Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 60-year-old male patient, originating from West Africa, developed acute and regressive neurologic symptoms associated with aphasia, apraxia, acalculia, behavioral impairments, and an epileptic phase. Eighteen months after the onset of the disease, the patient was almost normal. All along the clinical course, biological abnormality patterns were minor. We noted only a mild neutropenia in the blood. We also observed a weak lymphocytosis and elevated protein content in the cerebrospinal fluid. Electroencephalogram examination revealed slow waves which disappeared after remission. A weak ventricular dilatation was detected on CT scan. Neither vascular, nor tumoral, nor a classical infectious origin could be identified. While the patient was seronegative to HIV, a HIV-like virus was isolated twice from his peripheral blood lymphocytes during the disease. Eighteen months later, the patient remained seronegative. He developed neither AIDS nor immunodeficiency. The subtype of HIV has been isolated and characterized, and its neurotropism is being investigated.
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PMID:Isolation of HIV in a seronegative demented patient without symptoms of immune deficiency. 318 Jan 31

This case of microsporidiosis manifested as mutiple intracranial lesions separated in space and time, and neurological and radiological findings were improved with albendazole administration. A 33-year-old man presented with headache, fever, and dysphasia. His consciousness was clear. Neurological examination revealed acalculia, agraphia, and homonymous hemianopsia. He had a past history of febrile convulsive seizures of unknown cause until 14-years-old, but no history of immunodeficiency. T1-weighted magnetic resonance (MR) imaging showed a hypointense lesion with a hyperintense part, and ring-like enhancement with gadolinium-diethylenetriaminepenta-acetic acid (Gd-DTPA), in the left temporal lobe. T2-weighted and diffusion-weighted MR imaging showed the lesion surrounded by moderate hyperintense areas. He underwent gross total resection of the lesion. Histological examination demonstrated intracellular clusters of small basophilic spore-like bodies in the astrocytes, suggestive of microsporidia-infected astrocytes. However, immunohistochemical, polymerase chain reaction, and serological analyses failed to confirm the definitive diagnosis of microsporidiosis, so that he received no further treatment. Three years later, he presented with sensory disturbance in the left side of his face and left cerebellar ataxia, followed by fever, abnormal sensation in the left side of his face, and aggravated ataxia of the left upper and lower extremities on day 10 after admission. T1-weighted MR imaging with Gd-DTPA showed an enhanced lesion with irregular margin in the left cerebellar peduncle. T2-weighted MR imaging showed a diffuse hyperintense region around the lesion. Cerebrospinal fluid culture, serological analysis for autoimmune disease, and thoracic, abdominal, and pelvic computed tomography and 18F-fluorodeoxyglucose-positron emission tomography detected no abnormalities such as cancers or other lesions in the extracranial organs. No definitive diagnosis was obtained, but recurrence of microsporidiosis was the most probable cause. Administration of albendazole (600 mg/day) was started on day 15, because of rapid neurological and radiological deterioration. This treatment resulted in clinical improvement and disappearance of the lesion on MR imaging after daily administration for 4 weeks. He was discharged on foot with moderate sensory disturbance in the left side of the face and ataxia. Based on the clinical course and negative findings, the final diagnosis was microsporidiosis. This case suggests that microsporidiosis in the central nervous system can persist even in immunocompetent patients without involvement of any other organs, and that albendazole administration is likely to be effective.
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PMID:[Multiple intracerebral enhanced lesions strongly suspected to be microsporidiosis. A case report]. 1863 8