Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018991 (hemiplegia)
3,997 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Dyke-Davidoff-Masson syndrome is a condition characterized by seizures, facial asymmetry, contralateral hemiplegia or hemiparesis and mental retardation. We report the clinical and imaging features in two patients with epilepsy revealing a Dyke-Davidoff-Masson syndrome. Brain MRI showed unilateral loss of cerebral volume with hypertrophy and hyperpneumatization of the paranasal sinuses and mastoid cells. Ipsilateral fronto-parietal polymicrogyria was present in one patient.
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PMID:[Dyke-Davidoff-Masson syndrome: a report of two cases]. 1579 14

Bilateral hand skill assessment with a computerised version of the Peg Moving Task, and neuropsychological testing, were performed in 30 children aged 7 to 8 years with spastic cerebral palsy (CP) and without mental retardation, diplegia (n = 10), right hemiplegia (n = 10), or left hemiplegia (n = 10), and in 30 controls. Compared to controls: (i) 30% of the hemiplegic children showed impairment of the unaffected hand and 70% of the diplegic children showed impairment in both hands; (ii) children with CP were impaired only in oral repetition and in visual-motor tasks. Results of neuropsychological testing were not significantly different between the three groups of children with CP. Right minus left asymmetry in hand skill was not related to neuropsychological testing; however, degree of impairment of the right hand was associated with phonological and metaphonological skills, and of the left hand with visuospatial and counting performance. Hand skill was related to the ability to perform many daily living manual activities. It is concluded that impairment of hand function, rather than the side of the more affected hand, is related to neuropsychological deficits in children with cerebral palsy.
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PMID:Manual skill, hand skill asymmetry, and neuropsychological test performance in schoolchildren with spastic cerebral palsy. 1584 32

Dyke-Davidoff-Masson syndrome is clinically characterized by hemiparesis, hemiplegia, seizures, mental retardation, and facial asymmetry secondary to congenital or early childhood vascular insult. A 21-year-old man with Dyke-Davidoff-Masson syndrome presented with uncontrolled seizures. The authors present the magnetic resonance (MR) and positron emission tomography (PET) findings of this syndrome.
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PMID:Positron emission tomography in Dyke-Davidoff-Masson syndrome. 1610 Apr 87

The incidence of epilepsy in 110 patients with cerebral palsy (the majority with spastic tetraplegia) was 46,4%. Almost half of the patients with spastic tetraplegia (45%) and hemiplegia (52%) had epilepsy. The incidence was lower in patients with spastic diplegia (32%). Half of epilepsy in spastic hemiplegia were partial seizures and the other half consisted of generalized seizures, while generalized tonic-clonic episodes predominated in all other forms of C.P. A high incidence of West syndrome was observed in patients with spastic tetraplegia. Etiological factors of C.P. were perinatal in 75%. 16,65% had neonatal antecedents of convulsions; most of them had spastic tetraplegia (75%) and a significant mental retardation. A low intelligence quotient (I.Q.) was seen in most of the children with epilepsy, and patients with tetraplegia had significantly lower intelligence quotient than other groups.
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PMID:[Epilepsy in children with cerebral palsy]. 1678 10

The features of a characteristic type of encephalopathy caused by human herpesvirus-6 are presented. All patients have clusters of brief convulsions during the stage of skin eruptions. Sometimes the encephalopathy resulted in hemiplegia or mental retardation. Because the clinical course is similar, we propose a subtype of encephalopathy "human herpesvirus-6 encephalopathy with cluster of convulsions in eruptive stage" for further studies of pathophysiology and treatment.
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PMID:HHV-6 encephalopathy with cluster of convulsions during eruptive stage. 1716 1

Alternating hemiplegia of childhood (AHC) is a severe brain disorder, mainly characterised by episodes of hemiplegia, progressive mental retardation, and other severe paroxysmal and permanent neurological symptoms. Clinically and genetically, there is some overlap with sporadic (SHM) and familial (FHM) hemiplegic migraine, a severe monogenic subtype of migraine. Although no mutations were detected in the FHM1 CACNA1A and FHM2 ATP1A2 genes in sporadic AHC patients, a mutation was found in the FHM2 ATP1A2 gene in a family with AHC. Recently, a missense mutation was found in the SLC1A3 gene that encodes the glutamate transporter EAAT1, in a patient with alternating hemiplegia, episodic ataxia, seizures, and headache. Because of the remarkable clinical similarities and the potential role of glutamate in AHC, we analysed six sporadic patients with AHC for mutations in the SLC1A3 gene. No mutations were found. The SLC1A3 EAAT1 glutamate transporter gene does not seem to be involved in the pathogenesis of AHC.
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PMID:Alternating hemiplegia of childhood: no mutations in the glutamate transporter EAAT1. 1723 10

We report on the case of a 36-year-old Hispanic woman with a spinal cord infarct, who was subsequently diagnosed with methylmalonic aciduria and homocystinuria, cblC type (cblC). Mutation analysis revealed c.271dupA and c.482G > A mutations in the MMACHC gene. The patient had a past medical history significant for joint hypermobility, arthritis, bilateral cataracts, unilateral hearing loss, anemia, frequent urinary tract infections, and mental illness. There was no significant past history of mental retardation, failure to thrive, or seizure disorder as reported in classic cases of cblC. Prior to the thrombotic incident, the patient experienced increased paresthesia in the lower extremities, myelopathy, and impaired gait. Given her previous psychiatric history, she was misdiagnosed with malingering until hemiplegia and incontinence became apparent. The authors would like to emphasize the recognition of a neuropsychiatric presentation in late onset cblC. Ten other reported late onset cases with similar presentations are also reviewed.
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PMID:Late-onset combined homocystinuria and methylmalonic aciduria (cblC) and neuropsychiatric disturbance. 1785 53

Dyke Davidoff Masson syndrome (DDMS) is characterized by seizures, facial asymmetry, contralateral hemiplegia and mental retardation. The characteristic radiologic features are cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses. We report a case of DDMS in an 18-month-old girl who presented with right sided focal seizures, hemiparesis of the same side, and delayed milestones.
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PMID:Dyke-Davidoff-Masson syndrome. 1902 69

The Dyke-Davidoff-Masson syndrome is characterized by various symptoms related to hemiatrophy of the cerebrum and hypertrophy of the ipsilateral calvarium and paranasal sinuses. Clinical findings include hemiparesis or hemiplegia, seizures and/or mental retardation. The present report discusses the very unusual case of a late-diagnosed Dyke-Davidoff-Masson syndrome in a 75-year-old body donor who had suffered a left-sided stroke associated with the internal carotid artery in the course of tonsillitis at the age of 5.
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PMID:Postmortal diagnosis of a Dyke-Davidoff-Masson syndrome in a 75-year-old woman---a case report. 1909 69

Dyke-Davidoff-Masson syndrome, or cerebral hemiatrophy, is a pre- or perinatally acquired entity characterized by predominantly neurologic symptoms, such as seizures, facial asymmetry, contralateral hemiplegia, and mental retardation. Psychiatric symptoms are rarely reported. We report the first case of left cerebral hemiatrophy and a late onset of treatment-resistant schizoaffective disorder after a stressful life event. The patient finally responded well to clozapine. The clinical history and results from structural neuroimaging are highlighted to discuss the possible developmental bias for psychotic disorders.
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PMID:Treatment-refractory schizoaffective disorder in a patient with dyke-davidoff-masson syndrome. 1916 86


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