Status epilepticus-induced neuronal loss in humans without systemic complications or epilepsy.

PURPOSE: To determine the regional distribution of neuronal damage caused strictly by status epilepticus (SE) without systemic complications, underlying brain pathology, or a history of preexisting epilepsy. METHODS: The medical records and electroencephalograms (EEGs) of three deceased patients who developed SE in the hospital were reviewed. Their brains were formalin-fixed, and 17 brain regions were selected, embedded in paraffin, and sectioned. Alternate sections were stained with either hematoxylin and eosin and cresyl violet to determine the extent of neuronal loss and gliosis or glial fibrillary astrocytic protein to confirm the extent of astrocytic proliferation. RESULTS: The three patients died 11 to 27 days after the onset of focal motor SE; none had hypotension, hypoxemia, hypoglycemia, or significant hyperthermia. Two patients had no prior seizures and no underlying brain pathology. The third patient, who had leptomeningeal carcinomatosis, had one seizure 2 months before the onset of SE. The duration of SE was 8.8 hours to 3 days. EEGs showed unilateral temporal lobe sharp-wave discharges in one patient and independent temporal lobe sharp-wave discharges bilaterally in the other two patients. In addition to widespread neuronal loss and reactive gliosis in the hippocampus, amygdala, dorsomedial thalamic nucleus, and Purkinje cell layer of the cerebellum, we report for the first time periamygdaloid (piriform) and entorhinal cortical damage occurring acutely after SE in humans. CONCLUSIONS: In the absence of systemic complications or preexisting epilepsy, SE produces neuronal loss in a distribution similar to that from domoic acid-induced SE in humans and from kainic acid- and pilocarpine-induced SE in rats.

Subdural neomembranes and sudden infant death syndrome.

Cranial dura maters of 36 consecutive infants with sudden infant death syndrome (SIDS) and 16 control infants coming to the Department of Coroner were examined microscopically to determine if subdural neomembranes are associated with cases submitted as SIDS. Thirty-one percent (31%) of the infants with SIDS and 13% of control infants had organizing subdural neomembranes (p > 0.05). Overall prevalence of organizing subdural neomembranes was 25% in the group examined. In all but two cases, birth trauma could be excluded as a cause of head trauma by aging neomembranes histologically. No association was found between type of delivery (vaginal or Cesarean) and presence of a subdural neomembrane. Subdural neomembranes are common in infants autopsied in a forensic setting, but they may be missed without a microscopic examination. Subdural neomembranes have no demonstrated association with SIDS.

Progressive multifocal leukoencephalopathy in AIDS: a clinicopathologic study and review of the literature.

We reviewed the clinical, radiographic, and pathologic features of 15 patients with the acquired immune deficiency syndrome (AIDS) and progressive multifocal leukoencephalopathy (PML). Brain tissue from 10 autopsy and 6 biopsy specimens was studied using: in situ hybridization (ISH) for JC virus (JCV), immunohistochemistry for human immunodeficiency virus (HIV) p24 antigen, and electron microscopy. Thirteen patients presented with focal neurologic deficits, while 2 presented with a rapid decline in mental status. PML was commonly the initial opportunistic infection of AIDS and produced hemiparesis, dementia, dysarthria, cerebellar abnormalities, and seizures. Magnetic resonance imaging was more sensitive than computed tomography in detecting lesions, and often showed multifocal areas of PML. CD4+ T-cell counts were uniformly low (mean 84/mm3), except in 1 patient who improved on 3'-azido-3'-deoxythymidine (AZT). PML involved the cerebral hemispheres, brain stem, cerebellum, and cervical spinal cord. The distribution of brain involvement was consistent with hematogenous dissemination of the virus. In 2 brain specimens, multiple HIV-type giant cells were present within the regions involved by PML. When co-infection by HIV and papovavirus was present, PML dominated the pathological picture. ISH for JCV showed virus in the nuclei of oligodendrocytes and astrocytes. Occasionally there was staining for JCV in the cytoplasm of glial cells and in the neuropil, the latter possibly a correlate of papovavirus spread between myelin sheaths, as seen by electron microscopy. ISH demonstrated more extensive foci of PML than did routine light microscopy.

Adrenal to caudate transplantation--postmortem study.

A patient with Parkinson's disease who had undergone bilateral adrenal to caudate implantation by stereotactic technique came to autopsy. The implantation was shown to have produced minimal trauma and to have been well placed. Minimal surviving adrenal medullary tissue remained in the implanted coil, and no outgrowth of tissue into the striatum occurred.

