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)

Single photon emission computed tomography (SPECT) was performed 3 times during attacks and performed 4 times during postictal periods on a case of alternating hemiplegia of childhood. Hyperperfusion of the corresponding hemisphere to hemiparesis was suggested by asymmetric increase RI uptake during the ictal scans, whereas interictal scans showed symmetric topography of cerebral blood flow. Manifestations except hemiplegia included loss of consciousness and vomiting. These manifestations and ictal SPECT findings are the same evidence as hemiplegic migraine. It suggests that these two disorders have a similar pathophysiology. There are two interesting findings regarding this patient. The first finding is magnetic resonance imaging showed progressive cerebellar atrophy. Second finding is interictal SPECT showed a progressive decrease of cerebral perfusion, especially in cerebellar hemispheres. These two findings suggest alternating hemiplegia of childhood may be a chronic progressive disorder.
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PMID:[Single photon emission computed tomography findings in a case of alternating hemiplegia of childhood in relation to migraine]. 1616 48

Hemiplegic migraine is a rare subtype of migraine with aura associated with transient hemiplegia. The weakness is caused by motor aura. Hemiplegic migraine is the only headache syndrome associated with known genetic mutations and serves as a model for understanding more common varieties of migraine. Because the phenotype includes striking yet transient neurological signs and symptoms, it is imperative that clinicians know the differential diagnosis to rule out possible secondary etiologies when treating patients with hemiplegic spells. Hemiplegic migraine occurs with equal prevalence in either a sporadic or familial form differentiated only by family history. Thus far, treatment trials are anecdotal, although verapamil and acetazolamide have shown promise.
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PMID:Sporadic and familial hemiplegic migraine: diagnosis and treatment. 1662 31

We report here an 1-year-old boy who was diagnosed as sporadic hemiplegic migraine demonstrated transient changes in electroencephalographic (EEG) background activity accompanied with migraine attacks. The attacks were associated with various neurological complications such as impairment of consciousness and hemiplegia and so on, which coincided with the changes of EEG findings. When his first headache developed with fever and impairment of consciousness, diffuse slow waves (1-2 Hz, 250-300 microV) appeared transiently. The slow wave gradually improved thereafter, and disappeared on the 24th day. On the 25th day, when he had the second attack with left hemiplegia and impairment of consciousness and sensation, slow waves, (2-3 Hz, 250-300 microV) appeared on right side of the brain. Constriction of the right middle cerebral artery was found on MR angiography at that time. The EEG change had improved to normal background activity 3 days after the episode. This unique case demonstrated that transient EEG changes seem to be correlated with the existence of unconsciousness and the laterality of the ischemic insult. We may assume that the changes of electroencephalographic findings may represent the cortical dysfunction caused by vascular constriction.
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PMID:[Changes of electroencephalographic findings in a case of migraine with various neurological symptoms]. 1671 37

Prolonged hemiparetic migraine aura can cause diagnostic confusion and be mistaken for ischaemic stroke occurring during the course of a migraine--'migrainous infarction'. We report a case of prolonged hemiparesis occurring during the course of a migraine attack. Though initially confused with migrainous infarction, we suggest with sequential magnetic resonance imaging, magnetic resonance angiography, diffusion, perfusion images and magnetic resonance spectroscopy that the hemiplegia was not of vascular origin and that the patient had sporadic hemiplegic migraine. We hypothesize that the mechanisms of sporadic hemiplegic migraine probably lie at a cellular level, similiar to familial hemiplegic migraine.
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PMID:Imaging abnormalities in sporadic hemiplegic migraine on conventional MRI, diffusion and perfusion MRI and MRS. 1688 37

