Meige's Syndrome: Rare Neurological Disorder Presenting as Conversion Disorder

Abstract

Meige's syndrome is a rare neurological syndrome characterized by oromandibular dystonia and blepharospasm. Its pathophysiology is not clearly determined. A 35-year-old female presented to psychiatric department with blepharospasm and oromandibular dystonia with clinical provisional diagnosis of psychiatric disorder (Conversion Disorder). After thorough physical examination including detailed neurological exam and psychiatric evaluation no formal medical or psychiatric diagnosis could be made. The other differential diagnoses of extra pyramidal symptom, tardive dyskinesia, conversion disorder, anxiety disorder were ruled out by formal diagnostic criteria. Consequently with suspicion of Meige's syndrome she was referred to the department of Neurology and the diagnosis was confirmed. Hence, Meige's syndrome could be misdiagnosed as a psychiatric disorder such as conversion disorder or anxiety disorder because clinical features of Meige's syndrome are highly variable and affected by psychological factors and also can be inhibited voluntarily to some extent.

INTRODUCTION

Meige's syndrome or Brughel syndrome is a rare neurological disorder characterized by involuntary and often forceful contraction of the muscles of jaw (oromandibular dystonia) and involuntary muscle spasm and contraction of muscles around eyes (blepharospasm). The disorder is named after Henri Meige in 1910, the French Neurologist who first described the syndrome.[1] Its pathophysiology is not clearly known.

CASE REPORT

A 35-year-old female presented to the Department of Psychiatry with complaints of sideways deviation of the jaw since one and half year. At the beginning, the deviation was 4-6 times a day and was not causing much difficulty for the patient. Gradually frequency increased and she developed chin thrusting movement. She also developed increased rate of blinking and these movements were very embarrassing to her. With a progressive course the severity of symptoms increased causing difficulty in sleep and problem in daily routine activities. She could voluntarily inhibit the symptoms to some extent.

The symptoms were precipitated with the emotional stressors, suggesting some secondary gain. No relevant past or family history of any medical or psychiatric illness was found. She was educated up to class fourth. Premorbid personality was well adjusted. Mental status examination shows she had anxious mood. There was no formal thought and perceptual disorder. She was well oriented, alert, had normal fluency, repetition and naming. Her attention, calculation and abstraction were absolutely normal. General physical exam was unremarkable. Neurological exam showed intact cranial nerve, coordination, motor and sensory fun normal. Ophthalmological exam showed no visual defect, absence of Kayser Fleischler ring. Hematological examination–Haemoglobin, Differential Count, Total Leukocyte Count etc., were normal. Blood sugar, Liver Function Test, Thyroid function test, Computed Tomography Scan and Magnetic Resonance Imaging was within normal limit. Psychological assessment that is chemical and verbal abreaction could not reveal anything. The patient was sent to the neurologist for opinion and diagnosed as a case of Meige's syndrome. She was prescribed Trihexyphenidyl and Clonazepam. There was a little improvement on next follow-up.

DISCUSSION

The clinical features in this case were suggestive of conversion disorder. However, points against diagnosis of conversion disorder were- long duration, presence of symptoms at difficult position, persistence of symptoms at sleep. Since, there was no history of any antipsychotic use, possibility of extrapyramidal symptom or tardive dyskinesias were ruled out. According to DSM IV TR criteria, anxiety disorder was also ruled out.

Oromandibular dystonia and blepharospasm is referred as Meige's syndrome. Symptoms usually begin between ages 30and70 year and more common in woman than man (2:1). Symptoms comprise of Oromandibular symptoms (difficulty opening mouth, grinding of teeth, spasm of jaw opening, sideways deviant of jaw, lip tightening) and Blepharospasm symptoms (increased rate of blinking, uncontrollable squinting, photophobia).[2]

Sometimes dystonia may be provoked by talking, chewing, biting and may be alleviated by chewing gum, placing tooth pick, applying pressure over chin. Generally involved muscles are masseter, temporalis and platysma.[3] The exact cause is not known, however, hypothesis of dopaminergic and cholinergic hyperactivity is most widely accepted. Current medical research says that the condition is caused by a defect in a large network of brain cells especially in basal ganglia. It is commonly misdiagnosed. Usually a neurologist specializing in movement disorder detect Meige's syndrome. No other definite test to detect the syndrome. Symptoms usually disappear with a sleep and present at activity, with some variation with a stress. There is no complete cure for Meige''s syndrome.[4] Botulinum toxin injections in the facial musculature are considered the most effective treatment to date. Although the effects are temporary, the intramuscular injections provide rapid symptom relief.[5]

CONCLUSION

The case is reported to emphasize the point that motor movement along with blepharospasm for long duration having definite stressors warrants detailed evaluation. Meige's Syndrome, a rare neurological disorder should be considered as differential diagnosis of conversion disorder, like other neurological disorder presenting with Conversion symptoms e.g. multiple sclerosis, myestenia gravis.