Eun-Sook Cho

Vacuolar Myelopathy Pathologically Resembling Subacute Combined Degeneration in Patients with the Acquired Immunodeficiency Syndrome

Twenty of 89 consecutive patients with the acquired immunodeficiency syndrome (AIDS) in whom autopsies were performed over a 3 1/2-year period had a vacuolar myelopathy that was most severe in the lateral and posterior columns of the thoracic cord. Light and electron microscopy showed that vacuoles were surrounded by a thin myelin sheath and appeared to arise from swelling within myelin sheaths. Signs and symptoms referable to the spinal-cord lesions, including paraparesis, often accompanied by spasticity or ataxia (or both), were present in all five patients with marked pathological changes, in five of seven patients with moderate changes, and in two of eight patients with mild changes. Fourteen patients were demented. The clinical presentation was sufficiently distinctive to provide a guide for antemortem diagnosis. Possible causes of the vacuolar changes include uncharacterized viral infection or a metabolic derangement related to selective nutritional deficiency.

Neuropathology of Acquired Immunodeficiency Syndrome (AIDS): An Autopsy Review

In the brains and spinal cords of 153 adult patients dying with acquired immunodeficiency syndrome (AIDS) at New York and Memorial Hospitals a subacute encephalitis with multinucleated cells was present in 28% of all patients. This encephalitis was characterized by multinucleated cells primarily located in the white matter and associated with myelin pallor and sparse infiltrates of rod cells, macrophages, gemistocytic astrocytes and lymphocytes. The incidence per 12 month period ranged from 0 to 43% and significantly increased between 1983–84 (14%) and 1984–85 (43%). Recent virologic and pathologic studies suggest that this encephalitis may be caused by direct LAV/HTLV-III infection of the central nervous system (CNS). Cytomegalovirus encephalomyelitis and toxoplasmosis were the most common opportunistic infections (26% and 10%, respectively). Progressive multifocal leukoencephalopathy, herpes simplex ventriculitis, varicella-zoster leukoencephalitis and fungal infections were infrequent (less than 3% each). A nonspecific encephalitis with microglial nodules and with mild white matter changes occurred in 17%, vacuolar myelopathy in 29% and CNS lymphoma in 6%. Less than 20% of patients had either normal brains or terminal metabolic encephalopathies. This survey shows that neuropathologic complications of AIDS are frequent. Infections are the most common complication and are caused by probable LAV/HTLV-III infection, or by opportunistic organisms.

Immunopathology of secondary-progressive multiple sclerosis

Twenty‐three plaques obtained at early autopsy from 2 patients with secondary‐progressive multiple sclerosis were examined immunohistochemically for microglia/macrophages, and for immunoglobulins and components of activated complement. Most of the lesions examined in both cases exhibited evidence of low‐grade active demyelination of an unusual type (frustrated phagocytosis) in periplaque white matter. This included linear groups of microglia engaging short segments of disrupted myelin that were associated with deposits of C3d, an opsonin formed during complement activation. Similar microglia/C3d/myelin profiles were not observed in newly forming lesions in cases of acute multiple sclerosis or other central white matter diseases. As C3d coupling is known to increase the immunogenicity of potential antigens enormously, present findings point to disrupted myelin close to plaques as a possible source of the putative multiple sclerosis antigen. Ongoing myelin destruction found in a high proportion of old, established plaques was surprising. It suggests that slowly expanding lesions (progressive plaques), in which ongoing myelin breakdown occurs in the absence of florid perivascular cell cuffing or other histological signs of acute inflammation, contribute to disease progression in cases of secondary‐progressive multiple sclerosis.

Immunoglobulins and complement in postmortem multiple sclerosis tissue.

To identify evidence of a discrete, specific immune response in multiple sclerosis (MS) by analyzing the distribution of immunoglobulins and complement in tissue derived from cases of MS, and from control inflammatory white matter diseases known to express viral and autoantigens in the brain and spinal cord.

Neurological syndromes complicating AIDS.

