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Query: UMLS:C0004134 (ataxia)
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The authors report the case of an AIDS patient with rare neurologic manifestations: primary vasculitis of the central nervous system and VIII cranial nerve dysfunction. The authors make a review on the subject, and call special attention for the differential diagnosis. In fact, the patient, a 36 year old woman, with promiscuous life, presented with dizziness, gait ataxia, nausea, headache and hypoacusia. Seven days after the admission, she noted blurred vision in both eyes and soon she became blind. The physical examination showed bilateral optic neuritis and vestibulocochlear dysfunction, stiff neck and fever. No abnormalities were detected on CT scan. CSF showed 40 mononuclear cells/mm3, 79 mg/dl of proteins and normal glucose content. Microbiological research was negative. Serum anti-HIV test was positive. The hypothesis of primary CNS vasculitis was made, and pulse methylprednisolone therapy was introduced with good recovery of neurological syndrome except for persistent amaurosis.
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PMID:[Isolated vasculitis of the central nervous system and involvement of the 8th cranial nerve: rare manifestations of acquired immunodeficiency syndrome]. 130 67

Involvement of the central nervous system (CNS) is common in patients with advanced disease due to human immunodeficiency virus (HIV). Symptoms range from lethargy and apathy to coma, incoordination and ataxia to hemiparesis, loss of memory to severe dementia, and focal to major motor seizures. Involvement may be closely associated with HIV infection per se, as in the AIDS dementia complex, but is frequently caused by opportunistic pathogens such as Toxoplasma gondii and Cryptococcus neoformans or malignancies such as primary lymphoma of the CNS. The clinical presentations of attendant and direct CNS involvement are remarkably non-specific and overlapping, yet a correct diagnosis is critical to successful intervention. Toxoplasmic encephalitis is one of the most common and most treatable causes of AIDS-associated pathology of the CNS. A great deal has been learned in the last 10 years about its unique presentation in the HIV-infected patient with advanced disease. Drs. Benjamin J. Luft of the State University of New York at Stony Brook and Jack S. Remington of the Stanford University School of Medicine and Palo Alto Medical Foundation's Research Institute have studied T. gondii for many years and are two of the leading experts in the field. This commentary comprises an update of their initial review (J Infect Dis 1988;157:1-6) and a presentation of the current approaches to diagnosing and managing toxoplasmic encephalitis in HIV-infected patients.
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PMID:Toxoplasmic encephalitis in AIDS. 152 Jul 57

Neuro-cryptococcosis is a common opportunistic infection in AIDS or HIV infected patients. From a series of 10 neuro-cryptococcosis the four of them studied by magnetic resonance (MR) are reported. In AIDS patients a high suspicion of opportunistic infection of the CNS is needed as exemplified by two of the four patients who only presented cephalalgia. The other two patients suffered additional symptoms and signs of meningeal and CNS involvement, such as nuchal rigidity, cranial nerve palsies, papilloedema, gait ataxia and dismetria. Diagnosis was achieved (confirmed) by a positive culture, serology or indian ink test in CSF. CT scan did not contribute to the diagnosis and management of the patients. In contrast MR, showed in three of them a peculiar pattern of small, confluent, high-signal lesions, roughly symmetrically placed in the basal ganglia and the internal capsule. They probably correspond to the dilated Virchow-Robin spaces through which torulae migrate from the subarachnoid space.
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PMID:[Use of magnetic resonance in the diagnosis of neuro-cryptococcosis in the acquired immunodeficiency syndrome: study of 4 patients]. 155 79

