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[A case of MELAS presenting complex partial status epilepticus]
T Araki1, J Suzuki, Y Taniwaki
1Department Neurology, Kaizuka Hospital.
Rinsho Shinkeigaku = Clinical Neurology
|March 14, 2002
Summary
Complex partial status epilepticus can be the first sign of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS). Early recognition of these seizures in MELAS patients is crucial for timely intervention.
Area of Science:
- Neurology
- Mitochondrial Diseases
- Epilepsy
Background:
- Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) is a rare genetic disorder.
- Neurological manifestations in MELAS can be diverse, including seizures.
Observation:
- A 37-year-old male presented with complex partial status epilepticus as the initial symptom of MELAS.
- Clinical presentation included fluctuating consciousness, left homonymous hemianopsia, and conjugated eye deviation.
- Elevated blood and CSF lactic acid, ragged-red muscle fibers, and specific mitochondrial DNA mutation (A3243G) confirmed the diagnosis.
- MRI revealed T2-prolonged lesions in the right occipito-parieto-temporal lobes.
Findings:
- EEG monitoring showed high-amplitude, rhythmic sharp waves in the right parieto-temporo-occipital region during seizures.
- Seizures were characterized by conjugated eye deviation and consciousness loss, occurring every few minutes.
- Diazepam administration rapidly normalized EEG activity.
- Interictal EEG revealed frequent sharp waves in the right parietal and posterior temporal lobes.
Implications:
- Complex partial status epilepticus should be considered in MELAS patients presenting with fluctuating consciousness.
- This case highlights the importance of considering MELAS in adults with new-onset seizures and stroke-like symptoms.
- Accurate diagnosis and EEG monitoring are vital for managing neurological complications in MELAS.