RESUMO
In the 90's, clinico pathological studies have considerably improved the diagnosis of specific and rare neurodegenerative diseases. After a training in Parkinsons' disease in Paris, the author moved to French West Indies (Guadeloupe) and observed a high incidence of atypical parkinsonism with dementia, unresponsive to levodopa. Similar features were observed in Martinique. An environmental origin has been suspected with the exposure to toxins of annonaceae leaves and seeds. The candidate toxins are acetogenins acting as mitochondrial poison. This was demonstrated in neuronal cell cultures, and in animals. However, the agency for food security did not conclude that Annonaceae should not be used for herbal (medicinal) tea, even if the population is now aware about the possible risk of parkinsonism after exposure to annonaceae acetogenins.
Assuntos
Annonaceae/química , Demência , Alimentos/toxicidade , Transtornos Parkinsonianos , Chás de Ervas/toxicidade , Região do Caribe/epidemiologia , Demência/complicações , Demência/epidemiologia , Demência/etiologia , Resistência a Medicamentos , Guadalupe/epidemiologia , Humanos , Levodopa/uso terapêutico , Martinica/epidemiologia , Doença de Parkinson/classificação , Doença de Parkinson/epidemiologia , Doença de Parkinson/etiologia , Transtornos Parkinsonianos/complicações , Transtornos Parkinsonianos/epidemiologia , Transtornos Parkinsonianos/etiologia , Índias Ocidentais/epidemiologiaRESUMO
BACKGROUND AND PURPOSE: Accumulation of iron (Fe) is often detected in brains of people suffering from neurodegenerative diseases. However, no studies have compared the Fe load between these disease entities. The present study investigates by T2*-weighted gradient-echo 7.0 T magnetic resonance imaging (MRI) the Fe content in post-mortem brains with different neurodegenerative and cerebrovascular diseases. METHODS: One hundred and fifty-two post-mortem brains, composed of 46 with Alzheimer's disease (AD), 37 with frontotemporal lobar degeneration (FTLD), 11 with amyotrophic lateral sclerosis, 13 with Lewy body disease, 14 with progressive supranuclear palsy, 16 with vascular dementia (VaD) and 15 controls without a brain disease, were examined. The Fe load was determined semi-quantitatively on T2*-weighted MRI serial brain sections in the claustrum, caudate nucleus, putamen, globus pallidus, thalamus, subthalamic nucleus, hippocampus, mamillary body, lateral geniculate body, red nucleus, substantia nigra and dentate nucleus. The disease diagnosis was made on subsequent neuropathological examination. RESULTS: The Fe load was significantly increased in the claustrum, caudate nucleus and putamen of FTLD brains and to a lesser degree in the globus pallidus, thalamus and subthalamic nucleus. In the other neurodegenerative diseases no Fe accumulation was observed, except for a mild increase in the caudate nucleus of AD brains. In VaD brains no Fe increase was detected. CONCLUSIONS: Only FTLD displays a significant Fe load, suggesting that impaired Fe homeostasis plays an important role in the pathogenesis of this heterogeneous disease entity.
Assuntos
Encéfalo/metabolismo , Transtornos Cerebrovasculares/metabolismo , Imageamento por Ressonância Magnética/métodos , Doenças Neurodegenerativas/metabolismo , Idoso , Idoso de 80 Anos ou mais , Encéfalo/patologia , Transtornos Cerebrovasculares/patologia , Feminino , Humanos , Imageamento por Ressonância Magnética/instrumentação , Masculino , Pessoa de Meia-Idade , Doenças Neurodegenerativas/patologiaRESUMO
BACKGROUND: Superficial siderosis (SS) is a rare finding on T2*-weighted magnetic resonance imaging (MRI), regarded as a radiological marker of cerebral amyloid angiopathy (CAA). The present study investigates with 7.0-tesla MRI the prevalence of SS and its underlying pathological substrate in a consecutive series of post-mortem brains of elderly patients with various neurodegenerative and cerebrovascular lesions. MATERIALS AND METHODS: The prevalence of SS and associated lesions was screened using 7.0-tesla MRI and their neuropathological correlates in 120 post-mortem brains of patients with various neurodegenerative and cerebrovascular diseases. RESULTS: Eighty-three separate zones of SS were detected in 45 brains (37.5%), including 25 areas of disseminated SS (dSS) and 58 areas of focal SS (fSS), restricted to less than 3 sulci. dSS was spatially related to sequels of 14 lobar haematomas and 11 cerebral infarcts, while fSS was connected to 19 microbleeds and 39 micro-infarcts (p < 0.001). Comparison of the 15 CAA to the 30 non-CAA brains showed that dSS was due to an old lobar haematoma in 53% of the former group compared to 3% of the latter group (p = 0.003). fSS was due to a microbleed in 7% of the CAA brains and to 40% of the non-CAA brains (p = 0.03). CONCLUSIONS: SS is associated with both haemorrhagic and ischaemic underlying lesions. It is frequently observed on T2*-weighted 7.0-tesla MRI, and two types of SS may be described. Clinicians should keep in mind that SS may be found in other settings than CAA.
