Complex movement disorder in an elderly patient and the chimera effect

Distúrbio do movimento complexo em uma paciente idosa e o efeito quimera

LETTERS

Complex movement disorder in an elderly patient and the chimera effect

Distúrbio do movimento complexo em uma paciente idosa e o efeito quimera

Marco A. T. Utiumi; Renato P. Munhoz; Caroline Cartaxo; Hélio A. G. Teive

Movement Disorders Unit, Neurology Service, Internal Medicine Department, Hospital de Clínicas, Federal University of Paraná (UFPR), Curitiba PR, Brazil

^{Correspondence}

Movement disorders (MD) are traditionally divided into hypokinetic syndromes or parkinsonism, and hyperkinetic syndromes, including several involuntary movements, such as chorea, ballism, tremor, tics and dystonia. Complex movement disorder (CMD) is the term used to describe patients presenting with more than one type of movement disorder concomitantly¹.

Here we describe an elderly female patient with parkinsonism, symptomatic epilepsy and dementia due to multiple brain infarctions, associated to hemichorea-hemiballismus.

CASE REPORT

An 81-year-old female presented to the Neurology Service with sudden onset of involuntary random movements affecting her right hemibody characterized as hemichorea-hemiballismus. She had a past medical history of multiple ischemic strokes progressing to vascular dementia and parkinsonism, with irregular use of levodopa/benserazide 200/50 mg: ½ tablet tid. Additionally, she had a diagnosis of epilepsy with complex partial and secondary generalized seizures, using phenobarbital (100 mg/day) irregularly. On clinical examination, she had a severe cognitive impairment (Mini-Mental Status Examination 15/30) and right hemichorea-hemiballismus. During the bedside examination, she suffered an episode of complex partial seizures and, after that, the hemichorea-hemiballismus completely disappeared. After the complex partial seizure ended, hemichorea-hemiballismus re-emerged. She was managed with intravenous phenytoin (20 mg/kg) followed by maintenance treatment with oral valproic acid. She progressed with improvement of both her seizures and hemichorea-hemiballismus. A new reassessment was performed after 48 hours, and left upper limb rigidity, rest tremor and bradykinesia were documented, confirming the clinical diagnosis of parkinsonism probable due to vascular cause. A cranial computed tomography scan demonstrated multiple areas of brain infarctions, cortical atrophy and diffuse supratentorial hydrocephalus ex vacuo.

DISCUSSION

This case report illustrates the peculiar phenomenology of the association of MD that are traditionally viewed as mutually exclusive phenomena – a hypokinetic (parkinsonism) and a hyperkinetic (hemichorea-hemiballismus) disorder, associated with epileptic seizures, probably reflecting the involvement of multiple motor modulation pathways, in cortical and subcortical areas. The unpredictability of the clinical manifestations of the basal ganglia lesions, sometimes bordering on the inexplicable, was pointed out earlier as the Marsden's paradoxes: (1) different pathological lesions affecting similar sites might produce different clinical manifestations; (2) similar lesions impairing various basal ganglia might manifest with the same signs and symptoms; (3) the same lesions affecting the same areas produce a determined symptom or can be asymptomatic; and (4) certain disease affecting the basal ganglia have a wide range of MD². In elderly patients, more susceptible to the occurrence of epilepsy, stroke is the most commonly associated risk factor, occurring in up to half of all cases with an identifiable etiology³. Hypokinetic and hyperkinetic MD can occur after an ischemic or hemorrhagic stroke in 1.0–3.7% of cases, notably hemichorea, hemiballismus and dystonia⁴. The neurological dysfunction of multiple domains in the same patient is a phenomenon that will probably accompany population aging and the increasing frequency of vascular events, polipharmacy and neurodegenerative diseases, among other factors^3-5. The unusual case presented here represents a combination of diseases, each one with different underlying characteristics, and we named it as the chimera effect, as an allusion to Greek mythology.

Received 27 December 2011

Accepted 03 January 2012

Conflict of interest: There is no conflict of interest to declare.

• 1. Bharucha KJ, Sethi KD. Complex movement disorders induced by fluoxetine. Mov Disord 1996;3:324-326.
• 2. Marsden CD. Motor dysfunction and movement disorders. In: Asbury AK, McKhann GM, McDonald WI (Eds). Diseases of the nervous system clinical neurobiology. Second Edition. Philadelphia: W. B. Saunders Company; 1992. p. 309-318.
• 3. Brodie MJ, Elder AT, Kwan P. Epilepsy in later life. Lancet Neurol 2009;8:1019-1030.
• 4. Black SE. Vascular cognitive impairment: epidemiology, subtypes, diagnosis and management. J R Coll Physicians Edinb 2011;41:49-56.
• 5. Handley A, Medcalf P, Hellier K, Dutta D. Movement disorders after stroke. Age Ageing 2009;38:260-266.

Publication Dates

• Publication in this collection
  16 May 2012
• Date of issue
  May 2012