What is the appropriate management and treatment for a patient diagnosed with Monmeig syndrome, considering the potential for seizures, headaches, and visual disturbances?

Meige Syndrome: Management and Treatment

What is Meige Syndrome?

Meige syndrome is a type of cranial dystonia characterized by involuntary spasms of the eyelids (blepharospasm) and lower facial muscles, jaw, tongue, and mouth (oromandibular dystonia), which can spread to involve pharyngeal and cervical muscles over time 1. Blepharospasm typically appears first and progressively involves other cranial and extra-cranial muscles 1.

Primary Treatment: Botulinum Toxin Injections

Most patients with Meige syndrome are successfully treated with botulinum toxin injections, which should be the first-line therapy 1. This treatment targets the specific muscles involved in the dystonic movements and provides symptomatic relief in the majority of cases 1.

Identifying Primary vs. Secondary Causes

• Primary (idiopathic) Meige syndrome occurs when no underlying cause is identified and represents the majority of cases 1
• Secondary Meige syndrome can result from prolonged neuroleptic use or underlying brain disorders 1
• Evaluate medication history specifically for antipsychotic or dopamine-blocking agents, as these are common precipitants 1
• Consider neuroimaging (brain MRI) if there are focal neurologic findings, rapid progression, or features suggesting secondary causes 1

Management of Associated Conditions

Seizure Management

• If seizures occur, investigate for provoked causes including hypocalcemia, hypomagnesemia, fever, or medications 2
• Measure calcium, parathyroid hormone, magnesium, and electrolytes, as hypocalcemia can trigger seizures and may arise at any age 2
• Correct underlying electrolyte disturbances (hypocalcemia, hypomagnesemia) as primary treatment before or concurrent with antiepileptic therapy 2
• Use standard antiepileptic drugs tailored to seizure type, with a "start low, go slow" approach due to potential increased medication sensitivity 2
• Levetiracetam is preferred for generalized tonic-clonic seizures if they occur 3

Headache and Visual Disturbances

• Evaluate for concurrent movement disorders or neurologic conditions, as Meige syndrome has been described in patients with essential tremor, Parkinson's disease, and atypical Parkinsonism 1
• Visual disturbances may result from severe blepharospasm causing functional visual impairment 1
• Headaches should be evaluated for migraine features, as migraine management may be beneficial in some patients with cranial dystonia 4

Refractory Cases: Deep Brain Stimulation

For patients who fail to respond adequately to botulinum toxin injections, deep brain stimulation has emerged as an effective therapeutic option 1. This should be considered in consultation with a movement disorders neurologist for intractable cases 1.

Neurophysiological Considerations

• Meige syndrome shares neurophysiological features with other focal dystonias, characterized by abnormal plasticity and impaired inhibition 1
• These features help distinguish it from other movement disorders and support the diagnosis 1

Multidisciplinary Consultation

• Refer to a movement disorders neurologist for definitive diagnosis and management planning 2
• Consider neurology consultation for patients with suggestive features or symptoms refractory to initial treatment 2

Common Pitfalls to Avoid

• Do not confuse Meige syndrome with Maigne syndrome (a musculoskeletal cause of low back pain) or Meigs syndrome (ascites and pleural effusion with ovarian tumors) 5, 6
• Do not overlook medication-induced secondary dystonia, particularly from neuroleptics, as discontinuation may be curative 1
• Do not delay botulinum toxin treatment while pursuing extensive workup in typical presentations 1
• Do not miss hypocalcemia as a treatable cause if seizures develop, even in patients with previously normal calcium levels 2