Assuntos
Sistema Nervoso Central/patologia , Angiopatia Amiloide Cerebral/patologia , Hemorragia Cerebral/patologia , Imageamento por Ressonância Magnética , Doenças do Sistema Nervoso/patologia , Siderose/patologia , Idoso , Idoso de 80 Anos ou mais , Autopsia/instrumentação , Feminino , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Doenças do Sistema Nervoso/diagnóstico por imagem , Prevalência , RadiografiaRESUMO
Tauopathies with parkinsonism represent a spectrum of disease entities unified by the pathologic accumulation of hyperphosphorylated tau protein fragments within the central nervous system. These pathologic characteristics suggest shared pathogenetic pathways and possible molecular targets for disease-modifying therapeutic interventions. Natural history studies, for instance, in progressive supranuclear palsy, frontotemporal dementia with parkinsonism linked to chromosome 17, corticobasal degeneration, and Niemann-Pick disease type C as well as in amyotrophic lateral sclerosis/Parkinson-dementia complex permit clinical characterization of the disease phenotypes and are crucial to the development and validation of biological markers for differential diagnostics and disease monitoring, for example, by use of neuroimaging or proteomic approaches. The wide pathologic and clinical spectrum of the tauopathies with parkinsonism is reviewed in this article, and perspectives on future advances in the understanding of the pathogenesis are given, together with potential therapeutic strategies.
Assuntos
Transtornos Parkinsonianos/complicações , Tauopatias/complicações , Animais , Biomarcadores , Demência/complicações , Demência/genética , Demência/fisiopatologia , Desenho de Fármacos , Geografia , Humanos , Serina-Treonina Proteína Quinase-2 com Repetições Ricas em Leucina , Modelos Biológicos , Mutação , Doença de Niemann-Pick Tipo C/complicações , Doença de Niemann-Pick Tipo C/diagnóstico , Doença de Niemann-Pick Tipo C/fisiopatologia , Doença de Parkinson Pós-Encefalítica/complicações , Doença de Parkinson Pós-Encefalítica/fisiopatologia , Transtornos Parkinsonianos/patologia , Transtornos Parkinsonianos/fisiopatologia , Transtornos Parkinsonianos/terapia , Doença de Pick/complicações , Doença de Pick/patologia , Proteínas Serina-Treonina Quinases/genética , Paralisia Supranuclear Progressiva/complicações , Paralisia Supranuclear Progressiva/diagnóstico , Paralisia Supranuclear Progressiva/fisiopatologia , Tauopatias/patologia , Tauopatias/fisiopatologia , Tauopatias/terapia , Proteínas tau/genéticaRESUMO
The emergence of multiple sclerosis in island societies has been investigated only in a few Caucasian populations living in temperate regions. The effect of human migration on the risk of developing this disease is still an open question because of possible genetic selection. We conducted an epidemiological study of the multiple sclerosis population in the French West Indies (Martinique and Guadeloupe), a population which includes large numbers of West Indians who have returned after emigrating to metropolitan France. Standardized incidence ratios (SIRs) for multiple sclerosis among migrants were calculated and their genetic characteristics were compared to those of non-migrants. The crude prevalence of multiple sclerosis was 14.8/10(5) on December 31, 1999 (95% CI: 11.9-17.7); and its crude mean annual incidence for the period July 1, 1999 to June 30, 2002 was 1.4/10(5) (95% CI: 1.0-1.8), confirming its emergence in the French West Indies. Recurrent neuromyelitis optica, which is virtually the only form of multiple sclerosis in black African populations in tropical regions, represented not >17.8% of these cases. During the 1,440,000 person-years of follow-up, 33 incidence cases were identified in migrants. Since the number of expected cases was 19.3, the overall SIR was 1.71 (95% CI: 1.19-2.38; P < 0.01) among migrants. The increase in the SIR was more marked if the stay was made before the age of 15 years (4.05, 95% CI: 2.17-6.83; P < 0.0001). European ancestry in the two migrating and non-migrating populations was similar. Martinique, which has a higher rate of return migration, has a higher prevalence of multiple sclerosis (21.0/10(5) versus 8.5/10(5)) and a higher incidence (2.0/10(5) versus 0.7/10(5)) than Guadeloupe. The emergence of the disease in the French West Indies is of environmental rather than genetic origin. It may be explained either through the introduction by migrants of precipitating environmental factors that operate in a critical way before the age of 15 years, and/or by the recent disappearance from the French West Indies of protective environmental factors acting before this age.
