Basal ganglia involvement in herpes simplex encephalitis.

Herpes simplex encephalitis (HSE) is a leading cause of sporadic, nonepidemic viral encephalitis in children and adults. We report a very rare case of HSE with involvement of bilateral thalamus, putamen, upper pons and midbrain, with development of extrapyramidal symptoms which responded to corticosteroid therapy. A 15-mth-old female baby admitted with complaint of fever for 5 days and generalised tonic clonic seizure 10 hours before admission. On clinical examination patient was drowsy, temperature was 39.4 oC and vitals were stable with signs of increased intracranial tension. There were no signs of meningeal irritation. Patient gradually become unconscious in the next few hours and pupils were constricted bilaterally with development of atonia in all four limbs and neck muscles. Doll’s eye phenomenon was absent.

Biotin responsive limb weakness.

We report acute onset quadriplegia in a ten year old boy associated with basal ganglia lesions due to biotin deficiency. Prolonged raw egg consumption was identified as predisposing factor for biotin deficiency. Treatment with biotin resulted in remarkable recovery.

Alterações comportamentais graves em paciente com doença de Fahr / Severe behavioral changes in a patient with Fahr's disease / Severe behavioral changes in a patient with Fahr's disease

Relatamos o caso de um homem de 40 anos de idade com doença de Fahr, definida por calcificações idiopáticas bilaterais em núcleos da base, que desenvolveu transtorno depressivo, tiques motores e fônicos, comportamentos estereotipados tipo "punding" e alterações de personalidade com importantes implicações sócio-familiares. Discutimos as manifestações psicopatológicas da doença de Fahr e a importância dos núcleos da base na determinação do comportamento humano.

We report on a case of a 40 year-old man with Fahr's disease, defined by idiopathic bilateral basal ganglia calcification, who developed depressive disorder, motor and phonic tics, stereotyped behaviors such as punding and personality changes with significant social and familiar implications. We discuss about the psychopathology of FahrÆs disease and the relevance of the basal ganglia in the determination of humanÆs behavior.

Is Parkinson's disease a homogeneous disorder--what is the burden of Parkinson's disease in India.

Movement disorders are common neurological illnesses among the elderly. These include essential tremor, Perkinsonian disorders and chorea of different aetiologies. Parkinsonian disorders can be divided into two major groups of disorders--classical idiopathic Parkinson's disease and Parkinson plus syndrome. The most common and important cause of Parkinsonism is idiopathic Parkinson's disease. Idiopathic Parkinson's disease is most confidently clinically diagnosed if we follow the United Kingdom Parkinson's Disease Society Brain Bank Diagnostic Criteria for Parkinson's disease. The most common degnerative diseases, which minic idiopathic Parkinson's disease are collectively called Parkinson plus syndrome. The most important diseases comprising Parkinson plus syndrome are: progressive supranuclear palsy, multiple system atrophy, cortical-basal ganglionic degneration, diffuse Lewy body disease and Parkinson-dementia-ALS complex. In India the prevalence of Parkinson's disease varied markedly from one study to another. The prevalence rate is high among the urban Parsi community of Mumbai. Incidence and prevalence of Parkinson's disease increase with increasing age. Some risk factors for Parkinson's disease have been narrated briefly. As the number of cases of Parkinsonism is likely to increase along with increasing population, the general practitioners or consultant physicans should have to play a greater role referring the cases to attend neurologists or movement disorder clinic early.

Vitamin B12 deficiency presenting with an acute reversible extrapyramidal syndrome.

Vitamin B12 deficiency usually presents with pernicious anemia or various neuropsychiatric manifestations. Commonly seen neuropsychiatric manifestations include large fiber neuropathy, myelopathy (subacute combined degeneration of the spinal cord), dementia, cerebellar ataxia, optic atrophy, psychosis and mood disorders. The present report highlights an unusual presentation of vitamin B12 deficiency- acute onset extrapyramidal syndrome in a 55-year-old man. The patient presented with a 10-day history of slowness of all activities including a slow gait, mild tremors of hands and low volume speech. On examination, he had features of mask-like facies, reduced blink rate and cogwheel rigidity. He was investigated for the possible causes and was found to have laboratory features of vitamin B12 deficiency. Other causes for acute onset parkinsonism were excluded by appropriate investigations. He showed a dramatic improvement following treatment with intramuscular vitamin B12 injections. At a five-year follow up, he was found to be functionally independent with no neurological deficits.

