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MedGenMed Neurology & Neurosurgery
Kinesigenic Paroxysmal Hemidyskinesia as the Initial Presentation of Multiple Sclerosis
Posted 10/04/2006

Yara Dadalti Fragoso, MD, MSc, PhD; Mauro Gomes Araujo, MD; Nilton Luiz Branco, MD

Abstract and Introduction
Abstract

Fewer than a hundred cases of paroxysmal dystonia have been described in patients with multiple sclerosis (MS). Even fewer cases of hemidyskinesia triggered by repetitive movements (paroxysmal kinesigenic hemidyskinesia - PKD) have been reported in MS patients. We describe the case of a woman, age 18 years at the onset of MS, and a man age 35 years at the onset of MS, who presented with PKD as the initial symptom. Magnetic resonance images (MRI) of these patients showed different areas of acute lesions possibly related to PKD; MRI of one of the patients demonstrated 1 lesion in the subcortical parietal area and another in the thalamic region and showed 2 lesions in the cervical spinal cord in the other patient.
Introduction

Except for tremor, movement disorders are relatively rare in patients with multiple sclerosis (MS).[1] Since the classic review of 83 cases of paroxysmal dystonia (PD) in MS described by Tanchant and colleagues in 1995,[1] fewer than 20 cases have been reported.

PD can be classified according to precipitating factors, phenomenology, duration of attacks, and etiology.[2] Paroxysmal kinesigenic dyskinesia (PKD) consists of sudden attacks of involuntary movements precipitated by sudden or repetitive movements,[2] or startling noise.[1] These attacks are typically short-lasting (up to 5 minutes), frequent (up to 100 times per day), and usually unilateral. They may be disabling, interfering with walking, working, and daily activities.[3] Before the onset of these attacks, some patients report tingling or paresthesia in the affected limb. These phenomena are called "sensory aura," and they led to an earlier theory that these were a form of partial epileptic seizure.[2]

PKD has been described in only a few patients with MS, and rarely as the presenting symptom of the disease.[1] PKD has not yet been associated with lesions in a definite area of the central nervous system. Indeed, the distribution of MS plaques in patients with PKD were as diverse as the cervical and thoracic spinal cord, midbrain, thalamus, periaqueductal region, internal capsule, and cerebral peduncle.[1]

We report 2 new cases of PKD as the presenting symptom of MS, with magnetic resonance image (MRI) showing plaques in different locations. Both cases were fully investigated in order to exclude other diagnoses, according to the protocol for Reference Centers on MS of the Ministry of Health in Brazil.

Case 1 Presentation

The first patient was a 35-year-old white man who worked as a manual laborer at a factory. Nine months before the initial consultation, the patient experienced several episodes of "painful cramps" in his right leg while walking fast. His right knee and elbow would then take on a flexed position which lasted roughly 1 minute. These attacks were diagnosed at another service as reflex partial epilepsy and the patient was prescribed carbamazepine 800 mg/day, with partial improvement. Brain computed tomography scan and electroencephalogram were normal. Five months later, while working with a pipe, performing a repetitive "rolling" movement of his wrists and hands, the painful cramp and flexed position of the right knee and elbow reappeared.

At no time in his history did he experience more than a couple of attacks in a day, and 2 to 3 days could pass without any attacks. When he was referred to our service, we performed MRI of the brain and spinal cord, and spinal fluid analysis. A few demyelinating lesions were observed in the brain (Figure, top), and 2 large lesions were seen in the cervical spinal cord (Figure, top). The spinal fluid showed elevated protein levels and a high percentage of gamma-globulin and IgG, with the presence of oligoclonal bands. The symptoms improved with pulsed intravenous methylprednisolone. The patient was started on glatiramer acetate and remained asymptomatic for 18 months. Subsequently, he developed blurred vision in the right eye, left hemiparesis, and severe dizziness. Another session of pulsed intravenous methylprednisolone led to complete remission of these symptoms. He has now been symptom-free for 8 months.