Table 1.

Main neurological features in Mowat-Wilson Syndrome.

Neurological Features			Possible Therapeutic Intervention
head and brain malformations	head size and skull	microcephaly [2,24,49,53]
		Craniosynostosis [56,57]	neurosurgery [56,57]
	corpus callosum	complete agenesis [58]
		partial agenesis [58]
		hypoplasia 37% [58]
	hippocampus	bilateral morphological abnormalities or positional anomalies [58]
	white matter abnormalities	reduction of thickness [58]
		ventriculomegaly [12,58]
	cortical development	polymicrogyria, pachygyria, periventricular heterotopia [56,58,59]
		focal cortical dysplasia [4,58]
	cerebellar	hypoplastic or macro cerebellum [58,72]
		absent or small cerebellar vermis [58]
	other	CNS tumor [58,62]
		large basal ganglia [58]
		MC-I [15]
epilepsy	seizures	febrile seizures [17,63,64]	AEDs (VPA; LEV; bi-therapy, tri-therapy [7,17,63]); VNS [65]; Steroids [63]
		focal seizures [17,63]
		atypical absences [17,63]
	EEG	focal abnormalities [17,63,64]
		slowing of background activity [17,63,64]
		ESES [63,64]
sleep disorders	SDSC questionnaire	pathological results in “sleep wake transition” and “initiating and maintaining sleep” sub-scales [70]	melatonin; niaprazine, benzodiazepines [64,70]
	sleep architecture (polysomnography)	TST reduction and WASO increase [64]