Complex hyperkinetic movement disorders: an unusual presentation in multiple sclerosis

Abstract

Movement disorders (MD) in patients with multiple sclerosis (MS) has been few reported. The most common MD associated with MS are tremor and dystonia, but parkinsonism, myoclonus, chorea, ballism, paroxysmal dyskinesias and hemifacial spasm have been more rarely found in MS patients 1,2 . Complex hyperkinetic movement disorders (CHMD) is the term used to describe patients who present concomitantly more than one type of MD, although there is not always consensus as to the type of motor symptomatology 3 . We report on the first case of CHMD in a Brazilian patient with MS. CaseA 21-year old woman presented with acute left facial paresis and weakness in right upper limb. At age 27, she developed a sudden episode of visual loss, dysphagia, dysarthria, dizziness, incoordenation, loss of balance, weakness in left lower limb and gait difficulty. The diagnosis of MS relapse was made (EDSS score was 5.0), brain magnetic resonance imaging (MRI) demonstrated demyelinating abnormalities which fulfill the criteria for MS, and after treatment with intravenous methylprednisolone the symptoms partially remitted.However, during the following two years she presented progressive visual loss, weakness and postural instability associated to incapacitating involuntary movements, which restricted her to wheelchair (EDSS score was 8.0), despite treatment with interferon beta-1a (44 µg tiw). Neurological examination showed nystagmus; anarthria; facial paresis; muscle strength 4 in upper limbs and 3 in lower limbs (MRC score); arms and legs spasticity; brisk reflexes in the right leg; bilateral Babinski's sign; and CHMD characterized by no rhythmic tremor at rest (frequency of 3-4 Hz) which intensified during action in upper limbs (predominantly left), lower limbs (particularly in left) and jaw, cervical dystonia (spasmodic torticollis), ballistic movements in upper limbs, and left hemifacial spasms.The clinical features were thought to be consistent with secondary progressive form of MS and treatment with mitoxantrone was started (total dose of 80 mg for 9 months) which no reduce her disease progression (EDSS score was 9.5 after one year).