RESUMO
We report here a 70-year-old woman whose initial clinical presentation suggested corticobasal degeneration, but autopsy revealed dementia with Lewy bodies (DLB) with severe Alzheimer's disease (AD)-type pathology accentuated in the motor cortex, in conjunction with a high burden of both cortical and brain stem LB. Review of the literature disclosed four patients with AD whose peri-Rolandic region was particularly involved by the disease and who exhibited similar clinical and neuropathological findings as in our patient except they lacked LB. It appears that DLB if associated with severe AD-type pathology can, like some unusual cases of AD, mimic corticobasal degeneration.
Assuntos
Doença de Alzheimer/patologia , Corpos de Lewy/patologia , Córtex Motor/patologia , Doenças Neurodegenerativas/patologia , Idoso , Diagnóstico Diferencial , Feminino , Giro do Cíngulo/patologia , Humanos , Emaranhados Neurofibrilares/química , Emaranhados Neurofibrilares/patologia , Proteínas tau/análiseRESUMO
This study presents baseline and 3-month follow-up motor and neuropsychological data for 22 patients with Parkinson's disease (PD) who underwent anatomically guided unilateral posterior ventral pallidotomy (PVP). Postsurgical improvements were seen in psychomotor speed, fine motor accuracy, and dyskinesia, whereas grip strength decreased on the side contralateral to the surgery. No change was detected in overall level of cognitive functioning, nor were changes demonstrated in memory, language, or working memory when the entire sample of patients was evaluated. When the group was divided on the basis of side of surgery, patients with left-sided pallidotomies showed a decline in verbal fluency. Patients and caregivers reported improvement in psychosocial functioning. These initial findings of improved motor performance and largely unaffected cognitive functions are consistent with results obtained with functional PVP and provide support for the use of anatomically guided posterior ventral pallidotomy in the treatment of motor symptoms of PD.
Assuntos
Cognição , Globo Pálido/cirurgia , Destreza Motora , Doença de Parkinson/cirurgia , Idoso , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Parkinson/fisiopatologia , Doença de Parkinson/psicologia , Resultado do TratamentoRESUMO
Cyclosporine has been associated with various neurological side-effects including postural tremor, seizures, headaches, encephalopathy, cortical blindness, and visual hallucinations. We describe here two patients who developed parkinsonism, with rest tremor and bradykinesia, after receiving cyclosporin A following allogeneic bone marrow transplantation. The patients did not have pre-existing neurological disorders, and had not received significant amounts of dopamine-blocking drugs. One patient improved markedly with Sinemet (carbidopa-levodopa), while the other (who did not tolerate Sinemet) improved with decrease in cyclosporine dosage. The relation of the parkinsonian symptoms to cyclosporine therapy and lack of other evident causes for the symptoms, suggests that parkinsonism may be an occasional consequence of cyclosporine.
Assuntos
Ciclosporina/efeitos adversos , Imunossupressores/efeitos adversos , Doença de Parkinson Secundária/induzido quimicamente , Adulto , Transplante de Medula Óssea , Feminino , Humanos , MasculinoAssuntos
Cefaleia/diagnóstico , Pressão Intracraniana , Adulto , Diagnóstico Diferencial , Feminino , Cefaleia/etiologia , HumanosAssuntos
Braço , Músculos Faciais , Perna (Membro) , Esclerose Múltipla/complicações , Espasmo/etiologia , Adulto , Carbamazepina/uso terapêutico , Paralisia Facial/etiologia , Humanos , Hiperventilação/complicações , Hiperventilação/fisiopatologia , Masculino , Esclerose Múltipla/diagnóstico , Esclerose Múltipla/tratamento farmacológico , Esclerose Múltipla/fisiopatologia , Espasmo/diagnóstico , Espasmo/tratamento farmacológico , Espasmo/fisiopatologiaRESUMO
We describe 5 individuals (from three separate families) with a progressive neurological disorder characterized by sensorimotor peripheral polyneuropathy, cranial neuropathies (external ophthalmoplegia, deafness), and the syndrome of chronic intestinal pseudo-obstruction. Magnetic resonance imaging showed widespread abnormality of the cerebral and cerebellar white matter in the 2 patients studied. Autopsy examination in 3 revealed widespread endoneurial fibrosis and demyelination in the peripheral nervous system, possibly secondary to axonal atrophy, and poorly defined changes in cerebral white matter (leukoencephalopathy). The cranial nerves and spinal roots were less severely involved and the neurons in the brainstem and spinal cord were intact. The fatal gastrointestinal dysmotility was due to a severe visceral neuropathy. We suggest that these patients manifested a hereditary disorder with distinctive clinical, radiological, and neuropathological features, and propose the acronym POLIP to emphasize the distinctive tetrad of polyneuropathy, ophthalmoplegia, leukoencephalopathy, and intestinal pseudo-obstruction.
