Amyotrophic lateral sclerosis with dementia. Case report.

A patient is described in whom a profound and rapidly progressive dementia occurred in association with clinical features of amyotrophic lateral sclerosis. A magnetic resonance imaging showed signs of frontal and especially left temporal atrophy. The pattern of dementia indicated impaired frontotemporal lobe functions, evidenced by reduced tracer uptake in the frontotemporal lobes on brain single photon emission computed tomography. Neuropathological examination in this patient revealed mild frontotemporal atrophy with spongiform changes and neuronal loss affecting mainly layers II and III of the frontotemporal cortices. There was atrophy of the hypoglossal nuclei. The spinal cord changes were consistent with motor neuron disease. The patient showed an irreversible and progressive course. A review of the relevant literature was made.

[Binswanger's disease: clinicopathological study of a case]. / Doença de Binswanger. Estudo anátomo-clínico de um caso.

The authors describe a case of Binswanger's disease in a female patient with arterial hypertension followed during 10 years, before and after the demential syndrome. The clinical diagnosis was established through history, clinical and neurological examination, and neuropsychologic evaluation. Computed tomography and particularly magnetic resonance imaging suggested the diagnosis, that was confirmed by the anatomo-pathological study. They discuss behavioral and clinical data, and radiological and histopathologic aspects, comparing them with data found in the literature.

[Cognitive heterogeneity in Alzheimer's disease]. / Heterogeneidade cognitiva na doença de Alzheimer.

The authors describe and discuss neuropsychological aspects related to the functional organization of the brain connected to cognition in 81 Alzheimer's disease patients in the Laboratoire Théophile-Alajouanine du Centre de Recherche du Centre hospitalier Côte-des-Neiges in the University of Montreal. They confront the clinical of homogeneity against the heterogeneity within functional organization of the brain. The criteria used for diagnosis were those of the NINCDS-ADRDA. All of the patients were in the beginning of the evolution, in stages 3 and 4 of Reiberg (Functional Assessment Staging, FAST). The conclusion shows that there are two cognitive profiles: a non-recurrent profile, made up by the majority of the patients, and the heterogeneity depends on the genetic background of each individual; a recurrent profile, made up by a small group of patients that show changes in the heterogeneity of clinical, nosological, pathological and normal aging forms.

[Adequate neuropsychological evaluation of dementia]. / Avaliação neuropsicológica adequada às demências.

The authors discuss the protocol of the Optimal Neuropsychological Evaluation of Dementias of Montreal and its possible application in Brazil. This protocol is important for evaluation in neuropsychological evolution of dementia of the Alzheimer type and for bringing to the fore distinct affected cognitive profiles as much on a transversal base as longitudinal. The authors believe that its application would contribute to the progress of research in neurology, as in the clear distinction between normal aging and pathological aging.

[Back lumbar herniated discs and cauda equina syndrome as complications of Scheuermann's disease. A case report]. / Hérnias discais dorso-lombares e síndrome da cauda equina como complicações da doença de Scheuermann. Relato de caso.

The author reports the case of a patient with spinal cord compression by a thoracic herniated disc without paraplegia as well as a cauda equina syndrome due to spondylotic and discal lesions, with small protrusions of the L4-L5 and L5-S1 discs associated with Scheuermann disease. There was complete recovery in six months with clinical treatment. The radiological evaluation using standard radiography, myelography, computed tomography, magnetic resonance imaging of the vertebral column, and electromyographic evaluation indicate a precise correlation between clinical picture and the distribution and course of lumbar spinal cord degenerations. Furthermore, a strong correlation was also found with hernias, roots and the bony sequalae of spinal cord dystrophy found in Scheuermann disease.

[Adrenoleukodystrophy: clinical and histopathological study of a case associated to the use of abortifacient agents in the second month of pregnancy]. / Adrenoleucodistrofia. Estudo clínico e histopatológico de um caso associado ao uso de abortivos no segundo mês de gestação.

The authors report a case of adrenoleukodystrophy in a 8 years old male patient whose mother has taken several abortive drugs during the first three months of pregnancy. The disease was progressive starting with auditory, visual and mental disturbances, followed by neurovegetatives and motor changes with convulsion and fetal position. At the final stage the patient became blind, deaf, quadriplegic and dementiated. Death resulted from lung infection. The diagnosis was confirmed by the CSF, electrophysiological, radiological and necropsy findings. Necropsy changes in the brain and adrenal cortex are detailed.