Multiple meningiomas within the spinal canal: case report with 23 tumors

Na maioria das casuísticas de neoplasias do canal espinhal, medula espinhal e leptomeninges, os meningiomas compreendem 25%. A incidência de meningiomas múltiplos é pequena quando comparada com sua freqüência isolada. Na maioria dos casos, a ocorrência múltipla é intracraniana e espinhal. Meningiomas múltiplos localizados exclusivamente no canal raqueano são extremamente raros. Relatamos o caso de homem de 33 anos com 23 tumores, localizados na região torácica espinhal.

Nightmares without atonia as an early symptom of diffuse Lewy bodies disease

A male 70 years old patient with diffuse or ''pure'' Lewy body disease is described. The diagnosis was made based on clinical features of nightmares with no atonia, attention deficits with fluctuation in cognitive function, incapacity to find his way around the neighbourhood and other formerly familiar environments and mild neuropsychiatric symptoms. Neuropsychological assessment showed memory deficits, visuospatial and visuo-constructive disturbances. He had neither parkinsonism nor recurrent visual hallucinations typically well formed and detailled. Neuroimaging (computed tomography and magnetic resonance spectroscopy) showed mild diffuse cortical atrophy, mostly on the left temporal lobe and a decrease of N-acetil-aspartate levels. A cholinesterase inhibitor was prescribed to this patient during 6 months with clinically relevant behavioral effect. Diagnosis confirmation was made by post-mortem neuropathological findings. Macroscopical features were mild atrophy on the frontal, parietal and temporal lobes, notedly on the frontal lobes. Microscopically, there was neuronal loss and diffuse classic Lewy bodies. Brainstem (substantia nigra, raphe nucleus, locus coeruleus, pedunculopontine nucleus), limbic cortex, and neocortex (frontal, parietal and temporal) were the areas of predilection for Lewy bodies. Hematoxylin-eosin and Bielschowsky staining did not show neuronal swelling (balooned cell), argyrophilic inclusion (Pick's bodies), neurofibrillary tangles nor senile plaques. Immunohistochemical staining for anti-tau, anti-beta-amyloid, and anti-prion protein were negative. Antiubiquitine reaction was positive for Lewy body in the cerebral cortex and brainstem

Frontoparietal cortical atrophy with gliosis in the gray matter of cerebral cortex: case report

The case of a patient who suffered from progressive amnesia, depressive humor, language and visuospatial disturbances, and hallucination episodies with interference at the daily living activities is reported. She had moderate neuropsichological diffuse deficits at the first examination, especially at the executive and visuo-constructive functions. Her cerebrospinal fluid test presented high total protein. Magnetic resonance image showed slight white matter increase in periventricular, semi-oval center bilateral and left external capsule regions, besides light frontal and parietal lobe atrophy, bilaterally. Brain single photon emission computerized tomography revealed both a bilateral moderate frontal and a severe parietal lobe hypoperfusion, especially on the left side. Macroscopic examination showed cortical atrophy, severe on the frontal, moderate on the parietal and mild on the posterior third temporal lobes, bilaterally. There was a slight atrophy on the neostriatum in the basal ganglia. The histopathological findings of the autopsy showed severe neuronal loss with intensive gemioscytic gliosis and variable degrees of status spongiosus in cortical layer. Hematoxylin-eosin and Bielschowsky staining did not show neuronal swelling (balooned cell), argyrophilic inclusion (Pick's bodies), neurofibrillary tangles nor senile plaques. Immunohistochemical staining for anti-ubiquitin, anti-tau, anti-beta-amyloide, and anti-prion protein were tested negative

Classic Pick's disease type with ubiquitin-positive and tau-negative inclusions: case report

We report on a patient presenting Pick's disease similar to the one reported by Pick in 1892, with ubiquitin-positive and tau-negative inclusions. His diagnosis was made on the basis of clinical (language disturbance and behavioural disorders), neuropsychological (progressive aphasia of the expression type and late mutism), neuroimaging with magnetic resonance (bilateral frontal and temporal lobes atrophy) and brain single photon emission computed tomography (frontal and temporal lobes hypoperfusion) studies. Macroscopic examination showed atrophy on the frontal and temporal lobes. The left hippocampus displayed a major circumscribed atrophy. The diagnostic confirmation was made by the neuropathological findings of the autopsy that showed neuronal loss with gliosis of the adjacent white matter and apearence of status spongiosus in the middle frontal and especially in the upper temporal lobes. There were also neuronal swelling (ballooned cell) and argyrophilic inclusions (Pick's bodies) in the left and right hippocampi. Anti-ubiquitin reaction tested positive and anti-tau tested negative

Hypothalamic pilocytic astrocytoma and subarachnoid hemorrhage: case report

In this report, we present a case of a young women with a pilocytic astrocytoma in the chiasmo-hypothalamic region, in which the clinical presentation was subarachnoid hemorrhage

Amyotropic lateral sclerosis with dementia: case report

A patients 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.

Doença de Binswanger: estudo anátomo-clínico de um caso / Binswanger's disease: anatomoclinical study of a case

Descreve-se o caso de uma paciente com doença de Binswanger e que foi acompanhada durante 10 anos, antes e depois da síndrome demencial. O diagnóstico clínico ficou estabelecido pela história, exames clínico e neurológico, e avaliaçäo neuropsicológica. As imagens de tomografia cerebral computadorizada e, principalmente, de ressonância magnética do crânio sugeriram o diagnóstico, o qual foi confirmado pelo estudo anßtomo-patológico. Comentam-se os dados clínicos e comportamentais, aspectos de ordem radiológica e histopatológica, confrontando-os à literatura.

Doença de Erdheim: relato de um caso / Erdheim`s Disease: report of a case

Os autores relatam um caso da doença de Erdheim, uma degeneraçäo das estruturas que formam a túnica média da aorta, conduzindo habitualmente a uma aneurisma dissecante e hemorragia para o interior de cavidades do corpo. Este caso mostra aspectos clínicos e patológicos, incomuns nesta doença