1/4. Vialetto-Van Laere syndrome in two sisters born to consanguineous parents.Vialetto-Van Laere syndrome is a rare anterior horn cell disease affecting young persons. Bulbar palsy with deafness is the characteristic feature. Of the total 35 cases reported in the literature, only one case was from india. This article presents the second case report from india.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
2/4. Neoplastic obstruction of the trachea complicating pseudobulbar palsy.We have reported a case of pseudobulbar palsy with airflow obstruction initially attributed to chronic aspiration and chronic airflow obstruction caused by cigarette smoking. The finding of stridor led to a detailed evaluation of the obstruction. Flow-volume loops, fiberoptic bronchoscopy, CT scan of the upper thorax, and fine-needle biopsy of a lesion of the trachea were required to make the diagnosis of non-small-cell carcinoma involving the trachea.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
3/4. Cerebello-brain stem orthochromatic leukodystrophy with floppiness and bulbar paralysis.A two year and ten-month-old girl with cerebello-brain stem leukodystrophy is presented. She was hypotonic with spasticity and showed delayed motor development until she was twelve months old, when regression of motor activities began. Progressive hypotonia, dysphagia and fatal respiratory abnormalities developed. Auditory brain stem response was absent. Pathological examination revealed primary demyelination with a predilection for the cerebellum, brain stem and spinal cord, in association with changes of the cerebellar cortex, pontine nuclei, inferior olivary nuclei and part of the basal ganglia. Clinical, pathological and biochemical studies revealed that this disease is different from metachromatic leukodystrophy, Krabbe's globoid cell leukodystrophy and adrenoleukodystrophy. The clinical entity of cerebello-brain stem orthochromatic leukodystrophy is discussed.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
4/4. Infantile onset of hereditary ascending spastic paralysis with bulbar involvement.Primary lateral sclerosis and hereditary spastic paraparesis are both rare neurodegenerative disorders characterized by progressive weakness and spasticity of the lower limbs, with involvement of the corticospinal tracts and sparing of anterior horn cells. We describe a consanguineous family in which three sons developed progressive paralysis of the lower extremities in infancy with subsequent involvement of the upper extremities and bulbar muscles but cognitive sparing. This family presents the nosologic difficulty of distinguishing between hereditary spastic paraparesis and primary lateral sclerosis. We suggest that the diagnosis in this family is hereditary primary lateral sclerosis. This is the first instance of familial occurrence of primary lateral sclerosis.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |