[Neuropsychological study of 12 patients with pure degenerative cerebellar disease]. / Evaluación cognitiva en 12 pacientes con enfermedad cerebelosa degenerativa pura.

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.

Evaluación cognitiva de 12 pacientes con enfermedad cerebelosa degenerativa pura / Neuropsychological study of 12 patients with pure degenerative cerebellar disease

Introducción. El cerebelo se ha asociado tradicionalmente con el aprendizaje y el control motor. Sin embargo, en las últimas tres décadas se ha obtenido evidencia clínica y experimental que sugiere que el cerebelo podría intervenir en el procesamiento cognitivo no motor. Objetivo. Estudiar el rendimiento neuropsicológico de pacientes con enfermedad cerebelosa degenerativa pura no dementes. Pacientes y métodos. Se evaluaron 12 pacientes con enfermedad cerebelosa degenerativa pura, de causa hereditaria o de presentación esporádica (edad: 40,42 13,49 años; educación: 9,92 3,99 años; tiempo de evolución: 12,13 11,27 años; MMSE: 26,75 1,5) por medio de una batería neuropsicológica estandarizada. Se calcularon puntuaciones normalizadas Z, que se compararon contra 0 mediante el empleo de pruebas de t de una muestra. Resultados. Se observaron alteraciones significativas en las pruebas de función ejecutiva, visuoespacial, memoria y atención. En el test de Wisconsin se registró un número significativo de errores perseverativos. Los fallos en memoria se caracterizaron por dificultades en el aprendizaje y la evocación diferida, con buen desempeño en el reconocimiento del material presentado. Los déficit cognitivos no se correlacionaron con la gravedad del déficit motor elemental. Conclusiones. Los datos obtenidos sugieren la participación del cerebelo en la modulación de las funciones mentales

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

[Partial non convulsive epileptic status as initial presentation of limbic encephalitis]. / Estado epiléptico no convulsivo como forma de presentación de encefalitis límbica.

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.

Neurotropic cutaneous tumors of the head and neck.

OBJECTIVE: To determine the outcomes of patients with neurotropic cutaneous tumors of the head and neck. DESIGN: A retrospective review was conducted of 7852 charts of patients who underwent micrographically controlled excisions of skin cancers of the head and neck between 1984 and 1995, identifying neurotropic tumors and the outcomes of their treatments. SETTING: Tertiary care center (university hospital). PATIENTS: Thirty-seven patients with neurotropic tumors were identified (and confirmed by secondary histological review), constituting 0.47% of all patients. The median age at presentation was 68 years and all except an albino were white. Nine patients had basal cell carcinomas and 28 had squamous cell carcinomas. Twenty-five patients (69%) were referred after at least 1 prior excision was performed a median of 16 months previously. INTERVENTION: All patients underwent micrographic mapping and excision of the cutaneous portion of the tumor. As necessitated by tumor spread, additional soft tissue, skull base, and/or intracranial surgery and postoperative irradiation were also conducted. MAIN OUTCOME MEASURES: Thirty-four patients (3 patients were unavailable for follow-up) were assessed by physician examination a minimum of 19 months after treatment (median, 33 months). RESULTS: Of 25 patients with extracranial disease only, 19 had no evidence of disease during follow-up and 1 died of intercurrent disease at 20 months without evidence of tumor persistence. Of the 9 patients with intracranial neurotropic tumors at the time of presentation, 1 remained with no evidence of disease, 1 died of intercurrent disease at 21 months without evidence of tumor persistence, and the other 7 either died of or are living with an intracranial tumor. CONCLUSIONS: Micrographic tissue mapping to detect and then encompass neurotropic cutaneous malignancies, along with conventional surgery for deeper tumor invasions and irradiation in selected cases, was successful in 19 patients (76%) with an extracranial tumor. For those with neurotropic tumors approaching or penetrating the skull base, the prognosis was poor regardless of therapy method.