1/17. Significance of inflammatory changes in the brainstem in forensic autopsy cases.brain stem encephalitis is an uncommon disease. In order to assess the significance of inflammatory changes in the brain stem in a forensic autopsy material we reviewed the findings over a 12-year period. Between January 1st 1982-December 31st 1993, neuropathological examination of the brain was carried out in 29% of the autopsy cases from the Institute of forensic medicine, University of Oslo. Out of 4546 brains, 110 (2.2%) showed microglial nodules and perivascular lymphocytic cuffing in the lower brain stem. In 66 of the cases (60%), the abnormalities were limited to the nucleus and/or the spinal tract of the fifth cranial nerve. Only 16 of the 39 cases with more widespread changes, diagnosed as brain stem encephalitis, had a serious underlying or concomitant disease. Three particular cases of brain stem encephalitis are reported in more detail. In all three cases we suggest that the brain stem inflammatory changes may be either the direct or a contributory cause of death.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
2/17. Unusual presentation and MRI findings in Rasmussen's syndrome.Rasmussen's syndrome is a chronic disorder characterized by uncontrollable focal seizures and eventually epilepsia partialis continua, ipsilateral hemiparesis, developmental arrest, and cerebral inflammation. Viral and autoimmune etiologies have been postulated. A patient is presented who illustrates the wide variability of clinical and radiographic presentations in this disorder. The patient is an 8-year-old female who developed intermittent facial twitching at 2 years of age that eventually progressed to epilepsia partialis continua. electroencephalography demonstrated clinical seizures that emanated from the right parasagittal area. Cranial magnetic resonance imaging revealed pronounced atrophy of the right caudate nucleus, globus pallidus, and putamen, with mild increased T2-weighted signal in the right striatum, without accompanying cortical atrophy. Ictal single-photon emission computed tomography revealed markedly reduced uptake in the right hemisphere that was maximum in the right basal ganglia. cerebrospinal fluid, blood, and urine collected for metabolic and immunologic screening and dna testing for a wide variety of disorders were all unremarkable. Neuropsychologic testing demonstrated difficulties in memory, attention, and calculation. Brain biopsy revealed mild microglial activation, rare glial nodules, and collections of lymphocytes and histiocytes, consistent with the clinical diagnosis of Rasmussen's syndrome. After a modified hemispherectomy, she demonstrated marked clinical improvement.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
3/17. Paraneoplastic brain stem encephalitis in a woman with anti-Ma2 antibody.A woman developed brain stem encephalopathy in association with serum anti-Ma2 antibodies and left upper lobe lung mass. T2 weighted MRI of the brain showed abnormalities involving the pons, left middle and superior cerebellar peduncles, and bilateral basal ganglia. Immunohistochemical analysis for serum antineuronal antibodies was confounded by the presence of a non-neuronal specific antinuclear antibody. Immunoblot studies showed the presence of anti-Ma2 antibodies. A premortem tissue diagnosis of the lung mass could not be established despite two CT guided needle biopsies, and the patient died as a result of rapid neurological deterioration. The necropsy showed that the lung lesion was an adenocarcinoma which expressed Ma2 immunoreactive protein. Neuropathological findings included prominent perivascular inflammatory infiltrates, glial nodules, and neuronophagia involving the brain stem, basal ganglia, hippocampus and the dentate nucleus of the cerebellum. Ma2 is an autoantigen previously identified in patients with germ cell tumours of the testis and paraneoplastic brain stem and limbic encephalitis. Our patient's clinical and immunopathological findings indicate that this disorder can affect women with lung adenocarcinoma, and that the encephalitic changes predominate in those regions of the brain known to express high concentrations of Ma proteins.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
4/17. Glutaric aciduria type I diagnosed after poliovirus immunization: magnetic resonance findings.Glutaric aciduria type I is an uncommon inborn error of metabolism. It is a serious disease, often with a fatal outcome. magnetic resonance imaging findings and the clinical course of monozygotic twin females with glutaric aciduria type I who were admitted with acute encephalopathic crisis symptoms 3 days after immunization for poliovirus are presented in this report. magnetic resonance imaging findings revealed hyperintensity in the putamen, head of the left caudate nucleus, and globus pallidus, periventricular white matter (on T(2)-weighted images), arachnoid cysts in bilateral temporal regions, and enlargement of the sylvian fissures. Glutaric aciduria type I should be included in the differential diagnosis of patients with acute encephalopathic crisis occurring shortly after poliovirus immunization. Typical magnetic resonance findings guide urinary organic acid analysis in these patients.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
5/17. Neuropathological and ultrastructural features of amebic encephalitis caused by Sappinia diploidea.Here we present the neuropathological, ultrastructural, and radiological features of Sappinia diploidea, a newly recognized human pathogen. The patient was a 38-year-old man with visual disturbances, headache, and a seizure. Brain images showed a solitary mass in the posterior left temporal lobe. The mass was composed of necrotizing hemorrhagic inflammation that contained free-living amebae. Immunofluorescence microscopy showed that the organism was not a species of ameba previously known to cause encephalitis. trophozoites had a highly distinctive double nucleus, and transmission electron microscopy confirmed that they contained 2 nuclei closely apposed along a flattened surface. The 2 nuclei were attached to each other by distinctive connecting perpendicular filaments. This and several other unique structural features led to the diagnosis of S. diploidea encephalitis. The patient was treated postoperatively with a sequential regimen of anti-amebic drugs (azithromycin, pentamidine, itraconazole, and flucytosine) and is alive after 5 years. Guidelines to recognize future cases of S. diploidea encephalitis are as follows. 