TY - JOUR T1 - Diagnostic and therapeutic aspects of hemiplegic migraine JF - Journal of Neurology, Neurosurgery & Psychiatry JO - J Neurol Neurosurg Psychiatry DO - 10.1136/jnnp-2020-322850 SP - jnnp-2020-322850 AU - Vincenzo Di Stefano AU - Marianna Gabriella Rispoli AU - Noemi Pellegrino AU - Alessandro Graziosi AU - Eleonora Rotondo AU - Christian Napoli AU - Daniela Pietrobon AU - Filippo Brighina AU - Pasquale Parisi Y1 - 2020/05/19 UR - http://jnnp.bmj.com/content/early/2020/05/18/jnnp-2020-322850.abstract N2 - 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. ER -