Coexistence of epilepsy and Sandifer syndrome in a child with cerebral palsy

Introduction: In cerebral palsy various motor paroxysms (MPs) with or without loss of consciousness can be found (involuntary dystonia, dyskinesia, tonic neck reflex, epileptic attacks, stereotypies, medication induced acute dystonia). There are also MPs related to feeding and the commonly associated gastroesophageal reflux disorder (GERD). Sandifer syndrome (SS) is defined as a combination of GERD and neurologic symptoms (spastic torsional dystonia and opisthotonic body posturing with change in mental status). In healthy children SS is characterized by normal diagnostic tests. In children with cerebral palsy the existence of various MPs and abnormal EEG make the diagnosis of SS much more difficult. Aim: To show the importance of 24-hour video EEG polygraph monitoring (DVEEG) in differentiating motor paroxysms in a two-year old child with cerebral palsy (tetraparesis, mental retardation, epilepsy, feeding through a nasogastric tube). Methods: Case history: full-term infant (birth weight 3,5 kg), arrest in labor, hypoxic-ischemic brain damage, neonatal convulsions, West syndrome, brief period without seizures, then infrequent partial attacks. At 15 months frequent respiratory difficulties begin and at 17 months there is a sudden decline and diagnosis of GERD. He was treated with various antiepileptics, risperidone, baclofen, esomeprazole, and domperidone. Diagnostic work up: neurologic, pulmonary, gastroenterologic with DVEEG. Results: Various MPs were registered during DVEEG with or without ictal abnormalities on the EEG. During wakefulness, interictal EEG was dominated by beta and interpolated theta activity along with focal spikes from several independent sites. Interictal EEG during sleep maintained graphoelements of physiologic sleep, but with distorted sleep microarchitecture and paroxysmal multifocal activity. DVEEG showed poor spontaneous movement with only spastic MPs, asymmetric tonic neck reflexes, and total spastic and opisthotonic MP patterns during periods of irritability. These MPs were not associated with clear ictal EEG changes (there were frequent artifacts in cerebral and extracerebral parameters). During wakefulness and associated with feeding, there were about 20 attacks of spastic torsional dystonia/opisthotonia and tonic body posturing for half a minute without clear ictal EEG changes, which is characteristic for SS. There were also various MPs recorded with ictal EEG changes characteristic for epileptic attacks. Conclusion: DVEEG is the method of choice for differentiating motor paroxysms, epileptic seizures and Sandifer syndrome in cerebral palsy.

Keywords: EPILEPSY – drug therapy, diagnosis; CEREBRAL PALSY
Category: Original scientific paper
Volume: Vol. 53, No 3, july - september 2009
Authors: Lj. Popović Miočinović, N. Barišić, G. Paškov Dugandžija, M. Čikara, K. Bošnjak Nađ
Reference work:
DOI:

Read more