumatic brain injury. The management does not differ from the treatment of the underlying disorders, and usually involves antipsychotic medications, although in dementia patients, cognitive enhancing treatments and nonpharmacologic strategies that diminish disorganization should be used.

Fregoli syndrome, named after the Italian actor Leopoldo Fregoli who was renowned for his ability to make quick changes of appearance during his stage act, is the inverse of Capgras syndrome. It is also a delusion of misidentification and is characterized by a person's delusional belief that persecutors or familiar people can assume the guise of strangers, in that different people are in fact a single person who changes his or her appearance or who appears in disguise.

As in Capgras syndrome, Fregoli syndrome occurs most often in patients with schizophrenia, although it has also been reported in patients with dementia or epilepsy and after traumatic brain injury. Again, the management does not differ from the treatment of the underlying disorders, and usually involves antipsychotic medications, although in cases of dementia cognitive-enhancing treatments and nonpharmacologic approaches that diminish disorganization should be used.

Cotard Delusion

Cotard syndrome is a specific nihilistic delusion named after Jules Cotard, a French neurologist, who first described the condition, which he called "le délire de négation" (negation delirium), in 1880. The affected person holds the delusional belief that he or she is already dead, does not exist, is putrefying or has lost his or her blood or internal organs.

It is most frequently observed in patients with psychotic depression or schizophrenias and is managed by focusing on the treatment of the underlying disorder.

Reduplicative Paramnesia

Reduplicative paramnesia consists of the delusional belief that a place or location has been duplicated, in that it exists in 2 or more places simultaneously, or that it has been 'relocated' to another site. It is basically the delusion of doubles of the Capgras syndrome, only that is does not refer to a person but to a place.

"Reduplicative paramnesia" was first used by neurologist Arnold Pick in 1903 to describe a condition in a patient with suspected Alzheimer disease.

Alien Hand Syndrome

Alien hand syndrome is the misattribution and belief that one's hand does not belong to oneself, but that it has its own life. The afflicted person has normal sensation in the hand and leg, but believes that the hand, while still being a part of their body, is acting autonomously, having 'a will of its own.' In effect, afflicted people lost the 'sense of agency' associated with the purposeful movement of the limb while retaining a sense of 'ownership' of the limb.

Sufferers of alien hand syndrome will often personify the alien limb, believing it to be "possessed" by some spirit or an entity that they may name or identify. There is a clear distinction between the behaviors of the 2 hands in which the affected hand is viewed as "wayward" or "disobedient," while the unaffected hand is under normal volitional control. At times, particularly in patients who have sustained damage to the corpus callosum that connects the 2 cerebral hemispheres (see also split-brain), the hands appear to be acting in opposition to each other, which has been termed "intermanual conflict" or "ideomotor apraxia."

Alien hand syndrome is usually caused by stroke or other brain damage, particularly in the areas of the corpus callosum, or frontal or parietal lobes

Alice in Wonderland Syndrome or Todd Syndrome

Alice in Wonderland syndrome (AIW) or Todd syndrome is a neurologic condition in which a patient's sense of body image, space, and/or time are distorted. Sufferers may experience micropsia or Lilliputian hallucinations, macropsia, or size distortion of other sensory modalities, which includes also an altered sense of velocity, produced by the distorted sense of size, perspective, and time.

AIWS is a result of change in perception as opposed to the eyes themselves malfunctioning. AIWS affects the sufferer's sense of vision, sensation, touch, and hearing, as well as one's own body image and sense of time. The most prominent and often most disturbing symptom is that of altered body image: the sufferer will find that he is confused as to the size and shape of parts of (or all of) his body. These symptoms can be alarming, causing fear, even panic. Distortions can recur several times a day and may take some time to abate.

It is often associated with migraines, brain tumors, or the use of psychoactive drugs and can also present as the initial sign of the Epstein-Barr virus or during high fever. Rest is the best treatment. If associated with migraines, treatment is the same as that for other migraine prophylaxis, including anticonvulsants, antidepressants, beta blockers, and calcium channel blockers, together with strict adherence to the migraine diet.

