Panic attacks in girls with double cortex syndrome: ictal amaurosis

Case Report: Within the last 3 year we saw seven inpatients with double cortex syndrome at our Department in Kork. Five out of seven girls had a history of visual auras which were accompanied by panic attacks in two. We report of a girl with impressive transient MRI findings supporting the hypothesis of visual auras.

The 15-year old girl with mild to moderate cognitive impairment presented with a typical double cortex syndrome in MRI. There were no other affected relatives and her development was primarily retarded. At the age 4 years a first generalized tonic-clonic seizure occurred. In the following year she suffered from repetitive grand-mal and by the age of 7 years increasing and more dyscognitive seizures with staring and cyanosis appeared. Attacks with complete loss of vision for several minutes were reported. However, no prophylaxis with anticonvulsive drugs was started and seizures were treated with acute benzodiazepine administration. At age 13/8 years she had a day with several seizures with confusion and repetitive eye deviation. Because of panic attacks she was hospitalized for the first time. Prolonged dyscognitive seizures (atypical absences), headaches, vertigo, and eye deviations to the left were recognized. MRI 5 days after admission showed a marked edema on T2 images in the right parieto-occipital region. Control after 8 days revealed a complete recovery in MRI. After 6 months she was again admitted to hospital with an episode of several days with repetitive visual auras, with suspected amaurosis and vertigo. An acute MRI demonstrated again an edema in the identical right parieto-occipital area.

Conclusion: Auras are a common seizure manifestation in lissencephaly. To our experience, visual auras are frequent. They may lead to panic attacks that can be falsely interpreted as behavioral problems, especially in mentally handicapped patients.