Assuntos
Emigração e Imigração , Esclerose Múltipla/epidemiologia , Adolescente , Adulto , Fatores Etários , População Negra , Meio Ambiente , Métodos Epidemiológicos , Feminino , França , Guadalupe/epidemiologia , Humanos , Masculino , Martinica/epidemiologia , Pessoa de Meia-Idade , Esclerose Múltipla/etnologia , Esclerose Múltipla/genética , Classe Social , UrbanizaçãoRESUMO
INTRODUCTION: Neuromyelitis optica (NMO) is a rare inflammatory and demyelinating disorder of the central nervous system, restricted to optical nerves and spinal cord. The main neuroradiological aspects, now summarized into a complete set of diagnosis criteria, are a normal cerebral MRI at onset and longitudinal involvement of the spinal cord concerning more than 3 vertebral segments. The clinical course and frequency of typical lesions remain unknown. OBJECTIVE: We here report neuroradiological data from patients suffering from NMO. METHODS: Brain and spinal cord MRI were systematically reviewed for 32 afro-Caribbean patients. RESULTS: A typical longitudinal spinal lesion was seen in 44.7 percent with or without edema; a lesion involving less than 3 vertebral segments in 26.3 percent and no lesion in 21.1 percent. Longitudinal study of a few bouts suggested a progressive normalisation of spinal cord appearance. Atrophy was negatively correlated with immunosuppressive treatment. Cerebral lesions usually absent at onset were correlated to the follow-up. In a non-recursive condition, patients completed diagnostic criteria for encephalic and spinal lesions in 82.8 percent and 48.1 percent. CONCLUSION: Radiology of spinal bouts showed multiple aspects besides the typical form. The notion of multiple bouts must be added to the spinal criteria to achieve good sensitivity. A typical extensive spinal lesion is usual in the follow-up, but seen after less then half of the bouts. Requiring such a lesion would delay the diagnosis.
Assuntos
Imageamento por Ressonância Magnética , Neuromielite Óptica/diagnóstico , Nervo Óptico/patologia , Medula Espinal/patologia , Doença Aguda , Adulto , Feminino , Humanos , Imunossupressores/uso terapêutico , Estudos Longitudinais , Masculino , Exame Neurológico , Neuromielite Óptica/tratamento farmacológico , Neuromielite Óptica/imunologia , Testes Neuropsicológicos , Nervo Óptico/imunologia , Estatística como Assunto , Siringomielia/diagnóstico , Siringomielia/tratamento farmacológico , Siringomielia/imunologia , Resultado do TratamentoRESUMO
INTRODUCTION: Behavioral symptoms are common in dementia, and seem to be more frequent in men than in women. Agitation is frequently responsible for caregiver burn-out and leads to institutionalization. The dramatic increase in the prevalence of Alzheimer's disease and related disorders requires better management of behavior symptoms. Although environmental adaptation has been proposed recently, for many years, psychoactive medications and physical restraints were the primary approach. However, in severely demented patients, both pharmacologic and non-pharmacologic treatments are inoperative. In this situation, alternative pharmacologic approach should be tested. Cyproterone acetate, an antiandrogen and progestative steroid has never been proposed to prevent aggressive behavior in dementia, but its favorable effect is well described in rat and monkey aggressivity. PATIENTS AND METHODS: Cyproterone acetate was proposed for 19 demented patients who developed severe aggressive behaviors or an agitation unresponsive to psychoactive drugs (even in association) or to environmental adaptation. Clinical and behavioral analysis was carried out using the Cohen-Mansfield agitation inventory associated with an assessment of dependency in daily life activities, before and during treatment with cyproterone acetate. The behavioral status was stable, with permanent or repetitive agitation. Seven patients had vascular dementia, 7 had Alzheimer's disease, 2 had fronto-temporal