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Título

PAROXYSMAL DYSTONIA AS ATYPICAL INITIAL MANIFESTATION OF MULTIPLE SCLEROSIS

RESUMO

CASE PRESENTATION: A 14-year-old male with autism spectrum disorder started with gait disturbance and vertigo, evolving with tingling in his hands and feet, in addition to several daily episodes of sustained and painful flexion of the left upper limb, lasting 1-2 minutes. On examination, he had dystonic posture in the left hand and forearm, hyperactive patellar reflex, and bilateral clonus. No changes in cranial nerves, strength, sensitivity and coordination. Therapy with carbamazepine showed progressive improvement of dystonic spasms. Investigation demonstrated CSF with oligoclonal type 2 bands and cranial resonance with T2/FLAIR hypersignal in the juxtacortical, periventricular, calloseptal and bulbar paramedian interface and gadolinium enhancement in the corpus callosum. He was diagnosed with Multiple Sclerosis (MS) and treated with steroid pulse therapy followed by plasma exchange. At discharge, the patient was asymptomatic, with complete improvement of dystonia. DISCUSSION: Paroxysmal symptoms in MS are a heterogeneous group of motor, sensory, cerebellar, or brainstem changes, characterized by repetitive, stereotyped and short episodes that can last from days to months. The incidence is 1.6 to 17% in MS cases, of which 24% are the initial manifestations. The most important are dyskinesia, myokymia, lhermitte sign, trigeminal neuralgia, vertigo, diplopia and ataxia. Paroxysmal dystonia is an involuntary, abrupt and also stereotyped movement. It may be painful or not, kinesiogenic or not, focal or generalized. Although not completely understood, it is speculated that this phenomenon may occur due to erratic transmission of ectopic nerve impulses by partially demyelinated axons. These may present a dysfunction in ion channels, which generates neural desensitization to stress factors. This is also strengthened by its good therapeutic response to anticonvulsant drugs. Furthermore, its location occurs due to lesions in the motor pathway, such as in the posterior horn of the internal capsule, basal ganglia, brainstem and cervical spinal cord. We report a case of a patient who presented with a first onset of MS associated with dystonic spasm, with dramatic improvement of dystonia after the introduction of carbamazepine. CONCLUSION: Paroxysmal symptoms are uncommon in MS, posing a diagnostic challenge. Recognition as a possible manifestation of MS is of fundamental importance, as it can avoid delay in diagnosis and provide adequate early treatment.

Palavras Chave

MULTIPLE SCLEROSIS, DYSTONIA, PAROXYSMAL SYMPTOMS