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Diagnostic and Therapeutic Aspects of Hemiplegic Migraine

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Diagnostic and Therapeutic Aspects of Hemiplegic Migraine Migraine J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp-2020-322850 on 19 May 2020. Downloaded from REVIEW Diagnostic and therapeutic aspects of hemiplegic migraine Vincenzo Di Stefano ,1 Marianna Gabriella Rispoli,2 Noemi Pellegrino,3 Alessandro Graziosi,3 Eleonora Rotondo,3 Christian Napoli,4 Daniela Pietrobon,5,6 Filippo Brighina,1 Pasquale Parisi 7 1Department of Biomedicine, ABSTRact hypothesised a role for gene PRRT2 in migraine Neuroscience and Advanced Hemiplegic migraine (HM) is a clinically and genetically pathophysiology, but its specific implication in Diagnostic (BIND), University of HM is still debated. Given the lack of a recognised Palermo, Palermo, Sicilia, Italy heterogeneous condition with attacks of headache 2Department of Neuroscience and motor weakness which may be associated with fourth autosomal dominant gene for FHM, other Imaging and Clinical Sciences, impaired consciousness, cerebellar ataxia and intellectual genetic mechanisms might be supposed and further ’G. d’Annunzio’ University, disability. Motor symptoms usually last <72 hours and genetic analyses are needed.2 Sporadic hemiplegic Universita degli Studi Gabriele are associated with visual or sensory manifestations, migraine (SHM) shares similar clinical character- d’Annunzio Chieti e Pescara, Chieti Scalo, Chieti, Italy speech impairment or brainstem aura. HM can occur istics with FHM but it differs for the absence of 6 3Pediatrics, University Gabriele as a sporadic HM or familiar HM with an autosomal family history for HM. d’Annunzio of Chieti Pescara dominant mode of inheritance. Mutations in CACNA1A, As HM is a rare condition, few studies are Department of Medicine and ATP1A2 and SCN1A encoding proteins involved in ion reported and there are no specific treatments. In Aging Science, Chieti, Abruzzo, this review, we will discuss in detail about epidemi- Italy transport are implicated. The pathophysiology of HM is 4Department of Medical Surgical close to the process of typical migraine with aura, but ology, clinical presentation, diagnostic assessment, Sciences and Translational appearing with a lower threshold and more severity. We differential diagnosis and treatment of this complex Medicine, Sapienza University of reviewed epidemiology, clinical presentation, diagnostic disorder. Rome, Roma, Lazio, Italy assessment, differential diagnosis and treatment of 5Department of Biomedical Sciences & Padova Neuroscience HM to offer the best evidence of this rare condition. Epidemiology Center, University of Padova, The differential diagnosis of HM is broad, including Although migraine is a common condition with a Padova, Italy other types of migraine and any condition that can 6 prevalence between 15% and 20% in the general CNR Neuroscience Institute, cause transitory neurological signs and symptoms. population, HM is rare. The onset is generally in Padova, Italy 1 4 7 8 7Dipartimento di Neuroscienze Neuroimaging, cerebrospinal fluid analysis and adolescence between 12 and 17 years and the 9 Salute Mentale e Organi di electroencephalography are useful, but the diagnosis is overall estimated prevalence is 0.01%. Females are Senso (NESMOS), University clinical with a genetic confirmation. The management more frequently affected, with a variable female/ of Rome La Sapienza Faculty relies on the control of triggering factors and even male ratio among 2.5:1 and 4.3:1.9 10 The frequency of Medicine and Psychology, hospitalisation in case of long- lasting auras. As HM is a Roma, Lazio, Italy and severity of attacks progressively decrease with rare condition, there are no randomised controlled trials, increasing age.1 http://jnnp.bmj.com/ Correspondence to but the evidence for the treatment comes from small Professor Pasquale Parisi, studies. Clinical manifestations Dipartimento di Neuroscienze HM is characterised by recurrent attacks with Salute Mentale e Organi di headache and aura manifestations. Emotional and Senso (NESMOS), University of Rome La Sapienza Faculty of intense physical stress, viral infections and head Medicine and Psychology, Roma INTRODUCTION trauma are the more common reported trigger 11 12 00189, Lazio, Italy; pasquale . Hemiplegic migraine (HM) is an uncommon factors for HM attacks. Table 1 describes diag- parisi@ uniroma1. it on October 6, 2021 by guest. Protected copyright. subtype of migraine with aura that usually starts nostic criteria for HM, according to the definition 1 Received 21 January 2020 in the first or second decade of life. It is a clini- of the third edition of International Classification 3 Revised 22 April 2020 cally and genetically heterogeneous condition that of Headache Disorders. Accepted 24 April 