Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038220 (status epilepticus)
 7,272 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

There have been no serial studies about neuroradiological findings of neurosyphilis in the literature. There have been only case reports concerning HIV negative patients with neurosyphilis. We present 8 HIV negative neurosyphilis patients two of whom are women. The mean age of the patients was 48 years+/-12.37. Five of the 8 patients had general paresis, two optic atrophy and one multiple cranial neuropathies. The CSF findings were quite similar in 6 of 8 patients. In half of the patients cranial MRI showed mild cerebral atrophy. Nonspecific hyperintense small foci in 3 patients are thought to be related to syphilis. Hyperintensity involving bilateral medial and anterior temporal regions more prominent on the left side was seen in one of the patients with general paresis. This finding may be due to cytotoxic edema associated with status epilepticus and may mimic herpes simplex and other limbic encephalitides. Though not typical, certain MRI findings guides for the diagnosis of neurosyphilis.
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PMID:Clinical and magnetic resonance imaging findings of HIV-negative patients with neurosyphilis. 1734 38

Non-herpetic acute limbic encephalitis (NHALE) is a cause-unknown, inflammatory disease entity that affects the limbic system restrictedly. Neuropathological changes of NHALE have been described in only one report. A 53-year-old Japanese woman developed high fever, disturbance of consciousness and intractable generalized convulsions. Her symptoms were resistant to aggressive therapy including corticosteroid, acyclovir, gamma-globulin and anticonvulsants, and progressed acutely, leading to status epilepticus at 6 days after the onset. Brain MRI revealed inflammatory changes restricted to the limbic system. Herpes encephalitis was excluded by laboratory tests. Although paraneoplastic limbic encephalitis could not be excluded completely, no tumor lesions were detected on chest CT and abdominal ultrasonography. The patient died of multiple organ failure at 34 days after the onset of the disease. Histologically, there were neuronal loss and severe gliosis with an increase in hypertrophic astrocytes and with perivascular cuffings around several small blood vessels restricted to the hippocampus and the amygdala. Immunohistochemically, macrophages and activated astrocytes were distributed more widely over the limbic system, namely internal capsule, thalamus, caudate nucleus and substantia nigra. Herpes simplex virus I(1) and II(2) were negative immunohistochemically. The neuropathological findings of this case were similar to that of the previous report. NHALE is supposed to be a distinct disease entity neuropathologically and the fulminant form of NHALE exists.
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PMID:An autopsy case with non-herpetic acute limbic encephalitis (NHALE). 1828 67

In Japan,the prevalence of non-herpetic acute limbic encephalitis (NHALE),characterized by a lack of evidence of the herpes simplex virus (HSV) genome or enzyme-linked immunosorbent assay (ELISA) antibody,has shown an upward trend. The causes of NHALE include several anti-neural antibodies related NHALE. Among them,NHALE that is characterized by the onset of abnormal behavior and presence of anti-glutamate receptor epsilon2 (GluRepsilon2) antibody is gaining attention. NHALE was identified in 1994 during a survey of herpes simplex encephalitis in Kyushu District. This disease has not been reported in individuals belonging to countries other than Japan. In this review article,3 cases of NHALE patients with positive GluRepsilon2 antibody titers and ovarian-teratoma-related anti-N-methyl-D-aspartate receptor (NMDAR) antibody-positive encephalitis are briefly described. In addition,the naming of this disease as well as its pathogenesis,clinical features, prognosis, and sequels are discussed in this report. Patients in the acute stage of NHALE frequently exhibited schizophrenic-like symptoms such as abnormal behavior, incoherence, delusions, and hallucinations followed by convulsive seizures, status epilepticus, and autonomic seizures. Mild signs of meningeal irritation were also detected. Magnetic resonance imaging (MRI) in these patients often revealed bilateral abnormalities in the limbic areas, including the hippocampus and amygdala. Examination of the cerebrospinal fluid (CSF) revealed mild pleocytosis, and sometimes, a lack of the pleocytosis. The CSF level of interferon-gamma remained unchanged, whereas that of interleukin-6 was increased. The prognostic outcome of the patients was rather favorable. Further, differential diagnosis for herpes simplex encephalitis is important in order to decide the initial treatment-antiviral drug therapy or immunological therapy. The most commonly described sequel of this condition is memory impairment; however,patients should be monitored for personality or emotional changes.
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PMID:[Clinical characteristics of non-herpetic limbic encephalitis]. 2071 33