Germinoma of the basal ganglia: a case report and review of literature.

Germs cell tumors of extrapineal region are extremely uncommon. The clinicopathological features of germinoma in the basal ganglia, in a 21 year old male are presented in this report. Post-operative radiotherapy is advocated, in view of the good radiosensitivity of these tumors.

Involuntary hand levitation associated with parietal damage: another alien hand syndrome

The alien hand syndrome (AHS) usually consists of an autonomous motor activity perceived as an involuntary and purposeful movement, with a feeling of foreignness of the involved limb, commonly associated with a failure to recognise ownership of the limb in the absence of visual clues. It has been described in association to lesions of the frontal lobes and corpus callosum. However, parietal damage can promote an involuntary, but purposeless, hand levitation, which, sometimes, resembles AHS. In the present study, four patients (cortico-basal ganglionic degeneration -- n=2; Alzheimer's disease -- n=1 and parietal stroke -- n=1) who developed alien hand motor behaviour and whose CT, MRI and/or SPECT have disclosed a major contralateral parietal damage or dysfunction are described. These results reinforce the idea that parietal lobe lesions may also play a role in some patients with purposeless involuntary limb levitation, which is different from the classic forms of AHS

Degeneraçäo lobar frontotemporal: aspectos neuropsiquiátricos, diagnóstico, diagnóstico diferencial / Frontotemporal lobar degeneration neuropsychiatric: aspects, diagnosis, differential diagnosis

As síndromes clínicas associadas com degeneraçäo lobar frontotemporal (DLFT) apresentam como principal sintomatologia os distúrbios de comportamento (desinibiçäo, apatia, esterotipias), psicológicos (delírios, alucinaçöes, distúrbios afetivos) e de linguagem precedendo o comportamento cognitivo. A neuroimagem é característica com atrofia e hipoperfusäo nos lobos frontais e temporais, sem alteraçöes eletroencefalográficas. Evidência recentes demonstram que as síndromes da DLFT podem ser diagnosticadas em vida utilizando-se parâmetros clínicos e de neuroimagem, uma vez que estes säo característicos. Esta constataçäo direcionou esforços na elaboraçäo de critérios diagnósticos operacionais que permitem a orientaçäo clínica e facilitam a comunicaçäo entre pesquisadores. A DLFT deve ser diferenciada de outras demências como a doença de Alzheimer, demência com corpos de Lewy e demências vasculares e distúrbios psiquiátricos como desordem do humor, quadros psicóticos e distúrbios de personalidade

Intoxicación aguda por paratión-metílico con manifestaciones extrapiramidales antes no informadas / Acute poisoning by methyl parathion with previously unreported extrapyramidal manifestations

Femenina de 14 años de edad que intenta suicidio mediante la ingestión de insecticida paratión-metílico concentrado, quien presentó intoxicación aguda grave con manifestaciones muscarínicas, nicotínicas y neurológicas, así como descenso de 88 por ciento de la actividad de la colinesterasa. Al noveno día de su evolución, súbitamente presentó síntomas extrapiramidales, crisis oculógiras y bucolinguales, distonía de torsión de cuello y tronco, hipertonía y temblores digitales; mismos que mejoraron con la administración endovenosa de difenhidramina. Se descartó la administración de neurolépticos, la exposición a otros tóxicos capaces de originar esta sintomatología y la posibilidad de un síndrome intermedio por orgánico-fosforados. Se especula, dadas las propiedades bioquímicas del insecticida, que pudiera causar esta sintomatología al inhibir los receptores dopaminérgicos de la sustancia nigra y de los ganglios basales

Discinesia coreiforme asociada a broncodisplasia pulmonar / Bronchopulmonary dysplasia associated chorea like motor disorder. A case report

Introducción. La broncodisplasia pulmonar es una complicación frecuente del recién nacido prematuro que desarrolla enfermedad de membrana hialina; su repercusión sobre el neurodesarrollo incluye: alteraciones motoras, retardo en la adquisición de habilidades lingüísticas y microcefalia; recientemente se ha relacionado con el desarrollo de movimientos involuntarios de tipo coreico y temblor de origen extrapiramidal durante el primer año de vida. Caso clínico. Se reporta un caso típico de discinesia con movimientos coreicos, temblor, postura distónica y dificultades en la succión y deglución asociadas a disquinesia oro-buco-lingual que se desarrolló en los primeros 3 meses de vida en un paciente con broncodisplasia pulmonar y dependencia de suplementos de oxígeno inhalado. Conclusión. Se hace hincapié en el diagnóstico diferencial de los trastornos de la succión y deglución, los hallazgos video-electroencefalográficos y la necesidad de reconocer correctamente el síndrome, así como su tratamiento farmacológico

Pseudohypoparathyroidism-Albright hereditary osteodystrophy.

