Diagnostic and therapeutic aspects of hemiplegic migraine

被引:92
作者
Di Stefano, Vincenzo [1 ]
Rispoli, Marianna Gabriella [2 ]
Pellegrino, Noemi [3 ]
Graziosi, Alessandro [3 ]
Rotondo, Eleonora [3 ]
Napoli, Christian [4 ]
Pietrobon, Daniela [5 ,6 ,7 ]
Brighina, Filippo [1 ]
Parisi, Pasquale [8 ]
机构
[1] Univ Palermo, Dept Biomed Neurosci & Adv Diagnost BIND, Palermo, Sicilia, Italy
[2] Univ Gabriele dAnnunzio Chieti & Pescara, G dAnnunzio Univ, Dept Neurosci Imaging & Clin Sci, Chieti, Italy
[3] Univ Gabriele dAnnunzio Chieti Pescara, Dept Med & Aging Sci, Pediat, Chieti, Abruzzo, Italy
[4] Sapienza Univ Rome, Dept Med Surg Sci & Translat Med, Rome, Lazio, Italy
[5] Univ Padua, Dept Biomed Sci, Padua, Italy
[6] Univ Padua, Padova Neurosci Ctr, Padua, Italy
[7] CNR, Neurosci Inst, Padua, Italy
[8] Univ Roma La Sapienza, Dipartimento Neurosci Salute Mentale & Organi Sen, Fac Med & Psychol, I-00189 Rome, Lazio, Italy
关键词
headache; EEG; ion transport; clinical neurology; CLINICAL SPECTRUM; ABORTIVE THERAPY; AURA FREQUENCY; SEVERE ATTACKS; MUTATIONS; CACNA1A; ATP1A2; EPILEPSY; PATIENT; STROKE;
D O I
10.1136/jnnp-2020-322850
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Hemiplegic migraine (HM) is a clinically and genetically heterogeneous condition with attacks of headache and motor weakness which may be associated with impaired consciousness, cerebellar ataxia and intellectual disability. Motor symptoms usually last <72 hours and are associated with visual or sensory manifestations, speech impairment or brainstem aura. HM can occur as a sporadic HM or familiar HM with an autosomal dominant mode of inheritance. Mutations in CACNA1A, ATP1A2 and SCN1A encoding proteins involved in ion transport are implicated. The pathophysiology of HM is close to the process of typical migraine with aura, but appearing with a lower threshold and more severity. We reviewed epidemiology, clinical presentation, diagnostic assessment, differential diagnosis and treatment of HM to offer the best evidence of this rare condition. The differential diagnosis of HM is broad, including other types of migraine and any condition that can cause transitory neurological signs and symptoms. Neuroimaging, cerebrospinal fluid analysis and electroencephalography are useful, but the diagnosis is clinical with a genetic confirmation. The management relies on the control of triggering factors and even hospitalisation in case of long-lasting auras. As HM is a rare condition, there are no randomised controlled trials, but the evidence for the treatment comes from small studies.
引用
收藏
页码:764 / 771
页数:8
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