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Marfan's syndrome, recurrent complex partial status epilepticus and myoclonus: a case report
G Ambrosetto1, P Tinuper, C A Tassinari
1Institute of Neurology, University of Bologna, Italy.
Clinical EEG (Electroencephalography)
|January 1, 1988
Summary
Recurrent complex partial status epilepticus may cause permanent neurological deficits, particularly in patients with pre-existing brain damage. This condition, seen in a patient with Marfan syndrome, highlights potential long-term risks.
Area of Science:
- Neurology
- Genetics
- Epileptology
Background:
- Marfan syndrome is a genetic disorder affecting connective tissue.
- Epileptic seizures and neurological complications can occur in Marfan syndrome.
- Complex partial status epilepticus is a prolonged seizure state.
Observation:
- A 26-year-old woman with Marfan syndrome and intellectual disability experienced recurrent complex partial status epilepticus.
- Myoclonus at rest and transient cerebellar dysfunction worsened after each seizure episode.
- The patient had a two-year history of weekly seizures.
Findings:
- The recurrent seizures may have led to persistent neurological deficits, including myoclonus and cerebellar signs.
- The neurological deficits could be a consequence of Marfan syndrome, the seizures, or both.
- This case suggests a link between prolonged seizure status and permanent neurological damage.
Implications:
- Recurrent complex partial status epilepticus might be a risk factor for permanent neurological deficits.
- Patients with underlying brain conditions may be more vulnerable to seizure-induced damage.
- Further research is needed to understand the long-term impact of status epilepticus in syndromic patients.