Jerusalem Syndrome

Jerusalem syndrome is characterized by mental phenomena that involve the presence of either religiously themed obsessive ideas, delusions, or other psychosis-like experiences that are triggered by, or lead to, a visit to the city of Jerusalem. It is not restricted to any religion or denomination.

The condition seems to emerge when people who had a history of mental illness, or who were unwell before coming to the city, visit Jerusalem. It seems to consist of usually transient psychotic delusions, which tend to dissipate within a few weeks after being removed from the area.

Symptom-based approaches or careful withdrawal of antipsychotic drugs after cessation of the psychosis in those people in whom antipsychotic treatment was needed should be attempted. However, Jerusalem syndrome needs to be distinguished from a first or recurrent psychotic disorder that requires long-term antipsychotic treatment.

Paris Syndrome

Paris syndrome is a strange condition exclusive to Japanese nationals who experience a mental breakdown while visiting the famous French capital, but it has also been observed in Japanese tourists visiting France or Spain in general. Paris syndrome appears to be a severe form of culture shock that can express itself in many different forms, including physical and emotional symptoms of anxiety, derealization, depersonalization, as well as acute delusional states, persecutory ideas, and hallucinations.

Of an estimated 6 million Japanese tourists who visit the city every year, approximately 1-2 dozen suffer this illness. Usually, people with Paris syndrome do not have a psychiatric history. Hypotheses why Japanese people are affected include their apparent suggestibility regarding an idealized image of Paris, but the confrontation with very different cultural habits, a strong language barrier, and physical and mental exhaustion have also been suspected as triggers.

Psychotherapeutic and supportive approaches should be used, and comorbid conditions should be identified and managed as appropriate.

Fugue State

Fugue state, previously also called dissociative fugue or psychogenic fugue, is a rare psychiatric disorder characterized by reversible amnesia for one's personal identity, which includes the memories, personality, belongings and other identifying characteristics of one's individuality and life. Usually, the fugue state lasts hours to days, but it has lasted for months.

The DSM-IV defines fugue state as a sudden, unexpected travel away from home or one's customary place of work, with inability to recall one's past; confusion about personal identity, or the assumption of a new identity that is associated with significant distress or impairment.

Fugues are usually precipitated by a strong emotional or physical stressor or stressful episode. After recovery from the fugue, there may be amnesia for the precipitating stressor.

Dissociative fugue usually involves unplanned travel or wandering around, sometimes accompanied by the establishment of a new identity. After recovery from fugue, previous memories usually return intact, but usually there remains complete amnesia for the fugue.

Dissociative fugue has been observed in the context of severe psychological or physical trauma, the ingestion of psychotropic substances, or a general medical condition. It has also been related to bipolar disorder, depression, delirium, and dementia.

Psychotherapeutic and supportive approaches should be used, and comorbid conditions should be identified and managed as appropriate.

Foreign Accent Syndrome

The foreign accent syndrome is a rare condition whereby someone speaks their native language as if they had a foreign accent. This syndrome usually follows a head injury, trauma, or stroke affecting the speech center of the brain.

Stockholm Syndrome

Stockholm syndrome characterizes a psychological response that can be observed seen in a victim, in which the victim shows signs of sympathy, loyalty, or even voluntary compliance with the victimizer, regardless of the risk in which the victim has been placed. The syndrome is most often discussed in the context of hostage abduction, but has also been described in relationship to rape, and spousal and child abuse. It can be understood as a severe form of reaction formation that takes place under enormous physiologic and emotional stress.

Stockholm syndrome is named after a bank robbery in Stockholm, Sweden. The bank robbers held bank employees hostage from August 23 to August 28 in 1973 and the hostages became emotionally attached to their hostage-takers. They even defended their captors after they were freed, refusing to testify against them.