Upbeat nystagmus: clinicopathologic study of two patients.

Two patients had upward nystagmus on forward gaze before they died with acute caudal brainstem dysfunction. Bilateral dorsal paramedian damage in the rostral medulla, involving the perihypoglossal nuclei, was probably the critical lesion responsible for upbeat nystagmus.

CT of subinsular infarction and ischemia.

A number of CT head scans, covering a 2-year period and showing a variety of distinct curvilinear subinsular lucent lesions, were collected and reviewed. Variations in extent of involvement, tendency toward bilateral symmetry, and clinical background allowed the lesions to be grouped into four general patterns, most of which, to our knowledge, have not been specifically described in the radiologic literature. This project was undertaken first to bring to the attention of those involved in interpretation of cranial CT images several patterns of injury they may not heretofore have been aware of and second to attempt to derive a specific etiology for each of the patterns described. Pattern 1, which appears as a distinct curvilinear lesion (sometimes cystic) apparently limited to the lateral aspect of the putamen, is thought to represent the residua of previous lateral striatal hemorrhage. Pattern 2, occurring in a markedly younger age group appears as relatively symmetrical bilateral subinsular lucencies, which in one case completely resolved. A specific etiology for this pattern remains uncertain. Acute demyelination, either secondary to a variant of anoxic leukoencephalopathy or to a limited form of diffuse encephalomyelitis, is postulated. A third pattern, which extends from generalized deep frontal white-matter lucency across the anterior limb of the internal capsule and tapering posteriorly in the subinsular area is thought to be on the basis of chronic ischemia similar to subcortical arteriosclerotic encephalopathy. The fourth pattern, occurring as a broad band of lucency extending from the frontal horn of the lateral ventricle and also tapering posteriorly is due to relatively proximal occlusion of the lateral lenticulostriate arteries.

Narcolepsy: a neuropathologic study.

This article describes the neuropathologic findings in a woman who died suddenly at the age of 48 after having had symptoms of narcolepsy for 1 1/2 years. Gross examination of the brain showed an arachnoid cyst. The significant light microscopic findings consisted of focal gliosis of the ventrolateral caudal pons and the periventricular anterior hypothalamus. Although cases of symptomatic narcolepsy have been previously reported, to our knowledge structural brain changes in idiopathic narcolepsy have not been previously described. In the present case, there is no evidence that the narcoleptic symptoms were secondary to any other disorder, including the arachnoid cyst. This patient's symptoms may be related to the pontine and hypothalamic gliosis, the etiology for which is unknown.

Comparison of glucose metabolism, x-ray CT, and postmortem data in a patient with multiple cerebral infarcts.

We studied a 69-year-old man with multiple brain infarcts who died 8 days after being studied with positron emission tomography and (F-18)-fluorodeoxyglucose. Metabolic abnormalities were greater than structural changes in size and extent. They were found in areas with no gross pathologic abnormalities. Remote metabolic effects were attributed both to degeneration of fiber tracts with disconnection of remote structures, as demonstrated by hypometabolism in the left frontal cortex associated with an infarct in the genu of the left internal capsule, and to microscopic infarcts not apparent on gross examination but manifested as hypometabolic regions.

Locked-in syndrome due to tentorial herniation.

A 28-year-old man had a chronic locked-in syndrome following tentorial herniation caused by an epidural hematoma. Postmortem examination revealed bilateral corticospinal tract degeneration caudal to the midbrain, with infarction of the right internal capsule just rostral to the cerebral peduncle and pressure necrosis of the pyramidal portion of the left cerebral peduncle.

Small bowel resection with vitamin E deficiency and progressive spinocerebellar syndrome.

A 27-year-old woman who had undergone extensive small bowel resection at age 14 months developed kyphoscoliosis, ocular palsies, constricted visual fields, retinitis pigmentosa, progressive ataxia, muscular weakness, nearly absent vibration and impaired position sense, areflexia, extensor plantar responses, and macrocytic anemia. Her condition closely resembled Bassen-Kornzweig disease, but lipoprotein electrophoresis was normal. Mild fat malabsorption, lactic acidosis, and severe deficiency of vitamins A and E and carotene were documented. Serum B12 and folic acid levels were normal. During vitamin A and E therapy sufficient to elevate serum levels to the normal range, there was improvement of visual fields and visual acuity in dim light, lactic acidosis, and red cell volume. Progression of symptoms was halted during vitamin replacement therapy, and her gait improved. This syndrome is the human counterpart to vitamin E deficiency in experimental animals.

