Case Report: Chorea an unusual presentation of a patient with clinically isolated syndrome (multiple sclerosis)

Introduction

Multiple Sclerosis is a chronic disabling immune mediated inflammatory disease that affects the brain and the spinal cord.¹ Our patient presented with her first central nervous system demyelinating event which is known as clinically isolated syndrome which is a presentation that has features like multiple sclerosis but is isolated in time may or may not be isolated in space.⁴

Case report

Our patient was a 26-year-old university student studying arts in Jaffna who presented with choreiform movements of her upper and lower limbs of two months in duration. The movements affected her day-to-day activities and made her quite dependant on others. She had also developed two episodes of generalised tonic clonic seizures of 15 minutes duration one hour apart. There was no history of early morning headache or vomiting or upper and lower limb weakness. The patient had not developed any form of neurological weakness. There was a history of febrile illness and sore throat two weeks prior to the onset. During her illness she did not notice any joint pain or swelling or any rashes. There was no history of jaundice. She was not on any long term medications neither had she taken any medication both allopathic or ayruvedic recently. The patient did not use any recreational drugs, did not consume alcohol or use an ayruvedic medication. There was no history of psychiatric illness in her or her family. Patient was not on any oral contraceptives neither was she sexually active. There was no family history of neurological disease. There had been no antenatal or perinatal insult during her birth and her vaccination was up to date. She also developed a few episodes of generalised tonic clonic seizures lasting about 5 minutes each before and after admission.

Her examination findings were, she was conscious rational with a Glasgow coma score of 15/15. Her general exam did not reveal any icterus or rash, no stigmata of infective endocarditis. The respiratory, abdominal, and cardiovascular system examination were normal. In her neurological exam. On examination of both her fundus no papilledema was noted or no optic atrophy seen in both eyes. Swallowing was intact, her tongue had fasciculations and a jack in the box appearance. Motor examination revealed normal power and tone however she had abnormal movements more in the distal limb than in the proximal limbs. In the cerebellar exam she was not able to perform the finger nose test, heel shin she had dysdiadochokinesia in the left hand and the right hand and also the heel shin test was positive. Romberg's was positive and tandem gait was unstable. Deep tendon reflexes were +2 and symmetrical in the arms, the ankles and at the knees. Her plantar reflexes on both legs were down going. Pin prick, light touch and proprioception were normal. Romberg's was positive and tandem gait was unstable.

We had a few differential diagnoses in mind while investigating her mostly along the lines of diseases causing chorea in a young females such as Vasculitis, Wilsons disease, Hungtintons, hyperthyroidism. Eye referral done no Kayser Flecher rings were noted which is one of the eye signs noted in Wilson’s disease and as show below serum ceruloplasmin was normal which ruled out Wilson’s disease.

BLOOD INVESTIGATIONS

Full blood count

Erythrocyte Sedimentation rate (ESR)=25mm

Renal function Test

Liver function test

Imaging

Ultrasound abdomen= normal.

2DEcho=Normal no vegetations no valvular lesions

CT Brain: Multiple periventricular hypodensity? White matter disease

Visual evoked potentials = Markedly delayed bilaterally assuming there is no refractory error. The findings indicate demyelinating pathology affecting the visual pathway

The CT brain gave us a clue to the diagnosis and MRI and visual evoked potentials confirmed the diagnosis. Patient was given a course of methylprednisolone of 1g daily for three days and then started on prednisolone which was tailed off. Sodium Valproate 200 mg TDS and levecitracetum500mg daily was added for her seizures (Figure 1).

Figure 1. MRI Brain.

Peritrigonal centrum semiovale subcortical white matter signal intensity change. No contrast enhancement or diffusion restriction to suggest active lesions. Appearances are of white matter changes of Multiple Sclerosis.

Discussion

Multiple Sclerosis is a disabling disease affecting the brain and the spinal cord.² According to the National Multiple Sclerosis Society there are four subtype definitions Relapsing and remitting, secondary progressive, primary progressive and progressive relapsing other subtypes however have been identified. Clinically isolated syndrome is one such subtype.³ Clinical isolated syndrome refers to the first CNS demyelinating event that has features similar to Multiple sclerosis but is isolated in time may or may not be isolated in space.⁴

There are 5 different types of clinically isolated syndrome:

Type 1 which is clinically monofocal with atleast one asymptomatic lesion. Type 2 is clinically multifocal in nature with atleast one asymptomatic lesion. MRI lesion. Type 3 is clinically monofocal MRI may appear normal no asymptomatic MRI lesions. Type 4 clinically multifocal MRI may appear normal no asymptomatic MRI lesions Type 5 no clinical presentation to suggest demyelinating disease, but MRI is suggestive. The term clinically isolated syndrome ignores presentations that may be found only on laboratory data and paraclinical findings. Although a kind of exception to this is CIS type 5 where patients have nonspecific symptoms such as headache and dizziness but have MRI evidence of multifocal abnormalities of demyelination.⁵

Some of the clinical presentations of CIS include optic neuritis, a brain stem and or cerebellar syndrome, a spinal cord syndrome, or occasionally cerebral hemispheric dysfunction. We should also probe about whether the patient had some neurological weakness in the past, any blurring of vision, L hermitte sign, numbness or neurological weakness lasting about more than 24 hours. Questions about infections, inflammatory disorders, metabolic, genetic disorders, vascular and autoimmune diseases should also be sought.⁶

Chorea is rather an unusual manifestation of multiple sclerosis because multiple sclerosis is thought to be a white matter disease however there is evidence to prove now that it can involve both the cortical and sub cortical gray matter and the latter has myelinated fibers which may explain the involvement of the striatum, pallidum, thalamus and brainstem in multiple sclerosis.¹⁰ Usually multiple sclerosis patients may present with a tremor but movement disorders are less frequently reported.⁸ Giada Giovanni et al have reported on a case of hemichorea in a 39 year old women who had MRI evidence of multiple sclerosis and the CSF was positive for oligoclonal bands.⁹ Our patient presented to us with choreiform movements and she was diagnosed of having clinically isolated syndrome because of the lack of previous episodes of neurological presentations. There was sufficient evidence in her MRI; the lesions were suggestive of multiple sclerosis and her visually evoked potential showed marked delaying bilaterally indicating demyelinating pathology. In our patient oligo clonal bands were negative and in a study done by Jaganndha R et al showed that absence of oligo clonal bands is associated with a better prognosis in multiple sclerosis.¹⁰

Usually episodes of multiple sclerosis are mild and resolve without medication, severe visual loss, pain in optic neuritis, pronounced motor dysfunction, ataxia, vertigo in spinal or brainstem syndromes are some of the symptoms that indicate that treatment is needed. Corticosteriods are used when symptoms are functionally disabling as in our case. A combination of intravenous methylprednisolone and oral steroids have be noted to lower the risk of developing clinically definite multiple sclerosis in the following two years.⁷

Multiple sclerosis can manifest in different ways and although it is predominantly a white matter disease it can affect the subcortical gray matter explaining the presentation of chorea in this young lady.

Consent

Written informed consent for the publication of the case report and any associated images was sought from the patient prior to submission.