A famous example of Stockholm syndrome is Patty Hearst. She was a millionaire's daughter who was kidnapped in 1974 and later took part in a robbery organized by her and her kidnapper.

As in all cases of severe trauma, psychotherapeutic and supportive approaches should be used, and comorbid conditions should be identified and managed as appropriate.

Lima Syndrome

Lima syndrome is the exact inverse of Stockholm syndrome. In this case, hostage-takers or victimizers become sympathetic to the wishes and needs of the hostages or victims.

Lima syndrome is named after the Japanese embassy hostage crisis in Lima, Peru, that lasted from December 17, 1996 until April 22, 1997. Fourteen members of the Tupac Amaru Revolutionary Movement took several hundred diplomats, government and military officials, and business executives of many countries hostage at a party that took place at the official residence of Japan's ambassador to Peru. Curiously, within a few days of the hostage crisis, the militants had released most of the captives, with seeming disregard for their importance, including the future president of Peru, and the mother of the current president.

After months of unsuccessful negotiations, all remaining hostages were freed by a raid by Peruvian commandos, although 1 hostage was killed.

It is unclear if Lima syndrome can be explained by feelings of guilt, moral indecisiveness, second guessing of one's actions, or obliviousness.

Stendhal Syndrome

Stendhal syndrome is characterized by physical and emotional anxiety up to the level of a panic attack, dissociative experiences, confusion, and even hallucinations when an individual is exposed to art. The syndrome is usually triggered by art that is perceived as particularly beautiful or when the individual is exposed to large quantities of art that are concentrated in a single place. The term can also be applied to a similar reaction to an overwhelming experience, for example when confronted with immense beauty in the natural world.

Stendhal syndrome is named after the famous 19th century French author Stendhal who described his experience with the phenomenon during his visit to Florence, Italy, in 1817, when he was 34 years old. It has also been called hyperculturemia or Florence syndrome.

Usually, Stendhal syndrome is self-limited and not followed by lasting or severe mental sequelae, and no interventions beyond supportive measures are needed.

Diogenes Syndrome

Diogenes syndrome is a condition characterized by extreme self-neglect, social withdrawal, lack of shame, apathy, and compulsive hoarding of rubbish. It is found mainly in old people and is associated with progressive dementia.

Diogenes syndrome is named after the Greek philosopher Diogenes of Sinope (412 or 404 BCE until 323 BCE), who was a Cynic and Minimalist. The philosophy of Cynicism is based on the belief that the purpose of life is to live a life of virtue in agreement with Nature. To achieve this goal, one had to reject all conventional desires for wealth, power, health, and fame, and live a simple life free from all possessions.

Diogenes took Cynicism to its logical extreme. He is said to have lived in a wine barrel on the streets of Athens, promoting ideas of nihilism and animalism. Famously, when asked by Alexander the Great, the most powerful person of that time, what he wanted most in the world, he replied, "For you to get out of my sunlight!"

The syndrome is actually a misnomer because Diogenes was not known to hoard or neglect his own hygiene and he sought discussions with other people in the Agora.

Munchausen Syndrome

Munchausen syndrome, also sometimes called hospital addiction syndrome, is named after Baron von Munchausen (1720-1797), an 18th-century German officer who was known for embellishing the stories of his life and experiences.

Munchausen syndrome is currently classified as a type of factitious disorder characterized by a person's repeatedly acting as if he or she has a physical or mental disorder when, in truth, he or she has caused the symptoms. People with factitious disorders act this way because of an inner need to be seen as ill or injured, not to achieve a concrete benefit, such as financial gain. They are even willing to undergo painful or risky tests and operations to get the sympathy and special attention given to people who are truly ill. Some will secretively injure themselves to cause signs like blood in the urine or cyanosis of a limb.

However, there is discussion to reclassify Munchausen syndrome as a somatoform disorder in DSM V because it is unclear whether people are conscious of drawing attention to themselves. While the "patient" role is familiar and comforting, filling a psychological need in people with Munchausen syndrome, the condition is different from hypochondriasis in that patients with Munchausen syndrome are aware that they are exaggerating, whereas sufferers of hypochondriasis believe they actually have a disease.

