RESUMO
INTRODUCTION: The cerebellum has been traditionally associated with motor control learning and performance. However, since 1970 a growing body of clinical and experimental evidences has suggested that the cerebellum may be involved in nonmotor cognitive functions as well. OBJECTIVE: To explore the presence of eventual cognitive impairment in non-demented patients with isolated degenerative cerebellar diseases. PATIENTS AND METHODS: Twelve patients with the diagnosis of selective degenerative cerebellar disorders, either inherited or sporadic, were selected (mean age: 40.42 +/- 13.49 years; mean education level: 9.92 +/- 3.99 years; duration of illness: 12.13 +/- 11.27 years, MMSE: 26.75 +/- 1.5) and evaluated through a standardized neuropsychological tests battery. Normalized Z scores were estimated and compared against 0, employing the t test for one sample. RESULTS: Significant cognitive deficits were found in the following domains: executive, visuo-spatial, memory and attention functions. Performance on the Wisconsin test showed a significative number of perseverative errors. Memory deficits included verbal learning and free recall difficulties, with good recognition of the material presented. CONCLUSIONS: The findings of this study are consistent with the role of the cerebellum as modulator of mental functions. The cognitive deficits resulting from cerebellar pathology may be related with the disruption of cerebello-cortical connexions involving a complex network which includes the prefrontal region, suggesting that the cerebellum may process cortical information coming from different areas linked with the control of cognition.
Assuntos
Doenças Cerebelares/fisiopatologia , Cerebelo/patologia , Testes Neuropsicológicos , Adolescente , Adulto , Cerebelo/fisiologia , Cognição/fisiologia , Transtornos Cognitivos/fisiopatologia , Feminino , Humanos , Masculino , Transtornos da Memória/fisiopatologia , Pessoa de Meia-IdadeRESUMO
INTRODUCTION: Limbic encephalitis is an unusual presentation of paraneoplastic syndrome, which includes among its symptoms seizures. CLINICAL CASE: We report a case with a rare presentation of limbic encephalitis as initial symptom of small cell lung carcinoma. A 69 year-old woman presented with partial non convulsive status epilepticus and neuropsychiatric disturbances. Chest radiography and computed tomography showed mediastinal lymphadenopathy and lung nodules. Subsequently, small cell lung carcinoma was diagnosed by lymph node biopsy. The cerebrospinal fluid study was normal. The electroencephalography and magnetic resonance imaging (MRI) findings had distinctive features compatible with temporo-limbic dysfunction. The anti-Hu antibodies were negative. The neuropsychiatric symptoms improved significantly after systemic chemotherapy and adjuvant radiotherapy. A serial follow-up MRI of the head showed no evidence of intracranial metastasis three months after the diagnosis of cancer. Limbic encephalitis may be an initial manifestation of lung cancer. Paraneoplastic limbic encephalitis is considered a remote effect of cancer commonly associated with anti-neuronal antibodies (anti-Hu) and small cell lung carcinoma. CONCLUSIONS: Status epilepticus could be an early sign of limbic encephalitis. The absence of anti-Hu antibodies does not rule out the presence of an underlying small cell lung carcinoma in patients with a clinical diagnosis of limbic encephalitis. Greater awareness for diagnosis and early treatment of the primary tumor offers the best chance for improvement in patients with lung cancer presenting with limbic encephalitis.