RESUMO
Scorpionism is a public health problem in many tropical countries, especially in North Africa, South India, Latin America and the Middle East. In Brazil, patients with severe scorpion envenoming have mainly cardiovascular events, including acute heart failure, acute respiratory distress syndrome and shock, death is rare. We described 58 accidents presumably caused by Tityus obscurus in Brazilian Amazonia. Patients reported a sensation of "electric shocks" which could last hours. The vast majority of patients presented a clinical picture compatible with acute cerebellar dysfunction, beginning minutes and lasting up to 2 days after the accident. They presented cerebellar ataxia, dysdiadochokinesia, dysmetry, dysarthria, dyslalia, nausea and vomiting. Besides, some patients presented myoclonus and fasciculation which can also be attributed to cerebellar dysfunction or maybe the result of direct action on skeletal muscle. Two patients had evidence of intense rhabdomyolysis and acute kidney injury. The clinical picture in this scorpion envenoming is mainly characterized by an acute dysfunction of cerebellar activities and abnormal neuromuscular manifestations and in some cases muscle injury which are not described in any other region of the world. This work presents clinical, epidemiologic, laboratory and treatment aspects of this unmatched scorpion envenoming in the state of Pará, northern Brazil.
Assuntos
Doenças Cerebelares/patologia , Fasciculação/patologia , Mioclonia/patologia , Picadas de Escorpião/complicações , Picadas de Escorpião/epidemiologia , Brasil/epidemiologia , Doenças Cerebelares/etiologia , Criança , Pré-Escolar , Fasciculação/etiologia , Humanos , Masculino , Mioclonia/etiologiaRESUMO
White matter lesions (WML) and epilepsy have been occasionally seen in Wilson's disease. No cases of generalized myoclonus have been reported so far. We present a patient with psychiatric symptoms starting at age 16, followed by tremor, generalized dystonia and severe generalized myoclonus. In addition to classical findings, the MRI showed also extensive WML in temporal, parietal and frontal regions, preserving interhemispheric fibers. Necropsy revealed marked alterations of white matter, cortex and basal ganglia. We subsequently review the literature concerning WML and myoclonus in Wilson's disease.
Assuntos
Córtex Cerebral/patologia , Degeneração Hepatolenticular/complicações , Degeneração Hepatolenticular/patologia , Mioclonia/complicações , Mioclonia/patologia , Adolescente , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Literatura de Revisão como AssuntoRESUMO
We investigated a possible correlation between behavior during status epilepticus (SE) and underlying brain damage. Adult rats were electrically stimulated in the left amygdala to induce SE, which was stopped 2 hours later. We observed two different types of SE: (1) typical SE (TSE), with facial automatisms, neck and forelimb myoclonus, rearing and falling, and tonic-clonic seizures; (2) ambulatory SE (ASE), with facial automatisms, neck myoclonus, and concomitant ambulatory behavior. TSE was behaviorally more severe than ASE (P<0.05). Histology revealed neuronal loss in several brain areas. There was a positive correlation between SE type and amount of injured areas 24 hours and 14 days after SE (P<0.01). The areas more affected were piriform cortex and hippocampal formation. We suggest quality of seizures during SE may be considered in further SE studies, as our results indicate its influence on the severity of brain damage following this paradigm.
Assuntos
Encéfalo/patologia , Estado Epiléptico/patologia , Tonsila do Cerebelo/fisiologia , Animais , Comportamento Animal/fisiologia , Córtex Cerebral/patologia , Estimulação Elétrica , Eletrodos Implantados , Eletroencefalografia , Epilepsia Tônico-Clônica/patologia , Epilepsia Tônico-Clônica/psicologia , Hipocampo/patologia , Masculino , Atividade Motora/fisiologia , Mioclonia/patologia , Mioclonia/psicologia , Ratos , Estado Epiléptico/psicologia , Técnicas EstereotáxicasRESUMO
As doenças degenerativas, como a demência senil, as enfermedades de Alzheimer, Pick e Creutzfeldt-Jakob, dentre outras, despertam interesse crescente entre os clínicos, sendo porém necessária uma visäo realista das mesmas, o que só é possível à luz da patologia
Assuntos
Doenças do Sistema Nervoso Central/patologia , Doença de Charcot-Marie-Tooth/patologia , Demência/patologia , Doença de Alzheimer/patologia , Doença de Parkinson/patologia , Doença de Huntington/patologia , Mioclonia/patologia , Neurodegeneração Associada a Pantotenato-Quinase/patologia , Síndrome de Shy-Drager/patologia , Paralisia Supranuclear Progressiva/patologiaRESUMO
Two cases of Lance-Adams syndrome with anatomopathologic study are reported. There were evidences of diffuse neuronal degeneration in the brain. These changes were most seen in the neurones of the cortical layers, thalamus and subthalamic nuclei. The cells changes were similar to those seen in ischaemic disease. Some neurones showed intracytoplasmatic inclusions staining with the P.A.S. method. These inclusions were readily distinguished from the Lafora bodies.