A 13 year old girl with short stature, and retarded mental growth with coarse facies and deranged thyroid function test was initially suspected as a case of hypothyroidism and was started on thyroxine. Lack of response to treatment and on further investigations it was diagnosed as a case of pseudohypoparathyroidism. High index of suspicion and careful evaluation is important to diagnose such an entity.

Biopsia de ganglio linfático: comparación de la biopsia por aguja fina y excisional / Biopsia of linphatic ganglion: the fine needle biopsy and abscissional comparation

Entre septiembre de 1996 y octubre de 1997, se evaluaron a 31 pacientes con síndrome adenomegálico de etiología desconocida, que acudieron o fueron referidos al Servicio de Cirugía II, HUC.La relación hombres mujeres: fue de 2.875:1, con una media edad de 39.8 años. A estos se les realizó biopsia por aspiración con aguja fina (BAAF) y biopsia excesional (BE) del mismo ganglio linfático (GL). 72.42 de citologías adecuadas, con sensibilidad, especificidad, valores predictivos positivos y negativos y exactitud global de 64.285 100, 100, 66.55 y 79.19, respectivamente. Se concluye que la BAAF del GL es un procedimiento sencillo no asociado a morbilidad, con una exactitud global, sensibilidad, especificidad y valores predictivos buenos; y a pesar de no llegar al diagnóstico específico en los linfomas, sirve para distinguir los estudios diagnósticos sucesivos, incluyendo la BE

Demência e calcificaçao dos núcleos da base / Dementia and calcifications of the basal ganglia

O presente trabalho visa relatar 3 casos de pacientes com calcificações intracranianas, tendo em comum extensas calcificações dos núcleos da base e disfunção paratireoidiana. O primeiro paciente apresentava hipoparatireoidismo pós-cirúrgico (HPC), outro pseudo-hipoparatireoidismo (PHP) e o terceiro hipoparatireoidismo idiopático (provável) (HPI). Os pacientes foram avaliados através de exames neurológico, radiológico e laboratoriais (Ca, P, PTH) e submetidos a testagem neuropsicológica. O paciente com HPC apresentava síndrome cerebelar, o com PHP relato de crises convulsivas e o com HPI síndrome extrapiramidal. Os 3 apresentavam comprometimento cognitivo relevante (como atenção/concentração, memória, planejamento, abstração, coordenação visual-motora, flexibilidade cognitiva), com padrão sugestivo de demência subcortial. Confirma-se a necessidade de avaliação laboratorial nos casos de calcificações intracranianas para rastrear disfunção paratireoidiana. Ressalta-se a importância de avaliação neuropsicológica sistematizada, tendo em vista o papel dos núcleos da base nos circuitos relacionados aos processos cognitivos

Bilateral basal ganglia lucencies following acute febrile illness.

Bilateral striatal necrosis in children without damage elsewhere in the brain can present as an acute neurological disorder or as a progressive disorder. Three children of 6, 7 and 12 years age developed dystonic posture of limbs without any cranial nerve involvement or alteration of sensorium soon after recovery from acute high grade febrile illness of 3-4 days duration. Computerized tomographic scan of head showed bilateral necrosis of basal ganglia. We think that these patients probably constitute a clinically and radiologically distinct subgroup of disorder that produce bilateral striatal necrosis in children. The cause of the syndrome is unknown.

Disfunción neurológica aguda asociada a lesiones estriatales bilaterales en la infancia / Acute neurological dysfunction associated with bilateral striatal lesions in childhood

La disfunción neurológica aguda por daño a nivel del estriado en la infancia puede presentarse con diferentes formas clínica y en una amplia variedad de patología con diferentes pronósticos. Se presentan dos pacientes, y se revisa y discute la literatura