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

We present a Norwegian family with familial hemiplegic migraine (FHM) with possibly four affected in three generations. The family had a point mutation in the ATP1A2 gene that caused a change of the amino acid valine to methionine (V628 M). The symptoms were pure FHM with intra- and interindividual variability, and epilepsy is not part of the clinical picture. Attacks could be provoked by physical activity. The proband had prolonged attacks of FHM, and was hospitalized due to such an attack provoked by a minor head trauma. The initial management was conservative, but due to persistence of the hemiplegia on day 9, a continuous nimodipine infusion was initiated in order to prevent cerebrovascular vasospasm. However, the nimodipine infusion worsened the patient's symptoms and possibly provoked a generalized tonic-clonic seizure due to vasodilatation and reduced cerebral blood flow. The MRI showed cortical edema and the SPECT showed reduced perfusion on the contralateral side of the hemiplegia. We conclude that nimodipine is contraindicated in the management of prolonged FHM attacks, and recommend conservative management and supplement of sufficient intravenous fluid in nauseated patients in order to avoid hypovolemia.
 ...
PMID:Intravenous nimodipine worsening prolonged attack of familial hemiplegic migraine. 1884 13

We describe three cousins who presented with agitation, dysphasia and/or coma, and developed hemiplegia following initial onset of symptoms. Two cases followed minor head injuries, two were pyrexial and two were associated with neutrophilia. Two cases required ventilatory support on the intensive care unit. Magnetic resonance imaging in all three cases showed cortical swelling, and one had evidence of restricted water diffusion on diffusion-weighted imaging, suggestive of ischaemia/infarction. A complete family history at the time of presentation would have led to an earlier diagnosis of profound encephalopathy in familial hemiplegic migraine, which would have enabled better prognostication of their clinical course and caused less distress for the families.
 ...
PMID:Profound encephalopathy with complete recovery in three children with familial hemiplegic migraine. 1931 60

We describe the case of a patient with atypical hemiplegic migraine and associated basilar symptoms, where a large patent foramen ovale (PFO) and hypoplasia of basilar artery were found. The longer period of 4-year remission of the headache attacks was coincident with the percutaneous PFO closure. When 5 years after, hemiplegic migraine attacks relapsed, with more relevant basilar symptoms, a mild re-opening of PFO was found. The atypical presentation of attacks with basilar symptoms and prolonged hemiplegia does not strictly fit the diagnostic criteria of ICHD-II.
 ...
PMID:A case of atypical sporadic hemiplegic migraine associated with PFO and hypoplasia of vertebro-basilar system. 1942 7

An association between hemiplegic migraine (HM) and episodic ataxia type 2 (EA2) has been described; both disorders are linked to mutations in the CACNA1A gene. Although confusion occurs in 21% of patients with HM, we found only one case in the literature of confusional episodes associated with ataxia without hemiplegia. These findings raise the possibility of confusional episodes being part of both the HM and EA2 phenotype. However, a patient with episodic ataxia, confusional spells and CACNA1A gene mutations has not been identified. We describe four individuals, spanning three generations of a family, with episodic ataxia without hemiplegia and confusion, in association with a CACNA1A mutation. We follow with a description of the relationship between the CACNA1A mutations and the three syndromes, suggesting a potential need for a new classification in which the conditions can be subsumed.
 ...
PMID:Link between confusional migraine, hemiplegic migraine and episodic ataxia type 2: hypothesis, family genealogy, gene typing and classification. 1962 85

Hemiplegic migraine (HM) in the setting of Sturge-Weber syndrome (SWS) has been previously described. Here, we report clinical and multimodal imaging data on a 21-year-old man with SWS and HM, who presented during an acute HM attack with a dense left-hemispheric syndrome (expressive aphasia and right sensorimotor hemiplegia), lasting for more than 10 days. Repeated EEGs were without evidence of status epilepticus. Consistent with previous findings in prolonged migraine aura, perfusion computed tomography demonstrated left-hemispheric hyperperfusion on day 7. 18F-FDG positron emission tomography (day 7) revealed left-hemispheric hypermetabolism. After 14 days, the patient was symptom-free and discharged home. Follow-up after 30 days showed normal neurological status. Our observation confirms and reinforces the comorbidity of SWS and HM and shows that prolonged HM attacks are associated with complex changes of both cerebral perfusion and glucose metabolism. A pathophysiological model explaining both the association between SWS/HM and the observed imaging changes is presented.
 ...
PMID:A case of Sturge-Weber syndrome with symptomatic hemiplegic migraine: clinical and multimodality imaging data during a prolonged attack. 1973 61

