Table 2. Clinical and Electrophysiological Features of Relatives of the Dutch Familial Cortical Myoclonic Tremor with Epilepsy Pedigree, in Whom Additional Tests Were Performed^{16–18, 22, 23, 53}.

FCMTE	Age	Gender	Symptoms (age at onset)			AEDs	Electrophysiology			Additional Tests Performed
			Tremor	Myocl Seiz	GTCS		EEG	g-SEP	LLR	PA	Polymyography	Coherence	fMRI	TMS	Eye move
II:3	811	F	40	44?	44	VPA, PHT	±	n.d.	n.d.	+	n.d.	n.d.	n.d.	n.d.	n.d.
II:11	681	F	+	?	+	PB, CBZ	s-w	n.d.	n.d.	+	n.d.	n.d.	n.d.	n.d.	n.d.
III:1	60	M	45	–	52	VPA, CBZ	s-w	+	–	n.d.	+	+	+	+	+
III:5	57	M	30	30	43	OCB, CZP	s-w	–	?	n.d.	+	+	+	+	+
III:102	501	F	38	–	42	VPA, CZP	±	+	+	+	+	+	+	+	+
III:19	51	M	19	20	20	PB, VPA, CBZ	s-w	n.d.	n.d.	n.d.	+	+	+	n.d.	n.d.
III:20	54	M	12	13?	31	VPA, CZP	?	n.d.	n.d.	n.d.	+	+	+	n.d.	n.d.
IV:1	41	M	22	–	–	–	normal	+	+	n.d.	+	+	+	+	+
IV:2	39	F	20	–	–	–	normal	+	+	n.d.	+	+	+	+	+
IV:8	27	F	12	–	–	–	normal	+	+	n.d.	+	+	+	+	+
IV:9	21	F	+	–	–	–	n.d.	+	+	n.d.	n.d.	n.d.	n.d.	n.d.	+

Abbreviations: AEDs, antiepileptic drugs; CBZ, clobazam; Coherence, corticomuscular and intermuscular coherence analysis; CZP, clonazepam; EEG, electroencephalogram; Eye move, eye movement recordings; F, female; FCMTE, familial cortical myoclonic tremor with epilepsy; fMRI, functional magnetic resonance imaging; g-SEP, giant sensory evoked potential; GTCS, generalized tonic–clonic seizure; LLR, long latency reflex; M, male; Myocl Seiz, myoclonic seizure; n.d., not done; OCB, oxcarbamazepine; PA, neuropathology post-mortem; PB, phenobarbital; PHT, phenytoin; s-w, spike-wave complexes; TMS, transcranial magnetic stimulation; VPA, valproic acid.

+, present or performed; –, not observed or none; ±, some, not specific; ?, not known.

¹Age of death.

²Current case.