degeneration, 2 had Huntington's disease and 1 a probable diffuse Lewy bodies disease. Fifteen patients had prominent aggressive behavior and 4 had predominant aberrant motor behavior with aggressive behavior. RESULTS: Cyproterone (50 to 100mg - mean: 92.5mg daily) improved significantly aggressive and impulsive behavior related to Alzheimer's disease or vascular dementia but had no effect on aberrant motor behavior. When cyproterone was stopped, aggressive behaviors reappeared more rapidly in vascular dementia. CONCLUSION: Cyproterone acetate is then an interesting choice when aggressive behavior is not improved with psychotropic drugs. A detailed clinical analysis is required to avoid the use of cyproterone in non-aggressive and non-impulsive patients. The results of this preliminary study suggest a randomized double-blind study should be carried out in the near future. The behavior improvement could be related to the blockage of androgen receptors, and simultaneously to the sedative effect of progestative drugs.
Assuntos
Agressão/psicologia , Antipsicóticos/uso terapêutico , Ciproterona/uso terapêutico , Demência/psicologia , Agitação Psicomotora/tratamento farmacológico , Agitação Psicomotora/etiologia , Idoso , Doença de Alzheimer/psicologia , Antagonistas de Androgênios/uso terapêutico , Antipsicóticos/administração & dosagem , Demência Vascular/psicologia , Esquema de Medicação , Feminino , Humanos , Doença de Huntington/psicologia , Comportamento Impulsivo/psicologia , Masculino , Projetos Piloto , Agitação Psicomotora/diagnóstico , Índice de Gravidade de DoençaRESUMO
We made a retrospective evaluation of clinical and radiologic features, treatment, and outcome of Erdheim-Chester disease, a rare non-Langerhans cell histiocytosis. We had 7 patients coming from 3 French teaching hospitals and reviewed 52 cases from the literature. These cases were considered to have Erdheim-Chester disease when they had either typical bone radiographs (symmetrical long bones osteosclerosis) and/or histologic criteria disclosing histiocytic infiltration without features for Langerhans cell histiocytosis (no S-100 protein, no intracytoplasmic Birbeck granules). Ages at diagnosis ranged from 7 to 84 years (mean +/- SD = 53 +/- 14 yr) with a male/female ratio of 33/26. Bone pain was the most frequent clinical sign (28/59), mostly located in the lower limbs. Exophthalmos and diabetes insipidus were found in respectively 16/59 and 17/59 patients. General symptoms (fever, weight loss) and "xanthomas" (mainly located on the eyelids) were present in 11/59 patients. Retroperitoneal involvement was found in 17/59 patients. Skeletal X-ray showed typical osteosclerosis of the diaphysis of the long bones in 45/59 patients. Bone radiographs showed osteolytic lesions of the flat bones (skull, ribs) in 8 patients. Histologic diagnosis was performed after a bone biopsy (28 patients), a retroorbital biopsy (9 patients), and/or a biopsy of the retroperitoneal infiltration or the kidney (11 patients). Six of our 7 patients but only 5 of 52 patients from the literature had the complete histologic criteria, disclosing no Birbeck granules or S-100 immunostaining. In other cases, histologic results usually described a xanthogranulomatous infiltration by foamy histiocytes nested in fibrosis. Treatment was corticotherapy (20/59), chemotherapy (8/59), radiotherapy (6/59), surgery (3/59) and immunotherapy (1 patient). Twenty-two patients died after a mean follow-up of 32 +/- 30 mo (range, 3-120 mo). In conclusion, Erdheim-Chester disease may be confused with Langerhans cell histiocytosis as it sometimes shares the same clinical (exophthalmos, diabetes insipidus) or radiologic (osteolytic lesions) findings. However, it also appears to have distinctive features. Patients are older and have a worse prognosis than those with Langerhans cell histiocytosis, and the diagnosis relies on the association of specific radiologic and histologic findings.
