Relapsing hemiplegy and drop attacks in a patient with Sturge-Weber syndrome-like central nervous system abnormalities

Aims: Following normal pregnancy and birth a 1.5-year-old male patient experienced relapsing hemiplegy with clonic jerks of the right part of the body. Headaches or unconciousness were never observed. In between the hemiplegic episodes the child did not show any neurologic abnormalities and developed normally.

Methods: An EEG performed during alertness and sleep as well as a lumbar puncture showed normal findings. A cMRI revealed moderate dilatation of the frontotemporal outer cerebrospinal fluid space. A therapy with benzodiazepines was only partly successful, the hemiplegic periods lasted in part for several hours. Continuous treatment with valproate, levitiracetam and oxcarbazepine was also not successful and the hemiplegic symptoms occurred two to six times in a month. A further diagnostic evaluation using angiographic cMRI and spectroscopy did not show any evidence of vascular events or metabolic disorder.

Results: A repeated cMRI with a contrast agent showed a progressive left-sided hemiatrophy with leptomeningeal angiomatosis at the age of 3.5 years. This radiographic finding was accompanied by a mild and continuous right-sided hemiparesis with symmetrical reflexes. At the age of 3 years, the child presented with sudden and short right-sided drop attacks which were not associated with unconciousness or any abnormalities in EEG.

Conclusion: Despite the lack of classical skin and ocular manifestations of Sturge-Weber syndrome, the patient's radiologic findings are well compatible with this disorder. Familial hemiplegic migraine (FHM) can also be considered in the differential diagnosis as the aunt of the patient was reported to suffer from FHM-like symptoms. A molecular genetic testing of the family for the three typical FHM genes was however unremarkable.

hemiplegy - dropattacks - leptomeningeal angiomatosis - Sturge-Weber syndrome