This article presents a preliminary analysis of 118 acquired immunodeficiency syndrome (AIDS) patients with neurologic disease. 86% of patients in this series were homosexual, and the AIDS diagnosis was made on the basis of opportunistic infection in almost 50% and of Kaposis sarcoma with or without opportunistic infection in 38%. Central nervous system (CNS) infections (e.g., subacute encephalitis, toxoplasmosis, primary CNS lymphoma) were the most common group of complications, and autopsy findings indicated that 87% of patients eventually developed CNS complications. Subacute encephalitis was found alone or with other pathology in over 2/3 of autopsied brains. Principal neuropathologic changes in AIDS include the presence of scattered glial nodules, especially in gray matter. To define the clinical features of the glial nodule subacute encephalitis, the clinical features of 18 patients exhibiting these changes at postmortem examination were further analyzed. With the exception of seizures, clinical manifestations were nonfocal, correlating with the diffuse distribution of the microscopic pathology. CNS symptoms most commonly followed the diagnosis of AIDS by 2-9 months, and 11 of these 18 patients died within 6 months of onset of CNS dysfunction. The electroencephalogram (EEG) was abnormal in 100% of patients tested, while computerized tomographic (CT) scans were abnormal in 70%, with cortical atrophy being the most common finding. The cerebral spinal fluid (CSF) was abnormal in 67%. Early complaints were difficulty in concentration or memory loss which progressed in some to severe global dementia or coma. Neither the etiology nor the pathogenesis of subacute encephalitis have been established, although cytomegalovirus is regarded as a prime etiologic candidate.

Central nervous system tuberculosis: medical management and surgical indications.

BACKGROUND An increase in the incidence of tuberculosis in industrialized nations has prompted a need for earlier diagnosis, treatment, and isolation of disease. An associated rise in the number of patients with central nervous system tuberculosis (CNS TB) has forced neurosurgical services to reevaluate the indications for operative intervention. METHODS Seventeen cases of CNS TB were found in a retrospective review of all cases managed on the neurosurgical service between 1989 and 1994. These cases included eight with tuberculous meningitis, seven cases of supratentorial tuberculomas, and two cases of infratentorial tuberculomas. RESULTS Major permanent neurologic morbidity was seen in one case (6%). Five patients (29.4%) died of complications associated with their primary disease. Eleven patients (64.6%) had excellent outcomes. All patients in the latter group completed an 18-month course of antituberculous therapy. Cerebrospinal fluid shunts were necessary in three cases and emergent craniotomy was performed in three cases. Only four cases had human immunodeficiency virus (HIV) coinfection. CONCLUSION The neurosurgeons role in the management of CNS TB has once again become more evident. In the present series it is unclear as to whether this is due to multiple drug-resistant strains of Mycobacterium tuberculosis or HIV coinfection. It is clear, however, that vigilance over patient compliance and serial neurologic evaluation will determine the need for operative intervention.

Giant paraganglioma of the cauda equina in adolescence: magnetic resonance imaging demonstration.

A case of giant paraganglioma of the cauda equina is presented. This is only the second reported case in a minor and the first with magnetic resonance imaging. At 13 em long, this is the largest paraganglioma of the cauda equina ever recorded.

Vascular inflammation with eosinophils after the use of n-butyl cyanoacrylate liquid embolic system

Objective and importance Currently, n-butyl cyanoacrylate (n-BCA) is one of the most widely used liquid embolic agents in the treatment of intracranial arteriovenous malformations (AVMs). The cases of three patients are reported who underwent endovascular embolization with n-BCA, followed by resection in two and post-embolization hemorrhage with emergent evacuation in one, with histologic demonstration of an eosinophilic vasculitis found in resected AVM specimens. This is probably the first report of this tissue reaction, which may have theoretically serious clinical implications. Clinical presentation In this series, three patients (2 women, 1 man) presented with intracranial AVMs (Spetzler–Martin I–III) with the lesions located in the frontal lobe in two of the patients and in the parietal lobe in one. All patients presented with headache, and one also had new-onset seizures. Intervention All patients underwent embolization with n-BCA before a planned, staged surgical resection of the embolized AVMs. One patient had four embolizations over a 5-month period, one had three embolizations over 3 months complicated by hemorrhage after embolization requiring emergent evacuation of the hematoma, and the third patient had a single embolization. In all three patients, surgical and autopsy specimens showed an inflammatory response within the embolized vasculature with a prominent eosinophilic infiltrate. Conclusion The eosinophilic vasculitis seen in the pathology specimens may represent a previously undocumented hypersensitivity reaction following exposure to n-BCA, with the potential for adverse sequelae, including increased risk of hemorrhage as was seen in one of our patients.