Assuntos
Histiocitose/complicações , Histiocitose/diagnóstico por imagem , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Feminino , Histiocitose/patologia , Humanos , Masculino , Pessoa de Meia-Idade , RadiografiaRESUMO
Parkinsonian tremor may be suppressed by thalamic stimulation. For an equivalent clinical efficacy, its obvious advantage over micro-thalamotomy is its reversibility. This patient experienced postural tremor at the age of 44 years and akineto-rigid syndrome 8 years later. At the age of 60 years, intrathalamic stimulation was applied over a long-term period of 43 months until death and was efficient on tremor with low stimulation. This case is the first with anatomic verification. The extent of the lesion provoked by the electrode is very small. The location of the stimulation site was in the medio-inferior part of the intermedius complex at the entrance of cerebello-thalamic fibers. The stimulation of the cerebellar afferent axons could be the cause of the clinical effect. The stimulation site corresponds to the thalamic source of the precentral and accessory motor cortex, which correlates with changes observed in our PET study showing a regional cerebral blood flow decrease in cerebellar nuclei and also in precentral and accessory motor cortex. The places and mechanisms of the effects of stimulations and lesions could be different.
Assuntos
Terapia por Estimulação Elétrica , Transtornos dos Movimentos/prevenção & controle , Doença de Parkinson/patologia , Doença de Parkinson/terapia , Tálamo/patologia , Adulto , Circulação Cerebrovascular/fisiologia , Terapia por Estimulação Elétrica/efeitos adversos , Eletrodos Implantados/efeitos adversos , Evolução Fatal , Gliose/etiologia , Gliose/patologia , Humanos , Masculino , Doença de Parkinson/fisiopatologia , Substância Negra/patologia , Tremor/etiologiaRESUMO
The long-term neuropsychological and psychiatric sequelae of herpes simplex virus encephalitis (HSVE) and their relationship to the volume of temporal lesions and to amygdala and hippocampus damage remain undefined. We have conducted a prospective study of long-term sequelae in 11 patients with clinically presumed HSVE and detection of HSV DNA in the cerebrospinal fluid by polymerase chain reaction. Six months after encephalitis, patients underwent neuropsychological and language assessment. At the same stage, single photon emission computed tomography (SPECT) evaluated the occurrence of hypoperfusion with an index of asymmetry. MRI was used for the measurement of amygdala, hippocampus and cerebral lesions by two blind neurologists. The volume of the amygdala and hippocampus was compared with those of five controls, matched for age and level of education. Long-term memory disorders were seen in 6 patients, associated with the larger lesions and damage of at least two structures. Long-term behavioural changes with emotionalism, irritability, anxiety or depression were prominent in 7. Left prefrontal hypoperfusion appeared in 8 patients, associated with psychiatric disorders in 7 and left amygdala damage in 6. The reduction of amygdala and hippocampus volume was correlated with the overall volume of lesions. Different patterns of mesial temporal lobe damage occurred, involving either amygdala alone, or amygdala and hippocampus, but never hippocampus alone. MRI volumetric measurements in HSVE could be a good indicator of long-term prognosis. Persistant behavioural changes could be related to an amygdala and frontal dysfunction.
Assuntos
Tonsila do Cerebelo/fisiopatologia , Transtornos Cognitivos/etiologia , Encefalite Viral/psicologia , Herpes Simples/psicologia , Transtornos Mentais/etiologia , Aciclovir/uso terapêutico , Adulto , Idoso , Estudos de Casos e Controles , Circulação Cerebrovascular/fisiologia , Estudos de Avaliação como Assunto , Feminino , Hipocampo/fisiopatologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Testes Neuropsicológicos , Estudos Prospectivos , Tomografia Computadorizada de Emissão de Fóton ÚnicoRESUMO
In three cases of histologically proved Chester-Erdheim disease there was a large anterior epidural lesion from C-3 to L-2 in one patient; dural masses and orbital infiltration in a second patient; and dural, choroid plexus, retroorbital, and hypophyseal lesions in a third patient. Diabetes insipidus, exophthalmia, long bone lesions, and retroperitoneal infiltration were present.