Assuntos
Encefalopatias/complicações , Pseudo-Obstrução Intestinal/complicações , Oftalmoplegia/complicações , Doenças do Sistema Nervoso Periférico/complicações , Adulto , Encéfalo/diagnóstico por imagem , Encefalopatias/diagnóstico por imagem , Encefalopatias/genética , Sistema Digestório/diagnóstico por imagem , Feminino , Humanos , Pseudo-Obstrução Intestinal/genética , Pseudo-Obstrução Intestinal/patologia , Imageamento por Ressonância Magnética , Masculino , Oftalmoplegia/genética , Oftalmoplegia/patologia , Linhagem , Doenças do Sistema Nervoso Periférico/genética , Doenças do Sistema Nervoso Periférico/patologia , Radiografia , SíndromeRESUMO
The neurologic dysfunction in 7 patients treated for small cell carcinoma (SCC) of the lung by combination chemotherapy and prophylactic brain irradiation was evaluated. The disease appeared to be a diffuse encephalopathy frequently affecting the higher cortical functions. Five out of seven patients had progressive dysfunction leading to death in 1 to 26 months; one patient had stabilization of symptoms followed by death in 21 months, probably from the neurologic disease as well as SCC; one patient's symptoms improved. The clinical course of the neurologic disorder seemed different from the known reactions to brain irradiation and from the other neurologic syndromes associated with lung cancer. The relative contributions of cranial irradiation and treatment with chemotherapeutic agents in producing the neurotoxicity are not known. Computed tomographic (CT) brain scans done after the onset of symptoms did not show any focal signs or necrosis. However, there was a suggestion of progressive increase in intracranial fluid volume on the scans. The incidence of the disorder, 10.2% among a group of 49 patients, suggests the need for prospective studies to evaluate the problem.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Encefalopatias/etiologia , Neoplasias Encefálicas/prevenção & controle , Encéfalo/efeitos da radiação , Carcinoma de Células Pequenas/terapia , Neoplasias Pulmonares/terapia , Adulto , Idoso , Carcinoma de Células Pequenas/tratamento farmacológico , Carcinoma de Células Pequenas/radioterapia , Terapia Combinada , Ciclofosfamida/efeitos adversos , Doxorrubicina/efeitos adversos , Etoposídeo/efeitos adversos , Feminino , Humanos , Lomustina/efeitos adversos , Neoplasias Pulmonares/tratamento farmacológico , Neoplasias Pulmonares/radioterapia , Masculino , Pessoa de Meia-Idade , Mitomicina , Mitomicinas/efeitos adversos , Procarbazina/efeitos adversos , Vincristina/efeitos adversosRESUMO
Facial nerve paralysis developed in a man with tumor-stage mycosis fungoides (MF). Mastoidectomy disclosed that MF had involved the mastoid and middle ear. Meningeal lymphoma, confirmed by the finding of Sézary cells in the CSF, was subsequently established. Autopsy disclosed MF lymphoma in the leptomeninges, medulla, spinal cord, and cranial nerves. A unique feature was the formation of a communicating hydrocephalus. Case reports of 23 patients with MF of the CNS, including 21 autopsies, are reviewed. Practically all had tumor-stage or erythrodermic MF. Atypical mononuclear cells were found ante mortem in the CSF in eight patients. In contrast to other CNS lymphomas, bone marrow involvement was uncommon. Cranial, especially facial, nerve paralyses were often premonitory signs of meningeal lymphomas. Patients with MF having such symptoms should have cytologic examination of the CSF.
Assuntos
Neoplasias da Orelha/secundário , Orelha Média/patologia , Processo Mastoide/patologia , Neoplasias Meníngeas/secundário , Micose Fungoide/secundário , Neoplasias Cutâneas/patologia , Adulto , Paralisia Facial/etiologia , Humanos , Hidrocefalia/etiologia , Masculino , Meninges/patologia , Meningite/etiologia , Micose Fungoide/complicações , Micose Fungoide/patologia , Neoplasias Cutâneas/complicaçõesRESUMO
The first case of pure primary prostatic carcinoid is presented. Previous demonstration of the presence of enterochromaffin cells in the non-neoplastic prostate and in otherwise ordinary adenocarcinomas of the prostate furnishes a basis for the origin of this unique neoplasm.