People affected by Munchausen syndrome deliberately produce or exaggerate symptoms in several ways. They might lie about or fake symptoms, hurt themselves, or change diagnostic tests. Possible warning signs of Munchausen syndrome include the following:

• Dramatic but inconsistent medical history;

• History of seeking treatment at numerous hospitals, clinics, and doctors' offices, possibly even in different cities;

• Extensive knowledge of hospitals and/or medical terminology, as well as the textbook descriptions of illnesses;

• Willingness or eagerness to have medical tests, operations, or other procedures;

• Presence of multiple surgical scars;

• Unclear symptoms that are not controllable and that become more severe or change once treatment has begun;

• Appearance of new or additional symptoms following negative test results;

• Predictable relapses following improvement in the condition;

• Presence of symptoms only when the patient is not alone or not being observed;

• Reluctance by the patient to allow healthcare professionals to meet with or talk to family, friends, or prior healthcare providers; and

• Problems with identity and self-esteem.

The exact cause of Munchausen syndrome is unknown, but a history of abuse or neglect as a child, or a history of frequent illnesses requiring hospitalization, might be factors associated with the development of this syndrome. Also, personality disorders are common in individuals with Munchausen syndrome.

Medical professionals or doctors suspecting Munchausen syndrome should first rule out the possibility that the patient does indeed have a disease but in an early stage and not yet clinically detectable. Although a person with Munchausen syndrome actively seeks treatment for the various disorders he or she invents, the person usually is unwilling to admit to and seek treatment for the syndrome itself. When treatment is initiated, the first goal is to modify the person's behavior and reduce his or her misuse or overuse of medical resources. Any underlying psychiatric disorder, such as a mood disorder, anxiety disorder, or personality disorder, should be identified and treated. As with other factitious disorders, the primary treatment for Munchausen syndrome is psychotherapy, including cognitive-behavioral therapy and family therapy.

People affected by Munchausen syndrome deliberately produce or exaggerate symptoms in several ways. They might lie about or fake symptoms, hurt themselves, or change diagnostic tests. Possible warning signs of Munchausen syndrome include the following:

• Dramatic but inconsistent medical history;

• History of seeking treatment at numerous hospitals, clinics, and doctors' offices, possibly even in different cities;

• Extensive knowledge of hospitals and/or medical terminology, as well as the textbook descriptions of illnesses;

• Willingness or eagerness to have medical tests, operations, or other procedures;

• Presence of multiple surgical scars;

• Unclear symptoms that are not controllable and that become more severe or change once treatment has begun;

• Appearance of new or additional symptoms following negative test results;

• Predictable relapses following improvement in the condition;

• Presence of symptoms only when the patient is not alone or not being observed;

• Reluctance by the patient to allow healthcare professionals to meet with or talk to family, friends, or prior healthcare providers; and

• Problems with identity and self-esteem.

The exact cause of Munchausen syndrome is unknown, but a history of abuse or neglect as a child, or a history of frequent illnesses requiring hospitalization, might be factors associated with the development of this syndrome. Also, personality disorders are common in individuals with Munchausen syndrome.

Medical professionals or doctors suspecting Munchausen syndrome should first rule out the possibility that the patient does indeed have a disease but in an early stage and not yet clinically detectable. Although a person with Munchausen syndrome actively seeks treatment for the various disorders he or she invents, the person usually is unwilling to admit to and seek treatment for the syndrome itself. When treatment is initiated, the first goal is to modify the person's behavior and reduce his or her misuse or overuse of medical resources. Any underlying psychiatric disorder, such as a mood disorder, anxiety disorder, or personality disorder, should be identified and treated. As with other factitious disorders, the primary treatment for Munchausen syndrome is psychotherapy, including cognitive-behavioral therapy and family therapy.

