Paroxysmal Kinesigenic Dyskinesia: A Rare Neurological Disorder Posing Diagnostic Challenge but Therapeutic Rewards

Case Report

Austin J Clin Neurol 2015; 2(9): 1077.

Paroxysmal Kinesigenic Dyskinesia: A Rare Neurological Disorder Posing Diagnostic Challenge but Therapeutic Rewards

Jain RS¹, Prakash S²*, Nagpal K¹ and Handa R¹

¹Department of Neurology, SMS Medical College Hospital, India

²Neotia Getwel Health Care Centre, Siliguri, West Bengal, India

*Corresponding author: Swayam Prakash, Neotia Getwel Health Care Centre, Matigara, Siliguri-734010, India

Received: July 07, 2015; Accepted: August 09, 2015; Published: August 11, 2015

Abstract

Background: Paroxysmal kinesigenic dyskinesia (PKD) is one of the rare hyperkinetic movement disorder, characterized by brief, episodic hyperkinetic movement abnormalities like dystonia, chorea, hemiballismus or a combination, usually precipitated by sudden body movements, e.g. running, walking, standing, turning in bed, etc., lasting usually for a few seconds and followed by a variable period of refractoriness. By rule, consciousness is preserved and it usually responds well to antiepileptic drugs (AEDs).

Objective: To report clinical and demographic profiles of a series of three cases of sporadic PKD.

Observation: Three cases of idiopathic PKD, all male, two of them had onset in the second decade and one in the third decade, without a family history, with variable phenomenology, duration and precipitants but with remarkable response to carbamazepine.

Conclusion: A high index of suspicion should be maintained as diagnosis of PKD is largely clinical and accurate diagnosis is rewarded with remarkable therapeutic response with AEDs.

Keywords: PKD; PNKD; PED; PHD; Dykinesias; Hyperkinetic; Shoot-run; AEDs

Introduction

Paroxysmal kinesigenic dyskinesia (PKD) is one of the rare episodic hyperkinetic movement disorders, with typical childhood onset [1]. Other paroxysmal dyskinesias are paroxysmal nonkinesigenic dyskinesias (PNKD), paroxysmal exercise-induced dykinesias (PED) and paroxysmal hypnogenic dyskinesias (PHD) [2]. Family history is usually positive; however, sporadic cases are frequently reported [1,2]. We are reporting three cases of PKD with varying manifestations, without family history, and remarkable symptomatic improvement following treatment.

Case Presentation 1

A 22-year-old male, fresh paramilitary recruit, without any significant past, personal or family history presented with episodes of dystonic posturing, consisting of twisting and inward turning of left upper limb, turning of the head towards right and mild dysarthria but without loss of consciousness, at the start of ‘shoot-run’ (a run which entails very fast running from the word go and is a part of training of military and paramilitary recruits) (Figure 1/Video 1); for last two months, when he was inducted into such physical training. The episode abates completely within half or a minute after stopping and does not recur if he resumes running after subsidence of symptoms. This episode recurs only on restart of run after a period of quiescence. There is no history of occurrence of similar symptoms in other limbs; neither do these episodes occur with any activity other than ‘shootrun’.