Pontine spongy degeneration of white matter associated with hepatic encephalopathy.

Spongy degeneration of white matter localized to basis pontis was found in a 47-year-old woman with hepatic encephalopathy. In this clinical setting, the lesion resembled those changes described by Victor et al and found primarily in cerebral cortex, subcortical white matter, and putamen. Isolated involvement of the basis pontis and ventral midline tegmentum of pons has not, to our knowledge, been previously reported. The morphologic appearance and distribution distinguish this lesion from central pontine myelinolysis. The possible vascular cause of this localized interstitial edema is discussed.

Levator-sparing oculomotor nerve palsy caused by a solitary midbrain metastasis.

One month before death, a 63-year-old man with known lung carcinoma manifested a left third-nerve palsy and crossed ataxia. The oculomotor involvement began with a dilated left pupil and progressed to a complete left oculomotor nerve palsy, with the exception of nearly normal lid function. Pathologic examination revealed a solitary midbrain metastasis involving the left third-nerve nucleus and rootlets, with the exception of the caudal central oculomotor subnucleus and its outflow fibers.

Pathology of CNS cysticercosis.

The pathology of CNS cysticercosis is dependent upon the route of infection, the larval form, its predilection for certain anatomic sites, and the host reaction. Clinicopathologic correlates are illustrated.

Phaeohyphomycosis of brain: granulomatous encephalitis caused by Drechslera spicifera.

Drechslera spicifera is a dematiaceous fungus which occurs widely in nature but only rarely has been implicated in animal and human infections. Previous infections have occurred in superficial and subcutaneous sites or were encountered in immunologically compromised hosts. This report documents a case of granulomatous encephalitis due to Drechslera spicifera in an immunologically competent woman with no known underlying disease, from whom the fungus was isolated. Animal pathogenicity studies in mice reproduced the histopathologic features, and the fungus was reisolated from the animals.

Cerebritis due to group B streptococcus.

A premature infant who died of early-onset group B streptococcal meningitis was found to have cerebritis with direct bacterial infection of the basal ganglia. Although the organism was sensitive to penicillin by in vitro testing, it was not eradicated from the cerebrospinal fluid after 48 h of antibiotic treatment. These findings illustrate that suppurative extension with cerebritis of the basal ganglia as a complication of group B streptococcal meningitis may be one of the factors responsible for treatment failure.

Computed tomography of posterior fossa trauma.

A group of 1,700 cases of head trauma were reviewed to determine the incidence of posterior fossa injuries and to assess the value of computed tomography (CT) in their diagnosis and management. In 57 cases (3.3%), the most significant and primary injuries were within the posterior fossa. These included epidural hematoma (EDH), acute and chronic subdural hematoma (SDH), and parenchymal hemorrhage and contusion of the cerebellum and brainstem. The prognosis varies with the location and severity of the injury. Brainstem injuries are associated with a high mortality rate. Computed tomography proves particularly useful in the early recognition of brainstem injury. The demonstration by CT of obliteration of the cisterns surrounding the brainstem is a reliable sign of a grave prognosis in brainstem injury. Contrast enhancement is useful in demonstrating whether the dural sinuses are displaced, thus differentiating EDH from SDH. Except in the rare case of vascular injury unassociated with EDH, CT correlated with the neurological examination is an accurate method of determining the nature, location, and extent of significant posterior fossa injury. Scans of high quality are mandatory, and frequent supplementary contrast studies are recommended.

CT of brainstem injury.

Cranial computed tomography (CT) scans of 1,600 head trauma patients, 67 of which demonstrated evidence of brainstem injury, were reviewed. CT diagnosis of brainstem injury was based on direct and indirect evidence. Direct signs, which include focal hemorrhage, significant intraparenchymal contrast enhancement, hemorrhagic contusion, and edema of the brainstem, appear as areas of high density, mixed density, and low density on the CT scan. Indirect signs are obliteration of the pontine, cerebellopontine angle, and perimesencephalic cisterns. Mortality and morbidity rates after brainstem injury are 2-3 times greater than for head trauma with descending transtentorial herniation, but without brainstem injury.

Computerized tomography in central pontine myelinolysis.

We describe an autopsy-proven case of central pontine myelinolysis (CPM) with premortem computerized tomographic (CT) visualization of the lesion on two scans, performed with an interval of 2 weeks. This case demonstrates the capability of CT to support the clinical diagnosis of central pontine myelinolysis. Identification of the condition should facilitate prompt initiation of aggressive supportive care.