Munchausen Syndrome by Proxy

Munchausen syndrome by proxy (ie, through a substitute) is a type of factitious disorder in which a person acts as if an individual he or she is caring for has a physical or mental illness when the person is not really sick. The adult perpetrator has Munchausen syndrome by proxy and directly produces or lies about illness in another person under his or her care, usually a child under 6 years of age, but cases of adult victims have also been reported. Munchausen syndrome by proxy is considered a form of abuse by the American Professional Society on the Abuse of Children and occurs in about 2 out of 100,000 children.

People with Munchausen syndrome by proxy might create or exaggerate the child's symptoms in several ways. They might simply lie about symptoms, alter diagnostic tests (such as contaminating a urine sample), falsify medical records, or induce symptoms through various means, such as poisoning, suffocating, starving, and causing infection. The presenting problem may also be psychiatric or behavioral.

Common characteristics in a person with Munchausen syndrome by proxy include:

• Often a parent, usually a mother, but can be the adult child of an elderly patient;

• Might be a healthcare professional;

• Is very friendly and cooperative with the healthcare providers;

• Appears quite concerned (some might seem overly concerned) about the child or designated patient; and

• Might also suffer from Munchausen syndrome.

Other possible warning signs of Munchausen syndrome by proxy in children or cared-for adults include:

• The child has a history of many hospitalizations, often with strange symptoms;

• The child's reported condition and symptoms do not agree with the results of diagnostic tests;

• Worsening of the child's symptoms generally is reported by the mother and is not witnessed by the hospital staff;

• There might be more than 1 unusual illness or death of children in the family;

• The child's condition improves in the hospital, but symptoms recur when the child returns home;

• Blood in lab samples might not match the blood of the child; and

• There might be signs of chemicals in the child's blood, stool, or urine.

People with Munchausen syndrome by proxy have an inner need for the person they care for to be seen as ill or injured. The lying, fabrication of symptoms or harm is not done to achieve a concrete benefit, such as financial gain. People with Munchausen syndrome by proxy are willing to have the person under their care undergo painful or risky tests and operations to get the sympathy and attention given to people whose family member is truly ill. People who perpetrate this type of abuse are often affected by concomitant psychiatric problems, like depression, spouse abuse, psychopathy, or psychosis.

Etiologic and treatment considerations are identical to those in Munchhausen syndrome. The major difference lies in the fact that the first concern is to ensure the safety and protection of any real or potential victims. This might require that the child or elderly be placed in the care of others. Management often requires a team that includes social workers, foster care organizations, and law enforcement in addition to the healthcare providers. Successful treatment of people with Munchausen syndrome by proxy is difficult because those with the disorder often deny there is a problem.

Apotemnophilia and Acrotomophilia

Apotemnophilia, also known as body integrity identity disorder or amputee identity disorder, is most likely a neurologic disorder in which a person has the overwhelming desire to amputate healthy limbs or other parts of their body. Most date this desire to their childhoods and most often do not suffer from other psychological problems. In extreme cases, sufferers amputate their own limbs or ask others to do this for them. Because few surgeons are willing to amputate healthy limbs, this often means that the patients themselves will attempt to irrevocably damage the limb in question, necessitating formal amputation. After amputation, most report to being happy with their decision and often state, paradoxically, that they are now "complete." In addition, body integrity identity disorder also applies to people who wish to alter their bodily integrity in general.

Apotemnophilia is hypothesized to be related to right parietal lobe damage because the disorder has features in common with somatoparaphrenia, a type of monothematic delusion secondary to parietal lobe injury where the afflicted person denies ownership of a limb or an entire side of one's body.

The major problem in providing treatment is that most people with apotemnophilia do not seek professional treatment for their condition. Cognitive behavioral and aversion therapies have been tried.

Acrotomophilia is a form of sexual fetishism whereby the person without amputation or the wish to be amputated has a strong erotic interest in other people who are missing limbs. In the body integrity identity disorder community, these people are referred to as devotees. However, there might be some relationship between the 2 disorders, with some individuals exhibiting both conditions.