Neurosyphilis, a sexually transmitted disease that can cause neurologic damage, has become increasingly prevalent in the AIDS era. HIV carriers can contract neurosyphilis without the presence of other concurrent opportunistic infections. Because MR findings of neurosyphilis are seldom reported, we retrospectively reviewed and evaluated contrast-enhanced MR images of six young (average age, 33 years) HIV-positive men with high serum and CSF VDRL titers indicative of neurosyphilis. All six patients tested negative for concurrent opportunistic infections. Five patients had acute or subacute strokelike symptoms involving the basal ganglia or middle cerebral arteries; one had a parietal convexity mass mimicking meningioma with headache and ataxia. Contrast-enhanced MR images showed patchy enhancement involving the basal ganglia and middle cerebral artery territories in the first five patients and the convexity mass in the sixth patient. On the basis of brain biopsy, a convexity mass was diagnosed in the patient with syphilitic gumma. The imaging findings of the remaining five patients represented ischemic infarct caused by meningovascular syphilis. After penicillin treatment, serum and CSF VDRL titers decreased, and neurologic signs and symptoms improved in all six patients. A follow-up MR study in the patient with the gumma showed that the lesion resolved almost completely. In young HIV patients with stroke symptoms or a convexity mass, neurosyphilis should be considered. Contrast-enhanced MR can reveal the extent of involvement by neurosyphilis and should be used to facilitate diagnosis and proper treatment.
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PMID:Neurosyphilis in HIV carriers: MR findings in six patients. 159 Jan 35

Postmortem examination of 21 patients showed a vacuolar myelopathy resembling that associated with the acquired immunodeficiency syndrome. Underlying diseases included six cases of leukemia or lymphoma, five of carcinoma, three of systemic lupus erythematosus, two of chronic lung disease, and one each of cadaveric renal transplant, cirrhosis, diabetes, hemophagocytic syndrome, and viral encephalitis. Fourteen patients were on long-term steroid therapy and 10 of these also had immunosuppressive chemotherapy. No patient had the acquired immunodeficiency syndrome, although one received blood transfusions in 1978. Signs and symptoms consistent with myelopathy included paraparesis in seven patients, ataxia in one, and bilateral extensor plantar reflexes in one. Microscopic examination showed vacuolation in spinal cord white matter primarily located in posterior and lateral columns. Lipid-laden macrophages and axonal changes were proportional to the severity of the vacuolation, which was severe in five patients, moderate in 10, and mild in six. Eight patients had coexistent viral diseases elsewhere in the central nervous system, but viral-associated antigens or genomic material was not found in regions of vacuolated spinal cord white matter. Although the etiology of these myelopathies is unknown, their association with immune suppression and coexistent viral infection of the central nervous system suggests that an opportunistic viral infection may be important.
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PMID:Idiopathic myelopathies with white matter vacuolation in non-acquired immunodeficiency syndrome patients. 186 65

Twenty-six patients with cryptococcal meningitis were seen in Auckland between 1969 and 1989. The incidence of cryptococcal meningitis in Auckland residents was 0.12 cases/100,000/year. Ten (38%) of the patients were Maori or Pacific Island Polynesians. Nineteen (73%) had a predisposing cause, including immunosuppressive therapy in nine and the acquired immunodeficiency syndrome (AIDS) in seven. The most common presenting syndrome was a subacute or chronic meningitis. Other clinical syndromes included a slowly progressive ataxia, polyradiculopathy, and headache with vomiting. In two patients, the symptoms of meningitis were overshadowed by those of systemic cryptococcal infection. Delay in making the diagnosis was common. The most sensitive method for diagnosing cryptococcal meningitis was the cerebrospinal fluid cryptococcal antigen test. Antifungal therapy cured 17 of the 25 (68%) treated patients overall, 15 of the 19 (79%) without AIDS and six of the seven with no underlying disease.
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PMID:Cryptococcal meningitis in Auckland 1969-89. 202 Apr 43

Molecular epidemiology may help fill the gap between epidemiological and biological models for cancerogenesis, allowing useful comparisons between series of cases carrying different biological characteristics. The defective regulation of immune responses is probably the common basis of cancer origin in genetically determined immune deficiency (GDID). Lymphomas are the most common neoplasms, showing an extremely high incidence in early age, frequent unusual location (extra-nodal) and histology, and rapid progression and spread with little response to therapy. A high incidence of lymphoma is also found in acquired (AIDS) or iatrogenic (transplant recipients) immune defective patients. The emergence of a malignant clone may be linked to unregulated polyclonal B- or T-cell proliferation and to disturbances in chromosomal rearrangements and in clonal selection, which are unique features of the immune cells regulation. It is useful to compare patterns of malignancies observed in GDIDs and in chromosomal breakage syndromes (CBS). In ataxia-teleangectasia (AT), selective errors at sites of special recombination involved in immune cell rearrangements may account for both immune deficiency and frequent types of malignant transformation. Different cytogenetic alterations and different types of malignancies are more common in Bloom syndrome, and in other GDIDs unrelated to chromosomal fragility, possibly due to different regulatory impairments. Viral infection (EBV, HPV, CMV) is likely to be a factor in any of the above steps. Therefore, individual exposure to viral (or other environmental) agents may be related to frequent location (skin, ano-genital areas, digestive tracts) of nonlymphatic cancers both in some GDIDs and in acquired or iatrogenic immune deficiencies. Cells that are homozygous for GDID are not malignant themselves, but are more likely to undergo new mutations to malignancy, due both to disregulation of the immune system and to environmental agents.
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PMID:Molecular epidemiology of cancer in immune deficiency. 203 52