Assuntos
Encefalopatias/diagnóstico , Granuloma/diagnóstico , Histiocitose/diagnóstico , Imageamento por Ressonância Magnética , Osteosclerose/diagnóstico , Doenças da Medula Espinal/diagnóstico , Tomografia Computadorizada por Raios X , Xantomatose/diagnóstico , Idoso , Encefalopatias/etiologia , Encefalopatias/patologia , Diagnóstico Diferencial , Feminino , Granuloma/etiologia , Granuloma/patologia , Histiocitose/etiologia , Histiocitose/patologia , Humanos , Microscopia Eletrônica , Pessoa de Meia-Idade , Mielografia , Doenças Profissionais/diagnóstico , Doenças Profissionais/etiologia , Doenças Profissionais/patologia , Osteosclerose/etiologia , Osteosclerose/patologia , Fatores de Risco , Doenças da Medula Espinal/etiologia , Doenças da Medula Espinal/patologia , Síndrome , Indústria Têxtil , Xantomatose/etiologia , Xantomatose/patologiaRESUMO
The authors report on the long-term results of chronic stereotactic stimulation of the ventralis intermedius thalamic nucleus performed in 14 cases of disabling and intractable tremor. There were 10 patients with parkinsonian tremor and four with essential tremor. Three of the 10 parkinsonian patients had previously undergone contralateral thalamotomy. Tremor was assessed by clinical evaluation, surface electromyography, accelerometer, and videotape recordings before and after stimulation. The deep-brain electrode was implanted in the ventralis intermedius nucleus according to stereotactic procedure and connected to a subcutaneous pulse generator after a stimulation test period. Tremor suppression or reduction was obtained in all cases with high-frequency (130 Hz) stimulation. Marked functional improvement was maintained in 11 patients with a mean follow-up interval of 17 months. Levodopa-induced dyskinesias observed in five parkinsonian patients prior to surgery were improved or suppressed in four cases by thalamic stimulation. Stimulation was continued during the day and stopped at night in eight cases. Six patients were stimulated night and day to avoid a rebound effect which appeared as soon as the pulse generator was stopped. The only side effects were hand tonic posture in one case and persistent paresthesia in another case. The mechanism of action of this attractive treatment may be a functional alteration of the thalamic discharging area. The authors conclude that this technique is a good alternative to thalamotomy, especially when the risks of high-frequency coagulation are severe in frail and older patients.
Assuntos
Terapia por Estimulação Elétrica/métodos , Técnicas Estereotáxicas , Núcleos Talâmicos/fisiopatologia , Tremor/terapia , Adulto , Idoso , Terapia por Estimulação Elétrica/efeitos adversos , Humanos , Pessoa de Meia-Idade , Núcleos Talâmicos/cirurgia , Tremor/etiologia , Tremor/cirurgiaRESUMO
Behavioral disorders were a prominent clinical feature after the surgical treatment of an anterior communicating artery aneurysm rupture in a 44-year-old man. Callosal apraxia was associated with an alien hand. The latter remained 1 year after surgery while diagonistic apraxia disappeared after 3 months. Other callosal signs included left agraphia, tactile anomia and auditory suppression. MRI revealed posterior callosal infarction and a right frontal infarct. The association of diagonistic apraxia and alien hand is rarely reported.
RESUMO
Chronic thalamic-VIM stimulation was performed in 9 parkinsonian patients with disabling tremor and poor response to drugs. Neuropsychological assessment was performed before and after deep brain electrode implantation and stimulation. Mild cognitive disorders were observed prior to thalamic implantation. Neuropsychological testing failed to show intellectual function worsening after implantation and stimulation. We conclude that thalamic stimulation could be an appropriate treatment of untractable tremor as this could provide less neuropsychological side-effects than thalamotomy, especially in Parkinson's disease.
