What is the management of Dyke-Davidoff-Masson syndrome?

Management of Dyke-Davidoff-Masson Syndrome

The management of Dyke-Davidoff-Masson Syndrome (DDMS) should focus on controlling seizures with appropriate antiepileptic medications as the primary intervention, with a multidisciplinary approach addressing associated neurological deficits and comorbidities.

Clinical Presentation and Diagnosis

DDMS is a rare neurological condition characterized by:

• Cerebral hemiatrophy/hypoplasia
• Ipsilateral ventriculomegaly
• Calvarial thickening and hyperpneumatization of the frontal sinuses
• Clinical manifestations including:
  • Seizures (most common presenting symptom)
  • Contralateral hemiparesis/hemiplegia
  • Facial asymmetry
  • Intellectual disability
  • Developmental delays
  • Behavioral problems 1, 2

Diagnosis is primarily based on neuroimaging (CT or MRI) showing characteristic findings of cerebral hemiatrophy, ipsilateral ventriculomegaly, and associated bone abnormalities 2.

Treatment Approach

1. Seizure Management

• Antiepileptic drug therapy is the cornerstone of management:
  • Combination therapy with valproate and carbamazepine has shown effectiveness in reducing seizure frequency and duration 3
  • Other antiepileptic medications that may be used include topiramate 3
  • Medication selection should be based on seizure type and patient characteristics
  • Aim for seizure control while minimizing side effects

2. Neurological Management

• Regular neurological assessment to monitor disease progression
• Physical therapy and rehabilitation for hemiparesis/hemiplegia
• Occupational therapy for improving daily functioning
• Speech therapy for communication difficulties if present

3. Cognitive and Behavioral Management

• Cognitive rehabilitation for intellectual disability
• Behavioral therapy for managing behavioral problems 4
• Special education programs for learning difficulties
• Psychological support for patients and families

4. Surgical Intervention

• Hemispherectomy may be considered in carefully selected patients with intractable seizures 2
• Surgical intervention should be evaluated on a case-by-case basis by a neurosurgical team

Monitoring and Follow-up

• Regular follow-up with neurology for seizure control assessment
• Periodic neuroimaging to monitor disease progression
• Neuropsychological assessment to track cognitive function
• Adjustment of antiepileptic medications as needed

Special Considerations

• Early diagnosis and treatment are crucial to prevent intellectual decline 3
• Substance use disorders may coexist and require specific interventions 4
• Patients with crossed cerebro-cerebellar atrophy may require more complex management 5

Prognosis

The prognosis depends on the extent of brain damage, age of onset, and effectiveness of seizure control. Early identification and treatment can improve patient outcomes and quality of life 1.

Multidisciplinary Care Team

Management should involve coordination between:

• Neurologists
• Neurosurgeons (if surgical intervention is considered)
• Psychiatrists (for behavioral issues)
• Physical, occupational, and speech therapists
• Special educators
• Psychologists
• Social workers

Early intervention with appropriate antiepileptic medication and comprehensive rehabilitation can significantly improve the quality of life for patients with DDMS, even though complete resolution of symptoms may not be possible.