Hallmarks of central nervous system (CNS) disease in AIDS patients are headaches, fever, subtle cognitive changes, abnormal reflexes, and ataxia. Dementia and severe sensory and motor dysfunction characterize more severe disease. Autoimmune-like peripheral neuropathies, cerebrovascular disease, and brain tumors are also observed. Histological changes include inflammation, astrocytosis, microglial nodule formation, and diffuse de- or dysmyelination. Focal demyelination can also be seen. It is clear that AIDS-associated neurological diseases are correlated with greater levels of HIV-1 antigen or genome in tissues. In AIDS dementia, macrophages and microglial cells of the CNS are the predominant cell types infected and producing HIV-1. However, manifestations of the disease make it unlikely that direct infection by HIV-1 is responsible. It seems more likely that the effects are mediated through secretion of viral proteins or viral induction of cytokines that bind to glial cells and neurons. HIV-1 induction of such cytokines as interleukin 1 (IL 1) and tumor necrosis factor-alpha (TNF alpha) may lead to an autocrine feedback loop involving further productive virus replication and induction of other cytokines such as interleukin 6 (IL 6) and granulocyte-macrophage colony-stimulating factor (GMCSF). Interleukin 1 and TNF alpha in combination with IL 6 and GMCSF could account for many clinical and histopathological findings in AIDS nervous system diseases. As HIV-1 infected patients produce elevated levels of IL 1, TNF alpha, and IL 6, it will be important to make a formal connection between the presence of these factors in the CNS, which are all products of activated macrophages, astroglia, and microglia, their in vivo induction directly by virus or indirectly by virus-induced intermediates, and the clinical and pathological conditions seen in the nervous system in this disease.
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PMID:HIV-1, macrophages, glial cells, and cytokines in AIDS nervous system disease. 206 87

Spinal cord lesions, although far less frequent than brain lesions, are not uncommon in AIDS. Almost all diffuse or multifocal pathological processes involving the central nervous system and/or leptomeninges may also affect the spinal cord. However, some of them involve it predominantly causing specific, clinically overt, myelopathic diseases. The most characteristic of these is vacuolar myelopathy which usually manifests as progressive spastic ataxia. Its incidence varies in different pathological series and its pathogenesis is still controversial. Acute meningomyeloradiculitis in AIDS patients presents a remarkably uniform and distinct clinical and cerebrospinal fluid profile although they might be due to various causes including cytomegalovirus infection, syphilis, mycobacterial infection or leptomeningeal malignancy. Tumoral focal syndromes have exceptionally been recorded due to epidural lymphomas, intraspinal gliomas or toxoplasma abscesses. Spinal cord ischemic lesions have been documented in two cases: they were related to inflammatory lesions of the blood vessels in one case and to diffuse intravascular coagulation in the other. An ascending myelitis syndrome, secondary to spinal cord infection by both herpes simplex virus type 2 and cytomegalovirus, without significant vasculitis has also been reported.
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PMID:[Lesions of the spinal cord and spinal roots in human immunodeficiency virus infection]. 207 14

A HIV-2 strain named HIV-2ben was isolated from peripheral blood lymphocytes of a patient who, since 1984, had developed neurological symptoms such as Raynaud's syndrome, followed by paresthesia of extremities and ataxia, and finally paraparesis of the legs and incontinence. This new isolate could be distinguished from HIV-2rod by antibody-binding epitopes, peptide maps of core p24 and p18 polypeptides and restriction endonuclease cleavage pattern.
AIDS 1990 May
PMID:Isolation and characterization of HIV-2ben obtained from a patient with predominantly neurological defects. 211 42


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