Assuntos
Terapia por Estimulação Elétrica , Doença de Parkinson/terapia , Núcleos Talâmicos , Idoso , Humanos , Levodopa/uso terapêutico , Pessoa de Meia-Idade , Doença de Parkinson/complicações , Transtornos Psicofisiológicos/etiologia , Técnicas Estereotáxicas , Tálamo/cirurgia , Tremor/terapiaRESUMO
A 33-year-man with an encephalopathy of unknown aetiology, had an history of epilepsia for 30 years. Different types of seizures were seen, including grand mal and frontal attacks. Epilepsia was associated with mental retardation and behavioral disorders. At the age of 33, he was admitted for repetitive general convulsions. Epilepticus status lasted for two weeks and improved with vigabatrin et clonazepam. General seizures, frontal motor convulsions with arms and trunk antepulsion, and dacrystic attacks were seen. The latter seemed to be like normal crying because they were accompanied by lacrimation, contorted and mournful facies, and sobbing sounds. One year later, repetitive cardiac arrests occurred during a new epilepticus status. Cardiac arrests, observed on ECG holter lasted 10 to 24 seconds, without cardiac dysfunction. EEG patterns on ECG holter lasted 10 to 24 seconds, without cardiac dysfunction. EEG patterns included theta and delta activity with rhythmic slow wave epileptic activity, predominating on right side, in temporal areas. CT scan was normal. MRI showed right cerebral atrophy, prevailing in the temporo-mesial region, with right temporal horn enlargement. This case report of dacrystic seizures, the first one with MRI study, suggests that temporo-mesial structures of the non-dominant hemisphere may be involved in dacrystic and asystolic attacks.
Assuntos
Epilepsia Tônico-Clônica/fisiopatologia , Parada Cardíaca/etiologia , Estado Epiléptico/fisiopatologia , Adulto , Atrofia , Eletrocardiografia , Eletroencefalografia , Epilepsia Tônico-Clônica/complicações , Humanos , Imageamento por Ressonância Magnética , Masculino , Estado Epiléptico/complicações , Lágrimas , Lobo Temporal/patologiaRESUMO
This 44-year woman was admitted for weight loss and global intellectual slowing. She had mild chronic alcoholic neuropathy. She was discontinued alcoholic consumption for 6 months and was given disulfiram (1.5 g/day) since then. She developed over a 5-day period acute neuropathy, confusion and extrapyramidal symptoms with oculo-cephalogyric and dystonic movements and myoclonus. Electromyography revealed a severe polyneuropathy. After disulfiram withdrawal, confusion and extrapyramidal symptoms disappeared within a few days, but sensitivo-motor deficit improved more slowly. Nerve biopsy was suggestive of a pure axonal neuropathy.
Assuntos
Dissuasores de Álcool/intoxicação , Doenças dos Gânglios da Base/induzido quimicamente , Dissulfiram/intoxicação , Mioclonia/induzido quimicamente , Doenças do Sistema Nervoso Periférico/induzido quimicamente , Doença Aguda , Adulto , Doença Crônica , Feminino , Humanos , Fatores de TempoRESUMO
Positive diagnosis of Herpes simplex virus (HSV) encephalitis was rarely obtained in the past, when brain biopsy had been performed. Other tests (HSV antigen and HSV antibodies detection and interferon alpha measurement, in cerebrospinal fluid) failed to prove HSV infection. Polymerase chain reaction has been proposed for accurate and rapid diagnosis of HSV encephalitis. With 35 cycles of a DNA polymerase sequence duplication, sensitivity reaches 95% and specificity 100%. HSV PCR is a useful tool for the diagnosis of acute encephalitis. This should be available in many neurologic clinics. Therapeutic consequences include rapid disruption of aciclovir when clinical features, MRI study and negative PCR suggest non herpetic encephalitis.
