Cheng-Mei Shaw

CORRELATION OF EXPERIMENTAL ALLERGIC ENCEPHALOMYELITIS WITH DELAYED-TYPE SKIN SENSITIVITY TO SPECIFIC HOMOLOGOUS ENCEPHALITOGEN.

EAE is readily produced in many mammals and birds by a single injection of central nervous system (CNS) tissue in Freund’s “complete” adjuvants (water-in-oil emulsion with killed mycobacteria). The disease is generally believed to be due to delayed-type hypersensitivity, since it can be passively transferred by sensitized cells,’ but not by serum. Although delayed-type skin reactions to homologous and heterologous neural antigens have been reported in only heterologous reactions have been noted in guinea pigs.““ This discrepancy has been all the more puzzling since the guinea pig is generally regarded as the animal best suited for the demonstration of delayed-type hypersensitivity. Interpretations have, therefore, been cautious: positive reactions may merely have been temporal coincidences (“false witnesses”) of otherwise unrelated phenomena, and negative skin reactions may not have represented the true hypersensitivity reaction which can occur only in the CNS. Correlations between EAE and circulating antibody have been equally ambiguo~s ,~ , 5 , but may be clarified by other papers presented at this rn~nograph .~~’~ One possible explanation for the lack of correlation between EAE and either circulating antibody or delayed-type hypersensitivity may lie in the relative impurity of the encephalitogenic materials which have generally been used as test antigens. The cruder the antigenic mixture, the less correlation can be achieved between EAE and its specific encephalitogen; this is especially true when heterologous antigens are used, since foreign nonencephalitogenic antigens may be stronger than the organ-specific encephalitogenic antigen(s). The availability of a water-soluble, homologous, encephalitogenic protein which previously failed to produce any skin reactivityz9 but which recently has been shown to produce delayed-type skin reactions in guinea pigs3o provided the basis for this reinvestigation of a subject which has provided more controversy than theory suggests should be the case.

Multiple Sclerosis Beginning in Infancy

The clinical and autopsy findings on a 6-year-old child presenting with 11 episodes of relapsing neurological symptoms since age 10 months are reported. The brain showed multiple and irregular demyelinative lesions in the cerebral and cerebellar white matter as well as in the tegmentum and base of the brain stem. This case reconfirms the existence of typical multiple sclerosis (MS) in childhood, beginning even in infancy. In addition, elevated Epstein-Barr virus antibody titers during the clinical course of this patient raise an interesting but still speculative etiological possibility for MS. (J Child Neurol 1987;2:252-256).

Injury of the basilar artery associated with closed head trauma

The neuropathological findings at necropsy are described in three cases of basilar artery injuries and their consequences after head trauma and the mechanism of injuries is discussed. The first case was that of massive pontine infarct due to an occlusion of the basilar artery trapped in the fracture of the clivus, 13 days before death. The second case, also with a pontine infarct, survived for two and two-thirds months; the trauma was probably similar, but the presence of the basal skull fracture and the occlusion of the basilar artery can be surmised only in retrospect. The third case was one of delayed rupture of the basilar artery occurring three to four weeks after the injury, which caused aneurysmal formation in the left posterior cerebral artery.

Immediate and delayed neurotoxicity after mechlorethamine preparation for bone marrow transplantation.

Twenty-four patients, including two with aplastic anemia and 22 with malignancy, underwent marrow transplantation after preparation with mechlorethamine, 0.3 to 2.0 mg/kg body weight. Fourteen of the 21 neurologically evaluable recipients developed immediate neurotoxicity a median of 4 days after treatment (range, 0 to 34 days). Confusion and disorientation were observed in six patients, headache in six, hallucinations n four, lethargy in four, tremors in three, paraplegia in one, seizure in one, and vertigo in one. Whereas acute symptoms cleared in 11 patients, three remained symptomatic until death. Twelve evaluable patients survived more than 60 days; all six with previous acute toxicity subsequently developed delayed onset of new neurologic findings (personality change, confusion, seizure, diplopia, or dementia) a median of 169 days (range, 70 to 248 days) after treatment. Cerebrospinal fluid analysis was usually normal but cerebral computed tomographic scans showed ventricular enlargement and electroencephalograms showed diffuse slowing. Postmortem histologic examination of brain showed neuronal degenerative changes with increased vascularity, gliosis, and perivascular fibrosis. Neurotoxicity appeared to increase with age and mechlorethamine dose and was commoner in patients given additional procarbazine or cyclophosphamide.

