Melatonin and Angelman Syndrome: Implications and Mathematical Model of Diurnal Secretion

Table 1

Children with Angelman syndrome. The characteristics.

Age at diagnosis; (sex)	Patient 1; 10 yrs; (f)	Patient 2; 4 yrs; (f)	Patient 3; 3 yrs; (f)	Patient 4; 5 yrs; (m)	Patient 5; 5 yrs; (f)	Patient 6; 4 yrs; (m)	Patient 7; 3 yrs; (m)	Patient 8; 7 yrs; (m)	Patient 9; 9 yrs; (m)
Seizure type	TS, AA	IS, MS, AT, AA	MS, AA	GTCS, AA, AT	AA, GTCS	MS, AA, GTCS	MS, AA	MS, AA, GTCS	MS, AA, GTCS
EEG	Abnormal sleep pattern, generalized abnormalities
	With the left side predominance	Hipsarrhythmia	With the frontal and temporal predominance	With the temporal and occipital predominance	With the temporal and occipital predominance	With the frontal and temporal predominance	With the right side predominance	With the left side predominance	With the frontal and temporal predominance
AEDs	VPA, LTG	VPA, LTG	VPA, LTG	VPA, LTG	VPA, LTG	VPA, LTG	VPA, LTG	VPA, LTG	VPA, LTG
Neurological examination	Microcephaly, gait ataxia	Microcephaly, spastic paraparesis, ataxia	Microcephaly, ataxia	Microcephaly, spastic quadriparesis	Microcephaly, spastic paraparesis, ataxia	Microcephaly, spastic paraparesis	Microcephaly, ataxia	Microcephaly, ataxia, spastic quadriparesis	Microcephaly, spastic quadriparesis
DQ/IQ (according to revised Brunet-Lezine or Wechsler scale)	72	27	49	17	32	65	52	20	24
Brain MRI	Normal	Delayed myelination	Normal	Delayed myelination, widening of the perivascular spaces of Virchow-Robin	Normal	Delayed myelination	Normal	Delayed myelination	Frontal and temporal cortical atrophy, narrow corpus callosum, and widening and asymmetry of the ventricular system
Minimum melatonin concentration (pg/mL)	0.1787	8.2274	4.8639	10.0281	2.53770	9.2324	11.2680	7.8354	0.3444
Melatonin release amplitude (pg/mL)	466.7658	167.4662	208.3878	153.9621	79.88783	222.4951	162.7621	100.3606	201.1275
Phase shift of melatonin release (h)	5.6183	12.1787	6.3585	9.1097	2.30309	2.1005	3.5904	1.5535	2.1150
Estimated sleep duration (h)	8.402	11.2672	8.0544	6.8388	12.83515	7.2964	7.7087	7.3945	11.4236
Maximum melatonin concentration (pg/mL)	466.9445	175.6936	213.2517	163.9902	82.4255	231.7275	174.0301	108.1960	201.4719
DLMOon50 (h)	1.4173	6.5451	2.3313	5.6903	19.8855	22.4523	23.7360	21.8562	20.4033
DLMOoff50 (h)	9.8193	17.8123	10.3857	12.5291	8.7207	5.7487	7.4447	5.2507	7.8268
DLMOon25 (h)	0.2667	5.0021	1.2283	4.7537	18.1278	21.4531	22.6803	20.8436	18.8389
DLMOoff25 (h)	10.9699	19.3553	11.4887	13.4656	10.4783	6.7479	8.5004	6.2633	9.3912
Max gamma	3.2600	5.0490	2.0780	0.3255	3.6410	0.9718	2.6570	0.5221	3.2590

TS: tonic spasms; MS: myoclonic seizures; GTCS: generalized tonic-clonic seizures; AA: atypical absences; AT: astatic seizures; IS: infantile seizures; VPA: valproic acid; WGB: vigabatrin; LTG: lamotrygine; TPM: topiramate. Time in decimal scale.