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Query: UMLS:C0038454 (stroke)
 147,016 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 79-year-old woman was admitted to our hospital, due to acute onset of left hemiparesis and disturbance of consciousness. Although her symptoms improved temporarily, she developed gait disturbance and cognitive deterioration 2 months after the onset. After that, she presented with myoclonus and startle response, followed by akinetic mutism within 8 months after the onset. Serial EEGs revealed no periodic synchronous discharge. Serial diffusion-weighted MRIs showed that high intensity lesions, which initially limited to the right cerebral cortex, gradually spread to the bilateral cerebral cortices and basal ganglia, with relative sparing of central gyri, medial occipital cortices, and hippocampus. Prion protein gene analysis revealed a point mutation (Val-->Ile) at codon 180. The result of this patient suggests that this type of CJD might be associated with an atypical clinical course such as stroke-like episode and selective involvement of cortical and subcortical lesions.
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PMID:[A case of Creutzfeldt-Jakob disease with stroke-like episode as an initial symptom]. 1992 Mar 75

Genetic transmissible spongiform encephalopathies (TSEs) account for approximately 10-15% of overall human prion diseases worldwide, but genotype-phenotype correlations remain incomplete. Here we report the case of an 80-year-old man who developed rapidly progressive behavioral abnormalities and myoclonus following a stroke. Repeated electroencephalography (EEG) revealed a general slowing of the basic activity, as well as several episodes of triphasic waves, with neither periodic activity nor recorded seizure. 14.3.3 protein was detected in cerebral cerebrospinal fluid, and direct sequencing of the PRNP gene showed an E196K mutation associated with homozygosity for methionine at codon 129. The patient was diagnosed with probable genetic prion disease with a Creutzfeldt-Jakob disease-like phenotype. The PRNP E196K mutation has only rarely been described in the literature, and generally patients exhibited an atypical initial phenotype, mainly involving abnormal behavioral features. Further observations are needed to confirm this particular clinical pattern associated with the mutation.
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PMID:Rare E196K mutation in the PRNP gene of a patient exhibiting behavioral abnormalities. 2000 32

The literature is reviewed. Hashimoto encephalopathy is a steroid-responsive encephalopathy associated with Hashimoto thyroiditis (SREHT). The incidence of SREHT in childhood is underestimated. Two subtypes have been described. The diffuse progressive type is associated with insidious onset and progressive impairment of mental status, such as confusion, somnolence, and psychosis. The vasculitic type is characterized by acute stroke-like episodes associated with focal neurologic features and seizures. Hashimoto encephalopathy (SREHT) is a clinical condition with elevated thyroid antibody titers encompassing persistent or relapsing seizures, myoclonus, focal neurologic deficits and neuropsychiatric disorders such as psychosis, delusions and hallucinations, and affective disorders. By far, the vast majority of reports on paediatric and adult patients describe a dramatic clinical response to either methylprednisolone pulse therapy or daily oral prednisone or prednisolone.
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PMID:[Undetected Hashimoto encephalopathy--a diagnostic challenge in child psychiatry and child neurology]. 2004 73

Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disorder in which accumulation of a pathogenic isoform of prion protein (PrP(Sc)) induces neuronal damage with distinct pathologic features. The prognosis of sCJD is devastating: rapid clinical decline is followed by death generally within months after onset of symptoms. The classic clinical manifestations of sCJD are rapidly progressing dementia, myoclonus, and ataxia. However, the spectrum of clinical features can vary considerably. We describe a definite, neuropathologically verified sCJD in a 67-year-old woman who initially presented with progressive stroke-like symptoms: left-sided hemiparesis and ataxia within a few days. The initial brain magnetic resonance imaging (MRI) showed bilateral cortical hyperintensity on diffusion-weighted sequences (DWI) resembling multiple ischemic lesions. Despite anticoagulation with low-molecular-weight heparin, the patient deteriorated rapidly, became dysphagic and bedridden with myoclonic jerks on her left side extremities correlating with intermittent high-amplitude epileptiform discharges on electroencephalography (EEG). Basal ganglia hyperintense signal changes in addition to cortical ribboning were seen in DWI images of a follow-up MRI. Repeated EEG recordings showed an evolution to periodic sharp wave complexes. Protein 14-3-3 was positive in her cerebrospinal fluid specimen, in addition to an abnormally high total tau level. In the terminal stage the patient was in an akinetic, mutistic state with deteriorating consciousness. She died 19 days after admission to the hospital. Neuropathologic investigation corroborated the clinical diagnosis of sCJD with spongiform degeneration and immunohistochemical demonstration of the deposition of pathologic PrP(Sc).
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PMID:Progressive Stroke-Like Symptoms in a Patient with Sporadic Creutzfeldt-Jakob Disease. 2068 29

Dural arteriovenous fistulas (DAVFs) have a wide range of clinical presentations, including dementia associated with white matter changes (WMCs). We report a case of DAVF presenting as a rapid progressive dementia and myoclonus without WMCs. A 64-year-old hypertensive and diabetic man was admitted because of a 3-month history of progressive cognitive decline, extrapyramidal and cerebellar signs, and myoclonus. Magnetic resonance imaging (MRI) scans of the brain showed dilated cerebellar veins and T2WI hypersignal in the basal ganglia without WMCs. After admission, he suffered sequential bilateral deep intracerebral hemorrhages. A repeated angioMRI disclosed thrombosis of the distal sagittal and the proximal lateral sinuses. Angiography revealed a torcullar region DAVF. Embolization of the dural fistula was performed. On follow-up, the patients' cognitive deficits improved and myoclonus disappeared. The clinical picture may be explained by venous hypertension in the deep venous system, producing bilateral basal ganglia/thalamic dysfunction and in the posterior fossa. This case shows that DAVFs can produce subcortical dementia without involvement of the deep white matter.
J Stroke Cerebrovasc Dis 2012 Oct
PMID:Rapidly progressive cognitive impairment, ataxia, and myoclonus: an unusual presentation of a dural arteriovenous fistula. 2137 30

Hashimoto's encephalopathy (H.E.) is probably of autoimmune etiology, and manifests with seizures, stroke-like episodes, cognitive decline, neuropsychiatric symptoms, myoclonus. It is presumed to be autoimmune in origin with high serum titers of antithyroid peroxidase antibodies (anti-TPA). Thyroid function might often be normal. The diagnosis is arrived at by excluding other toxic, metabolic and infectious causes of encephalopathies, supportive clinical profile, elevated thyroid antibodies and optimum steroid response. We present the characteristic phenotypic manifestations, magnetic resonance imaging and electroechography observations and response to immunomodulation with follow-up in three cases of H.E. All the three cases manifested with subacute to chronic progressive encephalopathy, cerebellar dysfunction, seizures, behavioral abnormalities and oculomotor disturbances and had evidence of hypothyroidism, elevated titers of anti-TPA and positive thyroid anti-microsomal antibodies. Atypical and uncommon presentations are known. This report emphasizes that a high index of suspicion is often required in cases with "investigation negative encephalopathy" for early diagnosis of H.E.
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PMID:Steroid-responsive encephalopathy in autoimmune thyroiditis: Clinical spectrum and MRI observations in three cases. 2202 37

Mitochondrial respiratory chain disorders are relatively common inborn errors of energy metabolism, with a combined prevalence of one in 5000. These disorders typically affect tissues with high energy requirements, and cerebral involvement occurs frequently in childhood, often manifesting in seizures. Mitochondrial diseases are genetically heterogeneous; to date, mutations have been reported in all 37 mitochondrially encoded genes and more than 80 nuclear genes. The major genetic causes of mitochondrial epilepsy are mitochondrial DNA mutations (including those typically associated with the mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes [MELAS] and myoclonic epilepsy with ragged red fibres [MERRF] syndromes); mutations in POLG (classically associated with Alpers syndrome but also presenting as the mitochondrial recessive ataxia syndrome [MIRAS], spinocerebellar ataxia with epilepsy [SCAE], and myoclonus, epilepsy, myopathy, sensory ataxia [MEMSA] syndromes in older individuals) and other disorders of mitochondrial DNA maintenance; complex I deficiency; disorders of coenzyme Q(10) biosynthesis; and disorders of mitochondrial translation such as RARS2 mutations. It is not clear why some genetic defects, but not others, are particularly associated with seizures. Epilepsy may be the presenting feature of mitochondrial disease but is often part of a multisystem clinical presentation. Mitochondrial epilepsy may be very difficult to manage, and is often a poor prognostic feature. At present there are no curative treatments for mitochondrial disease. Individuals with mitochondrial epilepsy are frequently prescribed multiple anticonvulsants, and the role of vitamins and other nutritional supplements and the ketogenic diet remain unproven.
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PMID:Mitochondrial disease and epilepsy. 2228 95

Although rare, many different types of hyperkinetic and hypokinetic movement disorders have been described after both ischemic and hemorrhagic stroke in children and in adults. Current knowledge about these disorders comes from single case reports or small series of cases compiled from retrospective studies. Data from hospital-based studies suggest a prevalence of poststroke movement disorders ranging from 1.1 to 3.9%. However, despite the development of emergency care for stroke, these clinical syndromes remain insufficiently recognized. Poststroke movement disorders take place in the acute phase or following a variable delay after stroke onset, and could be transient or persistent. Dystonia is the most frequent movement disorder, occurring after a delay of several months, while chorea and hemiballism are most frequent in the acute stages. Amongst transient movement disorders, limb shaking is associated with high-grade stenosis or occlusion of the internal carotid artery, while myoclonus and asterixis are rare. From a pathophysiological point of view, most of these symptoms are induced by a lesion involving the basal ganglia, the thalamus, or the frontal subcortical pathways. In this article, we updated the clinical spectrum, neuropathophysiological mechanisms, and prognosis of stroke-induced movement disorders in adults and children.
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PMID:Clinical spectrum of movement disorders after stroke in childhood and adulthood. 2273 57

Acute ataxia is a fairly common emergency that confronts the pediatric neurologist in daily life. The differential diagnosis of acute pediatric ataxia is wide, but informed history and careful clinical examination can narrow it and help target investigations. This review discusses various etiologies of acute pediatric ataxia, focusing on clinical presentation, diagnostic considerations, and approach to investigation. Aspects of treatment and prognosis are also mentioned. Diseases with potentially high morbidity and mortality, such as acute cerebellitis, opsoclonus-myoclonus syndrome, and cerebellar stroke, receive particular attention.
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PMID:Acute ataxia in children: approach to clinical presentation and role of additional investigations. 2325 68

Movement disorders can occur as primary (idiopathic) or genetic disease, as a manifestation of an underlying neurodegenerative disorder, or secondary to a wide range of neurological or systemic diseases. Cerebrovascular diseases represent up to 22% of secondary movement disorders, and involuntary movements develop after 1-4% of strokes. Post-stroke movement disorders can manifest in parkinsonism or a wide range of hyperkinetic movement disorders including chorea, ballism, athetosis, dystonia, tremor, myoclonus, stereotypies, and akathisia. Some of these disorders occur immediately after acute stroke, whereas others can develop later, and yet others represent delayed-onset progressive movement disorders. These movement disorders have been encountered in patients with ischaemic and haemorrhagic strokes, subarachnoid haemorrhage, cerebrovascular malformations, and dural arteriovenous fistula affecting the basal ganglia, their connections, or both.
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PMID:Movement disorders in cerebrovascular disease. 2386 93


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