RESUMEN
Hypertrophy of the calves has been described in spinal muscular atrophy (SMA) syndrome. Pearn and Hudgson described a new spinal muscular atrophy syndrome characterized adolescent onset, gross hypertrophy of the calves, and a slowly progressive clinical course. We saw a 16-year-old female who had weakness of the thighs with atrophy and hypertrophy of calfmuscles. The patient was studied with EMG and muscle biopsy and thought to be as SMA with hypertrophied calf-muscles.
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Adolescente , Femenino , Humanos , Atrofia , Biopsia , Hipertrofia , Atrofia Muscular Espinal , MusloRESUMEN
A 19-year-old man with periodic alternating nystagmus (PAN) is presented. He reported that he had had oscillopsia and head oscillations for as long as he could remember. Acquired diseases associated with PAN were ruled out with various laboratory aids. This case is believed to be the first reported case of PAN in Korea.
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Humanos , Adulto Joven , Cabeza , Corea (Geográfico) , Nistagmo PatológicoRESUMEN
Giant cell arteritis (GCA) is an autoimmune vasculitic disorder of unknown origin. Systemic GCA causing cerebral infarction due to intracranial arteritis is rare. Early diagnosis and anti-inflammatory treatment of the GCA are necessary to prevent systemic involvement. A 66-year-old woman presented with dysarthria and left hemiparesis. A brain MRI showed ischemic lesions in the right temporoparietal area. We report a pathological case of GCA with clinical and neuroradiological evidence of cerebral infarction.
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Anciano , Femenino , Humanos , Arteritis , Encéfalo , Infarto Cerebral , Disartria , Diagnóstico Precoz , Arteritis de Células Gigantes , Células Gigantes , Imagen por Resonancia Magnética , Paresia , PatologíaRESUMEN
Marchiafava-Bignami disease(MBD), characterized by the primary degeneration of the corpus callosum, is a rare complication of chronic alcoholism. Recently, a few cases of MBD with reversible neuro-imaging abnormalities were reported. A 58-year-old, chronic alcoholic man was admitted with mental change, dysarthria, and a seizure attack. A T2-weighted Brain magnetic resonance imaging demonstrated high signal intensities in the body and splenium of the corpus callosum, multiple white matter, and cortical gray matter. Treatment with a multiple vitamin complex resulted in a near complete recovery of neurological manifestation. A brain MRI obtained four weeks after admission revealed a dramatic resolution of previous imaging abnormalities. We report a case of Marchiafava-Bignami disease with reversible neuro-imaging abnormalities.
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Humanos , Persona de Mediana Edad , Alcohólicos , Alcoholismo , Encéfalo , Cuerpo Calloso , Disartria , Imagen por Resonancia Magnética , Enfermedad de Marchiafava-Bignami , Manifestaciones Neurológicas , Convulsiones , VitaminasRESUMEN
No abstract available.
RESUMEN
In early thrombolytic therapy for acute focal ischemic stroke, the start of treatment within therapeutic time window is one of the most important thing. Recently, new imaging modalities such as SPECT, transcranial doppler, diffusion/ perfusion-weighted MRI, and MR anglography have been implicated to avoid time consumption and delayed therapy. Of these, MR angiography is nomnvasi and rapid technique to visualize large and medium-sized arteries. We explored the usefulness of MR angiography in early thrombolytic therapy. Arterial occlusion of three patients with severe ischemic stroke were demonstrated on MR anglography and wluch were treated with Urokmase (10, 000-20, 000 units/kg) by intravenous infusion within 2-4 hours after symptom onset. Recanalization and brain lesion was assessed by repeated MR angiography and MRI or CT 24 hours later. Clinical improvement was observed in two patients 5-24 hours after initiation of treatment. In one patient hemorrhagic infarction without clinical deterioration was detected by follow-up computed tomography. Recanalization was documented on repeated MR angiography of three patients. MR angiography can document occlusion of stroke-related vasculature without delay of thrombolytic therapy and repeated MR anglography can reveal whether recanalization has occurred.
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Humanos , Angiografía , Arterias , Encéfalo , Estudios de Seguimiento , Infarto , Infusiones Intravenosas , Angiografía por Resonancia Magnética , Imagen por Resonancia Magnética , Accidente Cerebrovascular , Terapia Trombolítica , Tomografía Computarizada de Emisión de Fotón ÚnicoRESUMEN
Symptoms of compressive cervical myelopathy classically include spasticity and weakness, predominantly involving the lower extremities. Sensory abnormalities are reportedly common in the upper extremities, but are often vague or misleading. The sensory findings are usually localized 2-3 spinal segments below the actual spinal cord compression. In our current series, 3 patients presented with progressive symptoms of weakness and hyperreflexia involving the lower extremities without upper extremity symptoms and with a distant thoracic sensory level ranging from T10 to T12. All 3 patients were eventually found to have a cervical herniated intervertebral disc. The direct physical effects of compression and vascular compromise in the central cervical cord compression may be responsible for the reported abnormality at a distinct thoracic sensory level. Failure to diagnose cervical myelopathy because of the presence of a thoracic sensory level can delay appropriate treatment or lead to incorrect therapy.
