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Epilepsy, familial adult myoclonic, 2(FAME2)

MedGen UID:
375031
Concept ID:
C1842852
Disease or Syndrome
Synonyms: BENIGN ADULT FAMILIAL MYOCLONIC EPILEPSY 2; CORTICAL MYOCLONIC TREMOR WITH EPILEPSY, FAMILIAL, 2; CORTICAL MYOCLONUS AND EPILEPSY, AUTOSOMAL DOMINANT; FAME2
 
Gene (location): STARD7 (2q11.2)
 
Monarch Initiative: MONDO:0011930
OMIM®: 607876

Definition

Familial adult myoclonic epilepsy-2 (FAME2) is an autosomal dominant neurologic disorder characterized by onset of tremor affecting the fingers, hand, and voice in adolescence or young adulthood with somewhat later onset of rhythmic myoclonic jerks and generalized tonic-clonic seizures. Electrophysiologic studies are consistent with cortical reflex myoclonus. Some patients may show cognitive decline or migraines; photosensitivity is common (summary by De Fusco et al., 2014; Crompton et al., 2012). For a phenotypic description and a discussion of genetic heterogeneity of familial adult myoclonic epilepsy, see FAME1 (601068). [from OMIM]

Clinical features

From HPO
Cerebellar ataxia
MedGen UID:
849
Concept ID:
C0007758
Disease or Syndrome
Cerebellar ataxia refers to ataxia due to dysfunction of the cerebellum. This causes a variety of elementary neurological deficits including asynergy (lack of coordination between muscles, limbs and joints), dysmetria (lack of ability to judge distances that can lead to under- or overshoot in grasping movements), and dysdiadochokinesia (inability to perform rapid movements requiring antagonizing muscle groups to be switched on and off repeatedly).
Myoclonus
MedGen UID:
10234
Concept ID:
C0027066
Finding
Very brief, involuntary random muscular contractions occurring at rest, in response to sensory stimuli, or accompanying voluntary movements.
Tremor
MedGen UID:
21635
Concept ID:
C0040822
Sign or Symptom
An unintentional, oscillating to-and-fro muscle movement about a joint axis.
Migraine
MedGen UID:
57451
Concept ID:
C0149931
Disease or Syndrome
Migraine is a chronic neurological disorder characterized by episodic attacks of headache and associated symptoms.
Cognitive impairment
MedGen UID:
90932
Concept ID:
C0338656
Mental or Behavioral Dysfunction
Abnormal cognition is characterized by deficits in thinking, reasoning, or remembering.
Bilateral tonic-clonic seizure
MedGen UID:
141670
Concept ID:
C0494475
Sign or Symptom
A bilateral tonic-clonic seizure is a seizure defined by a tonic (bilateral increased tone, lasting seconds to minutes) and then a clonic (bilateral sustained rhythmic jerking) phase.
Dementia
MedGen UID:
99229
Concept ID:
C0497327
Mental or Behavioral Dysfunction
A loss of global cognitive ability of sufficient amount to interfere with normal social or occupational function. Dementia represents a loss of previously present cognitive abilities, generally in adults, and can affect memory, thinking, language, judgment, and behavior.
EEG with photoparoxysmal response
MedGen UID:
765735
Concept ID:
C3552821
Finding
EEG abnormalities (epileptiform discharges) evoked by flashing lights or black and white striped patterns.
Enhancement of the C-reflex
MedGen UID:
765738
Concept ID:
C3552824
Finding
Increase in amplitude of a long-loop response upon somatosensory evoked potential testing, representing an electrically evoked myoclonic response.
Jerk-locked premyoclonus spikes
MedGen UID:
765739
Concept ID:
C3552825
Finding
Jerk-locked averaging (JLA) is used to record the timing and distribution of brain activity preceding brisk involuntary movements such as those observed in patients with myoclonus. JLA is capable of revealing a premyoclonus spike in the absence of paroxysmal activity in the routine EEG.
Intellectual disability
MedGen UID:
811461
Concept ID:
C3714756
Mental or Behavioral Dysfunction
Intellectual disability, previously referred to as mental retardation, is characterized by subnormal intellectual functioning that occurs during the developmental period. It is defined by an IQ score below 70.
Giant somatosensory evoked potentials
MedGen UID:
813291
Concept ID:
C3806961
Finding
An abnormal enlargement (i.e. increase in measured voltage) of somatosensory evoked potentials.
EEG with irregular generalized spike and wave complexes
MedGen UID:
871304
Concept ID:
C4025792
Finding
EEG shows spikes (<80 ms) and waves, which are recorded over the entire scalp and do not have a specific frequency.
Blepharospasm
MedGen UID:
599
Concept ID:
C0005747
Disease or Syndrome
A focal dystonia that affects the muscles of the eyelids and brow, associated with involuntary recurrent spasm of both eyelids.

Recent clinical studies

Etiology

Franceschetti S, Visani E, Panzica F, Coppola A, Striano P, Canafoglia L
Clin Neurophysiol 2023 Jul;151:74-82. Epub 2023 May 10 doi: 10.1016/j.clinph.2023.04.009. PMID: 37216715

Diagnosis

De Fusco M, Vago R, Striano P, Di Bonaventura C, Zara F, Mei D, Kim MS, Muallem S, Chen Y, Wang Q, Guerrini R, Casari G
Ann Neurol 2014 Jan;75(1):77-87. Epub 2014 Jan 2 doi: 10.1002/ana.24028. PMID: 24114805Free PMC Article

Prognosis

Franceschetti S, Visani E, Panzica F, Coppola A, Striano P, Canafoglia L
Clin Neurophysiol 2023 Jul;151:74-82. Epub 2023 May 10 doi: 10.1016/j.clinph.2023.04.009. PMID: 37216715

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