RESUMO
An HIV-1-infected patient with progressive multifocal leukoencephalopathy presented clinical deterioration and contrast-enhancing lesions on brain nuclear MR after the initiation of highly active antiretroviral therapy (HAART). Brain biopsy identified an inflammatory reaction compatible with immune reconstitution inflammatory syndrome. Treatment with corticosteroids and transient suppression of HAART led to marked neurologic improvement.
Assuntos
Síndrome da Imunodeficiência Adquirida/complicações , Terapia Antirretroviral de Alta Atividade/efeitos adversos , Encéfalo/efeitos dos fármacos , Encefalite/induzido quimicamente , Encefalite/imunologia , Leucoencefalopatia Multifocal Progressiva/tratamento farmacológico , Adulto , Anti-Inflamatórios/uso terapêutico , Afasia/induzido quimicamente , Afasia/imunologia , Afasia/fisiopatologia , Encéfalo/imunologia , Encéfalo/patologia , Quimiotaxia de Leucócito/efeitos dos fármacos , Quimiotaxia de Leucócito/imunologia , Dexametasona/uso terapêutico , Encefalite/fisiopatologia , Hemiplegia/induzido quimicamente , Hemiplegia/imunologia , Hemiplegia/fisiopatologia , Humanos , Corpos de Inclusão/imunologia , Corpos de Inclusão/patologia , Corpos de Inclusão/virologia , Vírus JC/imunologia , Vírus JC/isolamento & purificação , Leucoencefalopatia Multifocal Progressiva/fisiopatologia , Leucoencefalopatia Multifocal Progressiva/virologia , Macrófagos/efeitos dos fármacos , Macrófagos/imunologia , Imageamento por Ressonância Magnética , Masculino , Oligodendroglia/imunologia , Oligodendroglia/patologia , Oligodendroglia/virologia , Linfócitos T/efeitos dos fármacos , Linfócitos T/imunologia , Resultado do Tratamento , Suspensão de TratamentoRESUMO
BACKGROUND: Patients with hypothalamic hamartomas (HH) often have severe refractory epilepsy, incapacitating behavioral abnormalities, and cognitive decline. Attempts to control the seizure disorder by resection of apparently epileptogenic mesial temporal or other cortical structures have failed consistently. OBJECTIVE: To report a series of 13 patients in whom the hamartoma itself was resected. METHODS: All patients underwent preoperative evaluation between ages 2 and 33 years and had subtotal or complete resection of the hamartoma. Follow-up ranged from 1 to 5.5 years (mean: 2.8 y). RESULTS: Preoperatively, all patients had variable combinations of gelastic, complex partial, and generalized seizures. Eight had drop attacks. In addition, all had marked behavior abnormalities and cognitive impairment. Postoperatively, two patients are completely seizure-free and 11 are either seizure-free or have achieved a greater than 90% reduction of drop attacks and generalized tonic-clonic seizures. However, minor gelastic, complex partial, and atypical absence seizures have persisted in 11 patients, although at significantly reduced rates. In addition, there has been a dramatic improvement in behavior and cognition. Three patients had an anterior thalamic and one a capsular infarct, which left only minimal long-term deficits. Exact location of the lesion in relation to the interpeduncular fossa and the walls of the third ventricle correlated with extent of excision, seizure control, and complication rate. CONCLUSION: Resection can alleviate both the seizures and the behavioral and cognitive abnormalities of hypothalamic hamartomas, but complications are frequent.