We report acute encephalopathy in two cases with severe congenital hydrocephalus. Case 1 was a 23-month-old girl, born at of 36weeks gestation and delivered by cesarean section due to congenital hydrocephalus. Magnetic resonance imaging (MRI) showed prominent ventricular dilation associated with hydrocephalus, Dandy-Walker variant and cortical malformation. The blood test for toxoplasmosis, syphilis, varicella-zoster, rubella, cytomegalovirus, and herpes simplex virus (TORCH) complex and various metabolic tests of blood and urine specimens yielded unremarkable results. She was admitted to our hospital for respiratory failure with fever and her clinical course deteriorated, progressing to hemiconvulsion hemiplegia epilepsy syndrome. Case 2 was a 17-month-old boy, born by spontaneous vertex delivery at 39weeks. Severe, asymmetrical ventricular dilation associated with hydrocephalus, cerebellar and brainstem hypoplasia, and punctuate calcifications of the thalamus, third and fourth ventricles, around the aqueduct, were observed on computed tomography (CT). The blood test for TORCH complex and various metabolic tests of blood and urine specimens yielded unremarkable results. He was admitted to our hospital for status epilepticus with fever and his clinical course progressed to hemorrhagic shock and encephalopathy syndrome. In patients with brain disorders, diagnosis and treatment are likely to be delayed and prognosis may thereby be worsened. When status epileptics or prolonged coma manifests even in patients with severe brain disorders, we must consider encephalopathy in the differential diagnosis.
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PMID:Acute encephalopathy in two cases with severe congenital hydrocephalus. 2109 88

Periodic Lateralized Epileptiform Discharges (PLEDs) are usually seen in the context of destructive structural lesions of the cortex, more frequently in acute ischemic stroke and less common in tumours and meningoencephalitis, specially herpes simplex virus. Its origin and prognosis are uncertain but it is known that PLEDs are linked to epilectic seizures, including status epilepticus.We report on a 75-year old woman with pneumococcal meningoencephalitis who presented altered level of consciousness, acute focal deficits, convulsive seizures and PLEDs in left hemisphere. The finding of PLEDs on the electroencephalogram is related to focal lesions of heterogeneous origin, which up to date, have not been documented in pneumococcal infections of the central nervous system. Our case highlights the importance of identifying and addressing any modifiable etiologic factors of PLEDs.
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PMID:Periodic lateralized epileptiform discharges as manifestation of pneumococcal meningoencephalitis. 2170 2

We report on a 70-year-old woman with partial complex status epilepticus who was initially diagnosed with herpes simplex-2 (HSV-2) encephalitis, based on brain magnetic resonance imaging (MRI) findings, cerebrospinal fluid (CSF) lymphocytic pleocytosis and HSV-2 DNA detection by polymerase chain reaction (PCR) in the CSF, but without improvement on intravenous acyclovir. Anti-Ri antibodies were positive and computed tomography (CT) investigations revealed a small cell carcinoma at biopsy suggesting paraneoplastic encephalitis. The outcome was unfavourable and the autopsy showed typical features of paraneoplastic encephalitis but no evidence of viral inclusions. This case report is interesting because: (1) it is the first report of an autopsy proven paraneoplastic widespread encephalitis with anti-Ri antibodies; (2) despite a positive HSV-2 PCR in the CSF, there was no sign of herpetic infections of the nervous system; and (3) it illustrates the fact that if paraneoplastic antibodies are usually good markers of the underlying tumour, they are not always predictive of neurological deficits.
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PMID:Encephalitis with herpes simplex-2 in the cerebrospinal fluid and anti-RI (ANNA-2) antibodies: an infectious or a paraneoplastic syndrome? 2183 42

We report a 14-month old child with multiple episodes of febrile status epilepticus, followed by chorea and developmental regression, caused by human herpes virus-6 encephalitis. Chorea has been described as a complication of relapsing herpes simplex virus I infection, but not as a manifestation of human herpes virus-6 infection. It is uncertain whether the chorea was an autoimmune phenomenon or a direct effect of the virus. The child was treated with levetiracetam, intravenous immunoglobulin, and foscarnet. The seizures and chorea resolved with treatment, but developmental regression, with loss of language skills, persisted 6 months after the illness. This child illustrates a new clinical presentation of human herpes virus-6 encephalitis, adds to the spectrum of disorders caused by this virus, and strengthens the case for routine identification of specific viral agents in all cases of childhood viral infections with central nervous system symptoms to determine optimal treatment and prognosis.
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PMID:Chorea and developmental regression associated with human herpes virus-6 encephalitis. 2341 79