Assuntos
Aciclovir/uso terapêutico , Encefalite Viral/diagnóstico , Herpes Simples/diagnóstico , Reação em Cadeia da Polimerase , Simplexvirus/genética , Doença Aguda , Adolescente , Adulto , Idoso , Antivirais/uso terapêutico , Criança , Pré-Escolar , Encefalite Viral/terapia , Herpes Simples/terapia , Humanos , Lactente , Pessoa de Meia-Idade , Prognóstico , Recidiva , Sensibilidade e EspecificidadeRESUMO
After 10 years of clinical practice (1987-1997), chronic thalamic deep brain stimulation (DBS) is considered to be effective in the treatment of drug-resistant parkinsonian tremor. DBS has produced few side-effects, which are usually reversible. More recently, DBS has been applied to other movement disorders (akinesia and rigidity, dyskinesias, dystonia), using new targets: internal pallidum, subthalamic nucleus. These targets have been selected on the basis of neurophysiological or anatomo-clinical data suggesting they could be effective. Control of L-Dopa peak-dose dyskinesias by thalamic ventralis intermedius nucleus (V.im.) stimulation has been reported by the Lille team, but not by the Grenoble team. We therefore re-examined all teleradioanatomical data of both teams, and compared them with the therapeutic effects. Location of 99 monopolar electrodes of thalamic stimulation, applied to treat parkinsonian tremor, has been retrospectively measured. The Lille team included 21 patients (22 electrodes); the Grenoble team included 52 patients (74 electrodes). L-Dopa dyskinesias were suppressed in all 9 patients in Lille, and improved clearly in only 8 out of 32 patients in Grenoble. The mean center of electrodes was significantly different between both teams, being deeper, more posterior and medial in Lille. This did not correspond to the coordinates of the V.im., but seems to be closer to those of the centromedian and parafascicular complex (CM-Pf), according to stereotactic atlases. Considering only the dyskinetic patients, the therapeutic effects on L-Dopa dyskinesias were related to the differences observed in the electrode position, but not to the team membership. Improvement of L-Dopa dyskinesias was significantly associated with deeper and more medial placement of electrodes. Retrospective analysis of ventriculographic data confirmed that the electrode position and therapeutic effects of DBS are strongly related. Our study suggested that CM-Pf stimulation could control both tremor and L-Dopa dyskinesias. This hypothesis is consistent with neuro-anatomical data showing that CM-Pf is connected to internal pallidum, the stimulation of which controls specifically L-Dopa dyskinesias.
Assuntos
Discinesia Induzida por Medicamentos/terapia , Terapia por Estimulação Elétrica , Levodopa/efeitos adversos , Núcleos Talâmicos/fisiologia , Idoso , Ventriculografia Cerebral , Discinesia Induzida por Medicamentos/fisiopatologia , Eletrodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Técnicas Estereotáxicas , Núcleos Talâmicos/anatomia & histologiaRESUMO
Sickle cell disease (homozygotes SS) is known as a risk factor for both ischemic and hemorrhagic stroke, but heterozygotes AS seem to be spared. We carried out a retrospective study to assess the main risk factors and the influence of hemoglobin abnormalities on stroke in Guadeloupe. The percentages of AS, AC, and AA on 295 patients admitted for stroke were compared to the prevalence obtained on 72,000 newborn babies. Ischemic, hemorrhagic stroke and stroke complications represented respectively 83 p. 100, 10 p. 100 and 7 p. 100. Seventy one per 100 of patients had hypertension and 19 p. 100 had an association of diabetes and hypertension. The percentage of heterozygotes AS was significantly lower in the group with ischemic stroke (4 p. 100) in comparison with controls (8.5 p. 100), while AS were more represented in hemorrhagic stroke (16 p. 100). The risk of hemorrhagic stroke was 10 fold higher in AS patients admitted for stroke and the risk of ischemic stroke was reduced by 15 fold. These data suggest that the sickle cell trait could be associated to red cell and/or endothelial specificities which could prevent for ischemic stroke. The influence of AS heterozygote on the occurrence of stroke needs to be examined in a longitudinal, prospective study.
Assuntos
Isquemia Encefálica/epidemiologia , Isquemia Encefálica/etiologia , Hemorragia Cerebral/epidemiologia , Hemorragia Cerebral/etiologia , Traço Falciforme/complicações , Adulto , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Encéfalo/irrigação sanguínea , Isquemia Encefálica/diagnóstico , Hemorragia Cerebral/diagnóstico , Feminino , Guadalupe/epidemiologia , Hemoglobinas/análise , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Estudos Prospectivos , Fatores de Risco , Distribuição por SexoRESUMO
A 32-year-old woman complained of swallowing difficulty after a general seizure. Neurological examination revealed unilateral palsies of the 9th, 10th, and 12th cranial nerves. CT, MRI and internal carotid artery angiogram were normal. Selective catheterization of the external carotid artery and ascending pharyngeal system suggested a cranial nerve ischaemic arterial syndrome. The apparent sparing of the eleventh nerve may be explained by the double vascularization of this nerve. This may also be related to the double innervation of the trapezius and sterno-cleido-mastoid muscles by the 11th nerve and cervical spinal nerves.