Neurosurgical Considerations of Neurocutaneous Syndromes

Type III Sturge Weber Syndrome, An Uncommon Cause of Status Epilepticus

“…15 Imaging for characterization of SWS often starts with tomography, useful in this case because it helps to show calcifications. However, based on the ionizing radiation and limitation in the evaluation of brain parenchyma, the preferred method to evaluate this condition is contrastenhanced magnetic resonance imaging, 4,16,17 finding in the early phases of the SWS a transitory abnormal white matter signal, moreover, leptomeningeal enhancement is found because it is the most affected region, and limited diffusion can be found if there have been ischemic episodes. Late disease shows an increased signal in the T2 sequence related with gliosis and low enhancing of the pia mater and secondary brain parenchyma atrophy.…”

“…Medical management of seizures often has to be aggressive, and has included oxcarbazepine, because of a favorable rate of adverse effects reported, 4 in combination with carbamazepine or levetiracetam, particu-larly useful in patients with bilateral brain involvement; in cases when the patients have migraine-like headaches management could be complemented with valproic acid or topiramate. 1,4 Regarding surgical management, it is considered to have prophylactic potential, 16,17 and in up to 50% of the patients, hemispherectomy or a functional hemispherectomy can be performed with great outcomes regarding epilepsy control. 12,16,20 It was not considered in this patient because she had control of her epilepsy episodes.…”

“…1,4 Regarding surgical management, it is considered to have prophylactic potential, 16,17 and in up to 50% of the patients, hemispherectomy or a functional hemispherectomy can be performed with great outcomes regarding epilepsy control. 12,16,20 It was not considered in this patient because she had control of her epilepsy episodes. Management should also include anti-aggregation, usually with acetyl salicylic acid in dose between 3 and 5 mg/kg/d, because it helps to control seizures by diminishing the blood stasis 12 and may help to avoid hemiparesis and stroke-like episodes.…”

“…Management should also include anti-aggregation, usually with acetyl salicylic acid in dose between 3 and 5 mg/kg/d, because it helps to control seizures by diminishing the blood stasis 12 and may help to avoid hemiparesis and stroke-like episodes. 16,20 Considering the complexity of the SWS, management should be addressed by a multidisciplinary team, 16,19,20 where a proper clinical approach by the emergency and Patient with normal birth and development, who presented complex focal seizures since he was 9 mo. Those seizures were managed and controlled with valproic acid and clobazam during his initial 3 y.…”

Multidisciplinary neurocutaneous syndrome clinics: a systematic review and institutional experience

“…This patient population presents unique challenges in epilepsy treatment and has known indications for surgery and laser ablation. [15][16][17][18][19][20][21][22] SWS has also been shown to be associated with increased rates of drug-refractory epilepsy. 23 Although there is no known pathophysiological relationship between NF and tethered cord, there seems to be a correlation between the two.…”