Transsynaptic spread of varicella zoster virus through the visual system: A mechanism of viral dissemination in the central nervous system

We report a patient with pathologic evidence of anterograde spread of varicella zoster virus (VZV) through the visual system. A 29-year-old homosexual man developed the acquired immunodeficiency syndrome (AIDS) 2 months before the onset of left herpes zoster ophthalmicus. During the next 11 months, the zoster infection progressed to involve the left eye, with resultant keratitis, iritis, retinitis, and eventual blindness. Later, the patient developed bilateral blindness, left hemiparesis, and fatal pneumonia. At autopsy, the brain revealed destruction of the visual system and adjacent structures, with sparing of the remainder of the brain. Glial cells near the areas of necrosis showed Cowdry type A intranuclear inclusions. In situ hybridization with probes to VZV nucleic acid sequences were positive in the necrotic brain and retinal areas. Hybridization with probes to cytomegalovirus, herpes simplex virus type II, human immunodeficiency virus, and Epstein-Barr virus were negative. Electron microscopy revealed characteristic herpes group nucleocapsids. This case provides insight into the mechanisms of virus dissemination and the production of encephalitis.

Progressive myoclonus and epilepsy with dentatorubral degeneration: a clinicopathological study of the Ramsay Hunt syndrome.

Ramsay Hunts progressive myoclonus and epilepsy associated with dentatorubral degeneration is a rare disorder. We report a 19 year old woman with this clinical syndrome who also has a more mildly affected brother. Neuropathological in addition to dentatorubal involvement. The evidence suggests that this is a distinctive hereditary disorder producing neuromal degeneration at several levels in the central nervous system.

Do Neurological Signs Occur in Experimental Allergic Encephalomyelitis in the Absence of Inflammatory Lesions of the Central Nervous System

Guinea pigs with paralysis or other severe neurological signs of experimental allergic encephalomyelitis (EAE) always exhibited typical histological inflammatory lesions. A few animals inoculated with either the encephalitogenic emulsion or only the control adjuvant emulsion had mild weakness or slowness but no histologic lesions. In some instances, these signs were explained by coincidental non-neural disease or trauma. Therefore, such mild clinical signs cannot be considered pathognomonic of EAE. Reports from the literature suggesting that animals have developed clinical signs without histological lesions in EAE are considered invalid because of the nonspecificity of clinical signs, the occurrence of intercurrent diseases, the inadequacy or incorrect timing of histologic evaluations, and the lack of controls for specificity of the signs. There is no basis for the supposition that autoimmunity can cause major neurological signs in the absence of inflammatory lesions in the nervous system.

Nervous system disorders

A general classification of the pathologies or diseases that impact upon the nervous system. An unde…

Solitary eosinophilic granulomar of the temporal lobe: case report and review of the literature

A solitary eosinophilic granuloma of the central nervous system is an unusual manifestation of histiocytosis X. A unique case of a solitary eosinophilic granuloma of the right temporal lobe without osseous involvement is described. A 20-year-old man presented with a grand mal seizure. Magnetic resonance imaging demonstrated an intraaxial enhancing mass in the right temporal lobe with marked vasogenic edema. A right temporal craniotomy was performed for resection of the lesion and the diagnosis of an eosinophilic granuloma was confirmed by histopathology. Follow-up MR imaging obtained 5 years following resection demonstrated no recurrence. Solitary eosinophilic granuloma should be considered in the differential diagnosis of enhancing mass lesions affecting the central nervous system. Although the natural history of solitary eosinophilic granulomas remains poorly defined, surgical treatment still remains the mainstay of therapy for these unifocal cerebral lesions.

Radiation Therapy for Sarcoid of the ThalamussPosterior Third Ventricle

There are a limited number of previously reported cases involving the use of radiation therapy for sarcoid of the brain. The case of a 22-year-old man with a thalamic/posterior third ventricle sarcoid mass that grew despite steroid medication is presented. The patient was treated with external beam radiation to a total dose of 20 Gy, with 2-Gy fractions over 14 elapsed days. A complete radiographic response was achieved 4 months after radiation was completed. Radiographic follow-up through 8 months postradiation shows no evidence of disease recurrence. Fractionated radiation therapy in low-to-moderate doses appears to be efficacious in steroid-refractory sarcoid of the brain.