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Humanos , Disco Intervertebral , Extremidad Inferior , Espasticidad Muscular , Reflejo Anormal , Compresión de la Médula Espinal , Enfermedades de la Médula Espinal , Extremidad SuperiorRESUMEN
BACKGROUND: To clarify the clinicoradiological correlation and prognosis of acute ischemic stroke involving para-median territory of pons. METHODS: We studied 37 patients with first-ever ischemic stroke involving paramedian terri-tory of pons and divided them based on the shape and level of lesion shown in their MRI. The clinical features, MRI findings, and prognosis were assessed. RESULTS: The paramedian infarctions extending to the basal surface were found in 28 patients (76%), and small infarctions separated from the basal surface were found in 9 patients (24%). In patients with infarction extending to the basal surface, 23 patients (82%) had progressive or fluctuating onset, whereas all patients with small infarction separated from the basal surface had non-progressive onset. In the group with upper pon-tine lesion (14 patients), dysarthria-clumsy hand syndrome was found in 4 patients, ataxic hemiparesis (AH) in 3, pure motor hemiparesis (PMH) in 2, and pure sensory stroke in 1. In the group with middle and lower pontine lesion (22 patients), PMH was found in 9, AH in 3, and sensory motor stroke in 2. The mean Modified Rankin Disability Scale scores on admission and after follow-up (mean 29 months) of the group with upper pontine lesion were 2.36 +/-0.50 and 1 . 0 0 +/-0.55, those with mid-lower pontine lesions, 3.48 +/-0.51 and 1.17 +/-0.49 (P0.05 respectively). CONCLUSIONS: Paramedian pontine infarction extending to the basal surface usually presents with progressive onset. Paramedian pontine infarction most often produces classic lacune syndrome of which PMH is the most common. In our study, patients with mid-lower paramedian pontine infarction had more severe initial neurological deficits than those with upper paramedian pontine infarction. However, a late outcome was found to be favorable in both groups.
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Humanos , Estudios de Seguimiento , Mano , Infarto , Imagen por Resonancia Magnética , Paresia , Puente , Pronóstico , Accidente CerebrovascularRESUMEN
We reviewed 27 patients wlth cerebellar infarction which was demonstrated by brain CT and/or MRI. Infarction occurred in the territory of posterior inferior cerebellar artery (PICAj in 16 patients, and the territory of the superior cerebellar artery(SCA) was involved in 5 patients. Antenor inferior cerebellar artery(AICA) infarcts occurred in 3 patients. Both PICA and SCA temtories were involved in 2 patients. In the remaining 1 patient, the infarct encompassed the borderzone between the SCA and PICA territories. The main symptoms and signs were sudden onset of vertigo, dizziness, nausea, vomiting, dysmetria, ataxia, nystagmus, and headache. There were signs of associated brain stem infarction or occipitotemporal infarction; rostral basilar artery syndrome, classic SCA syndrome, Wallenberg syndrome, internuclear ophthalmoplegia, facial palsy, hearing impairment. Presumed cerebral embolism was the main stroke mechanism in the SCA terntories. Six patients with brainstem compression or brainstem involvement showed consciousness deterioration, and only one of them died as a result of extensive cerebellar infarctions involving both SCA and PICA territories Cerebellar infarction may run a more benign course than previously thought.
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Humanos , Arterias , Ataxia , Arteria Basilar , Encéfalo , Tronco Encefálico , Infartos del Tronco Encefálico , Ataxia Cerebelosa , Estado de Conciencia , Mareo , Parálisis Facial , Cefalea , Pérdida Auditiva , Infarto , Embolia Intracraneal , Síndrome Medular Lateral , Imagen por Resonancia Magnética , Náusea , Trastornos de la Motilidad Ocular , Pica , Accidente Cerebrovascular , Vértigo , VómitosRESUMEN
Parkinsonism is not commonly associated with intracranial tumors. The most common brain tumor causing parkinsonism is meningiomas. We are presenting two cases of meningiomas whose major manifestations included parkinsonism. In one, parkinsonian symptoms initially partially responded to L-dopa and bromocriptine. A CT scan taken for a stroke-like episode revealed a meningioma of left frontal convexity. After neurological sequelae of CO poisoning, the patient poorly responded to antiparkinsonian drugs. Surgical treatment of meningioma did not result in satisfactory control of extrapyramidal symptoms. In the other with a parasagittal meningioma, parkinsonian symptoms were postoperatiYely improved. Because no curative treatment is available for the majority of parkinsonian patients, early detection of an underlying neoplasm may result in a most rewarding outcome. It would be desirable to perform CT or MRI in cases of parkinsonism with other associated neurological manifestation. Unilaterality of parkinsonian symptoms may also be indication.
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Humanos , Neoplasias Encefálicas , Bromocriptina , Lóbulo Frontal , Levodopa , Imagen por Resonancia Magnética , Meningioma , Manifestaciones Neurológicas , Trastornos Parkinsonianos , Intoxicación , Recompensa , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND AND PURPOSE: Among the many factors having influence on seasonal incidence of thrombotic disease, we studied the seasonal variation of fibrinolytic activity, which might be thought to have influence on seasonal incidence of thrombotic disease. SUBJECT AND METHOD: Seasonal plasma tissue type plasminogen activator (t-PA) and euglobulin fibrinolytic activity (EFA) were measured in 18 male and 22 female normal volunteers during a year. RESULT: The level of t-PA was higher in winter than in spring (p<0.05), but was not significantly different in the other couples of four seasons. The level of EFA was higher in winter than in summer and in autumn (p<0.01), and was slightly higher in winter than in spring without significance. The level of EFA was higher in spring than in summer (p<0.05). There was no correlation between the level of t-PA and that of EFA in any of the seasons. CONCLUSION: The fibrinolytic activity is higher at lower temperature than at higher temperature. Thus, it seems that a rise in fibrinolytic activity in cold weather counters certain changes which make a thrombotic event.