1) It presented as a tumor-like cerebral mass without an abscess wall. 2) It had central necrotic and hemorrhagic inflammation that contained acute and chronic inflammatory cells without granulomas or eosinophils. 3) It contained trophozoites (40-70 microm diameter) that contained a distinctive double nucleus. 4) Cyst forms in the host were not excluded or definitely evident. 5) trophozoites engulfed host blood cells and were stained brightly with Giemsa and periodic acid-Schiff. 6) trophozoites often were present in viable brain parenchyma on the periphery of the mass without inflammatory response. 7) The prognosis after surgical excision and medical treatment was favorable in this instance.- - - - - - - - - - ranking = 2keywords = nucleus (Clic here for more details about this article) |
6/17. Double encephalitis with herpes simplex virus and cytomegalovirus in an adult.A 51-year-old housewife developed symptoms of a cold followed by high fever, delirium, coma, rigidity of extremity muscles, positive Babinski sign and generalized convulsions, while complement-fixing antibody titre to herpes simplex virus in the sera raised over 128 X and declined to 8 X in the course. She finally expired of bronchopneumonia following status epilepticus after 94 days of illness. Severe necrosis with extensive hemorrhage in the white matter was predominant in the temporal, insular and orbitofrontal cortex, thalamus and globus pallidus. Focal rarefaction of the cerebral cortex with a very few eosinophilic intranuclear inclusions in the oligodendroglia and nerve cells, nerve cell destruction in the substantia nigra with glial nodules and perivascular inflammatory cell cuffs were observed. Abundant cytomegalic inclusion cells, originating from hypertrophic astrocytes, were present in the necrotic areas of cerebrum as well as in the rarefied tissue in the subependymal layers of the brainstem and cerebellum. Electron-microscopic study of the cytomegalic cells demonstrated the presence of numerous virions in both nucleus and cytoplasm. Fortuitour infection of the brain by cytomegalovirus with necrotizing encephalitis by herpes simplex virus is unique. The cause of double viral infections and severe lesions by less virulent strains is discussed.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
7/17. Bulbar encephalitis complicating trigeminal zoster in the acquired immune deficiency syndrome.A 30-year-old homosexual man with the acquired immune deficiency syndrome and a recent history of zoster involving the mandibular division of the right trigeminal nerve was found at autopsy to have a predominantly demyelinating lesion restricted to the ipsilateral spinal trigeminal tract and nucleus. Cowdry A inclusions were readily apparent in the nuclei of numerous glia and isolated neurons, and inclusion-bearing cells were immunoreactive with an antiserum to the varicella zoster virus (VZV). This represents the first demonstration that zoster-associated trigeminal encephalitis is the result of brainstem invasion by VZV. The restricted topography of this lesion implicates axonal transport in its pathogenesis.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
8/17. herpes simplex encephalitis: further evidence for persistent infection in the CNS.herpes simplex virus (HSV) antibody titers and IgG, IgM and IgA concentrations in the cerebrospinal fluid were serially measured in a patient with HSV encephalitis during a follow-up period of 32 months. HSV antibody titers, all classes of immunoglobulins and Ig% showed significant elevation during the course of illness, though IgM% and IgA% gradually declined after the acute phase. Autopsied brain tissue failed to yield a virus isolate, but conspicuous perivascular lymphocyte infiltrate, which is compatible with HSV encephalitis, was seen. These observations suggest the occurrence of HSV persistence and persistent antigen stimulation in the central nervous system, analogous to the well-recognized latent condition within the trigeminal ganglion.- - - - - - - - - - ranking = 19.974328677266keywords = ganglion (Clic here for more details about this article) |
9/17. Chronic intestinal pseudoobstruction as a possible sequel to encephalitis.A patient is reported who presented with a typical intestinal pseudoobstruction syndrome. Before this illness, the patient had suffered from measles encephalitis at the age of 15 months. A postencephalitic syndrome was present which included severe mental retardation, parkinsonism, and epilepsy. There were no relatives with a similar disease. Clinically, there was a frank recurrent pseudoobstruction syndrome with occasional diarrhea. Radiographic barium studies showed delayed transit, hypomotility of the intestines, and gross distension of the esophagus, stomach, small intestine, and colon. Histological examination of the gastrointestinal tract revealed a normal appearance of the mucosa; however, there was a hypertrophic muscle layer with abnormalities of the plexuses. Both the submucosal and the myenteric plexuses were reduced in number and size. They showed a decreased number of ganglion cells, proliferation of schwann cells and infiltration by lymphocytes. The abnormalities were most strikingly present in the esophagus and the small intestine. The intestinal neuropathy resulting in clinical pseudoobstruction is proposed to be part of the generalised neurological pathology as a late sequel to the measles encephalitis.- - - - - - - - - - ranking = 19.974328677266keywords = ganglion (Clic here for more details about this article) |
10/17. Isolated bilateral complete ptosis.A 45-year-old man had bilateral complete ptosis attributed to damage to the central caudal nucleus in the nuclear complex of the third cranial nerve, the other subnuclei being spared. Many discrete inflammatory foci were found in the midbrain and brainstem, a picture resembling that seen in subacute encephalitis. The findings are related to Warwick's concept of the anatomy of the third nerve.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
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