Hemiplegic migraine is a condition associated with vascular alteration of the central nervous system and transient neurologic deficits. Permanent morphine-induced motor dysfunction has been reported after spinal ischemia. We report a persisting central neurological deficit after single-dose spinal administration of 400 microg of morphine in a patient with no previous neurological condition. Hemiplegic migraine was thought to be present when the patient emerged from anesthesia. Weakness in the left quadriceps and saddle anesthesia of the perineum and urinary retention of central origin remained present 3 yr later. Over the 3 postoperative years, the patient was admitted to a neurological unit 5 times because of acute headache associated with complete left-sided hemiplegia. These symptoms resolved within 24 h of onset. Hemiplegic migraine was thought to be the most likely diagnosis of these recurrent attacks. We hypothesize that the patient's persistent deficits were caused by a combination of spinal morphine and spinal cord vascular dysfunction associated with hemiplegic migraine.
 ...
PMID:Central sensory-motor deficit after uneventful single-dose spinal morphine administration in a patient with preexisting migraine headaches. 1984 10

A 42-year-old patient with cognitive deficits due to childhood meningitis suffered from recurrent episodes of familial hemiplegic migraine. Additionally, he developed concomitant psychotic episodes requiring subsequent in-patient psychiatric treatment. Following combined neurological and psychiatric treatment he always recovered from the episodes within a few weeks time. Prophylactic treatment of migraine using topiramate and acetazolamide (off-label) prevented attacks for several months. When off-label compensation was refused and, as a consequence, the drug discontinued, hemiplegia relapsed within a few days. Hence, acetazolamide was prescribed again and the family paid for the medication. Since that time, the patient did not show severe attacks for at least 8 months apart from a transient attack induced by acute flu-like illness.
 ...
PMID:[Off-label use of acetazolamide in a patient with familial hemiplegic migraine and concomitant psychotic episodes]. 2014 82

We report a SPECT and PET voxel-based analysis of cerebral blood flow and metabolic rate for glucose in a 23-year-old woman with familial hemiplegic migraine (FHM) caused by ATP1A2 gene mutation. In comparison with healthy subjects, a PET scan showed brain glucose hypometabolism, controlaterally to the hemiplegia, in the perisylvian area early in the attack (Day 1), without any SPECT perfusion abnormalities. Decrease in metabolic rate was only partially reversible at Day 78, concordant at this time with a remaining hemisensory loss. These findings provide further evidence for a primary cortical metabolic dysfunction in FHM.
 ...
PMID:Partially reversible cortical metabolic dysfunction in familial hemiplegic migraine with prolonged aura. 2023 46

A female patient presented with horizontal gaze nystagmus, mild cerebellar ataxia, recurrent headache and hemiplegia since childhood with cerebellar atrophy on magnetic resonance imaging. Genetic analysis revealed a CACNA1A gene mutation, leading to a diagnosis of familial hemiplegic migraine (FHM1). FHM is very rare, but should be considered as a differential diagnosis for childhood cerebellar symptoms and/or cerebellar atrophy. To avoid missing FHM1, a detailed clinical history including headache or hemiplegia is essential. Oral acetazolamide during the aura phase, comprising mild headache and abnormal leg sensation, relieved these symptoms in this patient, suggesting that acetazolamide could represent a first line of treatment.
 ...
PMID:Genetic diagnosis and acetazolamide treatment of familial hemiplegic migraine. 2054 93

Hemiplegic migraine is a rare form of migraine variant. It is characterized by recurrent attacks of headache associated with temporary neurological deficit, usually unilateral hemiparesis or hemiplegia. It can be difficult to distinguish from migrainous stroke clinically, and a full neurological work-up and careful review of medical history and symptoms are necessary for the diagnosis. Two forms of hemiplegic migraine are known: familial and sporadic, phenotypically similar, differentiated by the absence of family history of similar attacks in the sporadic form. We report a case of sporadic hemiplegic migraine with unusually prolonged deficit and progression to quadriplegia with complete return to baseline 7 days after onset. Diffusion weighted images helped in excluding infarction.
 ...
PMID:Prolonged hemiplegic migraine. 2106 45


<< Previous 1 2 3 4 5 6 7 Next >>