546792

research-article2015

MSJ0010.1177/1352458514546792Multiple Sclerosis JournalJB Lilleker, C Gall

Multiple Sclerosis Journal 21(2) Multiple Sclerosis Journal 2015, Vol. 21(2) 256­–258 DOI: 10.1177/ 1352458514546792 © The Author(s), 2015. Reprints and permissions: http://www.sagepub.co.uk/ journalsPermissions.nav

Correspondence to: JB Lilleker Department of Neurology, Royal Preston Hospital, Sharoe Green Lane, Preston, PR2 9HT, UK. [email protected] JB Lilleker C Gall R Dayanandan SK Chhetri Royal Preston Hospital, Preston, UK HCA Emsley Royal Preston Hospital, Preston, UK/University of Manchester, Manchester, UK

Paroxysmal dysarthria ataxia syndrome responds to lacosamide JB Lilleker, C Gall, R Dayanandan, SK Chhetri and HCA Emsley

Dear Editors, A previously healthy 20-year-old gentleman, born in the UK of Pakistani parents presented with an 8-week history of stereotyped episodic speech disturbance. Each episode was characterised by severe slurring of speech occurring synchronously with difficulty controlling the right arm and leg. The episodes had initially occurred daily, but over the course of 4 weeks the frequency had increased to several per hour. Between episodes, neurological examination was notable only for mild spasticity in the right arm and leg. During observed episodes, which were of abrupt onset and cessation and lasting for approximately 20 seconds, there was marked dysarthria in combination with right upper limb dysmetria and gait ataxia. A magnetic resonance imaging scan of the brain and spinal cord demonstrated widespread lesions, including within the medial midbrain, compatible with demyelination. Cerebrospinal fluid analysis revealed slight elevation of white blood cells (7 x 10^6/L) and unpaired oligoclonal bands consistent with central nervous system (CNS) demyelination. Visual evoked potentials and electroencephalograms (ictal and interictal) were normal. Based on the clinical presentation and investigation results, a working diagnosis of paroxysmal dysarthria ataxia (PDA) syndrome secondary to CNS demyelination was made, prompting treatment with 1g intravenous methylprednisolone daily for 3 days followed by an oral prednisolone taper.

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remyelinating axons, usually within the medial midbrain, which may interrupt cerebello-thalamo-cortical pathways and cause a parietal diaschisis.1,2 Beneficial effects are most often noted in response to treatment with carbamazepine, but have also been described with phenytoin, lamotrigine and acetazolamide.3 In our patient, levetiracetam was ineffective and further use of carbamazepine was prevented by side effects, despite the apparent beneficial effect. The use of lamotrigine was discounted given the possibility of cross-reactivity and consequent worsening of rash.4 Lacosamide was considered given its effect on neuronal membrane sodium channels, and its use in this patient coincided with complete cessation of further attacks. Lacosamide is a newer antiepileptic drug which selectively enhances slow inactivation of voltage-gated sodium channels. To our knowledge this is the only reported use of lacosamide in a patient with PDA. The beneficial effect observed in this case warrants consideration of its use in patients with PDA and other paroxysmal neurological symptoms. Conflict of interest None declared. Materials Informed written consent obtained for publication of clinical details and supporting materials. Ethical consent not required.

Carbamazepine was commenced, and led to a modest reduction in frequency and severity of episodes, but was associated with a severe skin rash requiring cessation after only two doses. Levetiracetam was trialled with no improvement achieved. Lacosamide 50 mg bd was commenced and was associated with almost complete cessation of episodes after the second dose.

References

Transient neurological symptoms, including PDA, are well recognised in multiple sclerosis (MS) and can be the only manifestation of acute relapse.1 In patients with PDA secondary to MS other authors have hypothesised that symptoms are generated by ephaptic transmission in an area of demyelinated or

1.

Iorio R, Capone F, Plantone D, et al. Paroxysmal ataxia and dysarthria in multiple sclerosis. J Clin Neurosci 2014; 21: 174–175.

2.

Blanco Y, Compta Y, Graus F, et al. Midbrain lesions and paroxysmal dysarthria in multiple sclerosis. Mult Scler 2008; 14: 694–697.

3.

Klaas JP, Burkholder DB, Singer W, et al. Harry Lee Parker and paroxysmal dysarthria and ataxia. Neurology 2013; 80: 311–314.

4.

Hirsch LJ, Arif H, Nahm EA, et al. Cross-sensitivity of skin rashes with antiepileptic drug use. Neurology 2008; 71: 1527–1534.

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Paroxysmal dysarthria ataxia syndrome responds to lacosamide.

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