New onset refractory status epilepticus (NORSE) is a relatively novel concept used to describe a cohort of previously healthy young adults mostly women presenting with denovo refractory status epilepticus which has a miserable impact on the outcome. Various infectious and non-infectious causes have been considered to be responsible for this dreaded syndrome; however, many a times the exact cause is not identified. As therapy with antiepileptic and anaesthetic drugs is not so successful, identifying and treating the exact cause could improve the outcome. Here the authors describe a woman who presented with NORSE. Investigations confirmed the diagnosis of herpes simplex encephalitis (HSE) and she responded drastically to acyclovir along with complete control of seizures. In this case, NORSE was the presenting feature of HSE and the refractoriness of her seizures was terminated only after treating the exact cause, that is, encephalitis.
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PMID:New onset refractory status epilepticus (NORSE) as the heralding manifestation of herpes simplex encephalitis. 2388 85

We present the case of a 52-year-old man with hypertension, diastolic congestive heart failure, end-stage renal disease on hemodialysis 3 times a week and a remote history of a hemorrhagic stroke who presented to the emergency department with a vesicular rash on his left arm. The rash was observed to be in a dermatomal distribution, and a diagnosis of herpes zoster was made. The patient was discharged home on valacyclovir 1 g 3 times a day for a duration of 7 days. The patient took 2 doses of valacyclovir before presenting to the hospital again with irritability and hallucinations. Over the next several days, the patient's neurologic status declined and he became disoriented and increasingly somnolent. Because of a concern for varicella zoster virus (VZV) or herpes simplex virus (HSV) meningoencephalitis, acyclovir was initiated intravenously at 600 mg (10 mg/kg) for every 12 hours. Computed tomography and magnetic resonance imaging of the brain failed to reveal an acute process. Electroencephalogram was interpreted as seizure activity versus metabolic encephalopathy. Lumbar puncture was not suggestive for meningitis, subarachnoid hemorrhage, or HSV/VZV infection. The patient subsequently had a witnessed seizure during dialysis and was felt to have status epilepticus due to acyclovir and valacyclovir neurotoxicity. The patient underwent daily hemodialysis for removal of the drug and eventually made a full neurologic recovery. Our case highlights that acyclovir neurotoxicity can result in status epilepticus, hallucinations, and altered consciousness. Differentiating acyclovir neurotoxicity from HSV or VZV meningoencephalitis is of crucial importance because the symptoms are similar but the management is vastly different.
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PMID:Valacyclovir and Acyclovir Neurotoxicity With Status Epilepticus. 2436 10

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a recently described and underdiagnosed entity that typically affects young, previously healthy individuals. Patients usually present in phases, which may include refractory seizures, psychosis, unresponsiveness, and autonomic instability. The diagnosis of anti-NMDAR encephalitis is challenging; however, prompt diagnosis and early treatment can lead to complete recovery. The incidence of anti-NMDAR encephalitis may be as high as four times that of encephalitis from herpes simplex, varicella-zoster, and West Nile viruses; however, it remains an underrecognized disorder. Early initiation of immunotherapy in anti-NMDAR encephalitis has been found to improve patient outcomes. Because of this, emergency physicians must be vigilant and consider this diagnosis in patients with altered mental status in whom a toxicologic or other etiology is not suspected. Early consideration of this diagnosis can facilitate urgent neurology consultation and prevent diagnostic delays arising from psychiatric referrals. It is essential to consider this diagnosis in suspicious emergency department presentations, particularly young patients who present with altered mental status, psychosis, or new-onset seizure activity when other obvious causes are ruled out. Emergency physicians should discuss the possibility of empirical intravenous immunoglobulin administration with neurology consultants if anti-NMDAR encephalitis is suspected. We describe the case of a 20-year-old man with anti-NMDAR encephalitis who presented to the emergency department with status epilepticus.
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PMID:Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis presenting to the emergency department with status epilepticus. 2522 54


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