2020 represents a challenge for the clinician because it Headache is almost always present during attacks Published Online First 19 May can occur with a dramatic and crippling clinical and it is often severe. The localisation of headache 2020 situation, resembling other more severe neurolog- is variable: bilateral, unilateral, ipsilateral or contra- 1 ical diseases (ie, stroke).1 2 lateral to the motor symptoms. HM can occur as a sporadic or familial condition Associated symptoms in HM can be distinguished if at least one first-degree or second- degree relative in aura symptoms that occur during attacks and © Author(s) (or their has the same form of migraine.3 Familial hemiplegic chronic symptoms that can be present interictally. employer(s)) 2020. Re- use permitted under CC BY-NC . No migraine (FHM) is the only migraine form for commercial re-use . See rights which an autosomal dominant mode of inheritance Aura symptoms and permissions. Published has been documented. Genetic studies have demon- Unilateral weakness is always present during HM by BMJ. strated the involvement of at least three distinct attacks and it is considered the most important To cite: Di Stefano V, Rispoli genes that encode proteins involved in ion trans- sign; weakness can rarely be bilateral and some- 2 MG, Pellegrino N, et al. J port (CACNA1A, ATP1A2 and SCN1A). Mutation times it may switch side. Besides motor weakness, Neurol Neurosurg Psychiatry of those genes explains for about 7%–14% of FHM sensory symptoms (such as tingling, numbness and 2020;91:764–771. in two population studies.4 5 Some authors have paraesthesia), visual defects (scintillating scotoma, 764 Di Stefano V, et al. J Neurol Neurosurg Psychiatry 2020;91:764–771. doi:10.1136/jnnp-2020-322850 Migraine J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp-2020-322850 on 19 May 2020. Downloaded from Table 1 Diagnostic criteria of hemiplegic migraine Diagnostic criteria ICHD-3 Hemiplegic migraine A. At least two attacks fulfilling criteria B. One or more of the following fully reversible aurasymptoms: 1. visual 2. sensory 3. speech and/or language 4. motor 5. brainstem 6. retinal C. At least three of the following six characteristics: 1. at least one aura symptom spreads graduallyover 5 minutes 2. two or more aura symptoms occur in succession 3. each individual aura symptom lasts 5–60 minutes 4. at least one aura symptom is unilateral 5. at least one aura symptom is positive 6. the aura is accompanied, or followed within 60 min, by headache D. Aura consisting of both of the following: 1. fully reversible motor weakness 2. fully reversible visual, sensory and/or speech/ language symptoms. Familial hemiplegic migraine (FHM) A. Attacks fulfilling criteria for Hemiplegic migraine B. At least one first- or second- degree relative has had attacks fulfilling criteria for Hemiplegic migraine. Familial hemiplegic migraine type 1 (FHM1) A. Attacks fulfilling criteria for Familial hemiplegic migraine B. A mutation on the CACNA1A gene has been demonstrated. Familial hemiplegic migraine type 2 (FHM2) A. Attacks fulfilling criteria for Familial hemiplegic migraine B. A mutation on the ATP1A2 gene has been demonstrated. Familial hemiplegic migraine type 3 (FHM3) A. Attacks fulfilling criteria for Familial hemiplegic migraine B. A mutation on the SCN1A gene has been demonstrated. Familial hemiplegic migraine, other loci A. Attacks fulfilling criteria for Familial hemiplegic migraine B. Genetic testing has demonstrated no mutation onthe CACNA1A, ATP1A2 or SCN1A genes. Sporadic hemiplegic migraine (SHM) A. Attacks fulfilling criteria for Hemiplegic migraine B. No first- or second- degree relative fulfils criteriafor Hemiplegic migraine. ICHD-3, third edition of International Classification of Headache Disorders. hemianopia) and aphasia are the most frequent aura symp- early onset CACNA1A- associated phenotype was proposed a toms. Sometimes, migraine attacks may include other signs and classification as ‘neurodevelopmental disorders’, suggesting thus symptoms such as fever, seizure, bilateral visual disturbances, a a close follow- up of psychomotor development and academic ‘brainstem aura’ with vertigo, dysarthria, ataxia, hyperacusia, performances.25 tinnitus, impaired consciousness and even, in the worse condi- Seizures in HM may be partial or generalised, with or without tions, coma.1 4 10 13 fever.26 Generally, the onset of epilepsy is in childhood and The duration of symptoms is usually 20–60 min but, in some sometimes it happens before the first HM attack. Hopefully, http://jnnp.bmj.com/ cases, the aura and motor deficit may onset quickly and simu- seizures in patients with HM have a benign evolution1 and higher late an ischaemic attack.8 The complete recovery from attacks is rates have been reported in families with FHM2.22 Of note, in the rule, but in severe
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