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 a case of a 15-year-old male who developed severe left-sided hemiplegia and hemi-sensory loss 20 minutes after arrival in post anaesthetic care unit following an uneventful general anaesthesia for removal of a cannulated screw of left hip. Initial CT and MRI/MRA with diffusion-weighted imaging were unremarkable. The patient was transferred to The Royal Children's Hospital Melbourne and was commenced on heparin infusion pending investigation results. Complete recovery occurred within 36 hours. Eventually, hemiplegic migraine was diagnosed on the basis of negative investigations and a retrospective history of the patient's migraine. This case demonstrates that the management of peri-operative neurological deficits must be approached in a systematic fashion. Hemiplegic migraine is a well-defined clinical syndrome. It is not merely a diagnosis of exclusion.
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PMID:Presentation of hemiplegic migraine--hemiplegia and hemi-sensory loss following general anaesthesia. 1759 Nov 40

A 20-year-old female with hemiplegic migraine was treated during an acute attack with intravenous verapamil, which reproducibly resolved the headache within 20 min but did not affect her hemiplegia. Magnetic resonance (MR) and computed tomographic (CT) angiography and perfusion performed during the attack showed vasodilation and hyperperfusion. Cerebral hyperperfusion concurrent with hemiplegia suggests a dissociation between cerebral perfusion and neuronal function in hemiplegic migraine. The beneficial effect of verapamil on headache but not hemiplegia suggests a distinct mechanism for pain and neuronal dysfunction in hemiplegic migraine, with the beneficial effect on pain not due to vasodilation.
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PMID:Hemiplegic migraine: hyperperfusion and abortive therapy with intravenous verapamil. 1761 29

Hemiplegic migraine is an unusual variant of migraine, characterised by a temporary hemiparesis or hemiplegia associated with headache. We report a patient with hemiplegic migraine who developed atypical migraine with apnoeic spells, aphasia and hemiparesis following general anaesthesia. We review the clinical features of hemiplegic migraine and the considerations for its anaesthetic management.
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PMID:Apnoeic spells following general anaesthesia in a patient with familial hemiplegic migraine. 1769 27

Sturge-Weber syndrome (SWS) is a sporadic disorder characterized by naevus (port wine stain), a pial angioma, and glaucoma. The angioma comprises abnormal tortuous vessels on the leptomeninges with underlying brain gliosis, calcification, and atrophy. The cerebral angioma is commonly unilateral but may be bilateral. Hemiplegia usually follows recurrent hemiconvulsions and may be related to venous stasis. The hemiplegia can be static, progressive, or fluctuating. Transient worsening of the hemiplegia can be seen with seizures and episodes resembling hemiplegic migraine. We report five patients (four females, one male) with SWS who have had transient worsening of hemiplegia following minor head injuries, occurring between the ages of 10 months and 12 years (median age 4y 6mo). An additional pilot survey suggests that this may affect up to 20% of patients.
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PMID:Transient exacerbation of hemiplegia following minor head trauma in Sturge-Weber syndrome. 1771 27

Hemiplegic migraine (HM) is a rare variety of migraine with aura characterized by the presence of a motor weakness during the aura. Hemiplegic migraine has two main forms according to the familial history: patients with at least one first- or second-degree relative who has aura including motor weakness have familial hemiplegic migraine (FHM); patients without such familial history have sporadic hemiplegic migraine (SHM). The prevalence of HM is one in 10,000 with FHM and SHM being equally frequent. Typical HM attacks include a motor weakness that is always associated with other aura symptoms, the most frequent being sensory, visual and speech disorders. In addition, basilar-type symptoms occur in up to 70% of the patients. Severe attacks may occur in FHM as well as in SHM with prolonged hemiplegia, confusion, coma, fever and seizures. The clinical spectrum also includes permanent cerebellar signs (nystagmus, ataxia, dysarthria) and less frequently various types of seizures and intellectual deficiency. FHM is the only variety of the autosomal dominant migraine and all three know genes encode ion-transporters. A genetic diagnosis is now possible by screening the three known genes involved in FHM (CACNA1A, ATP1A2 and SCNA1). Prognosis is usually good. Treatment is similar to approaches used in other varieties of migraine with aura, excepted for triptans that are contraindicated in MHF/MHS. Based on new pathophysiological insight, preventive treatments by various antiepileptic agents seem promising.
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PMID:[Familial and sporadic hemiplegic migraine]. 1840 71


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