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PubMed This is a summary of 14 peer-reviewed journal articles Updated
Neurology

The Triad of FAME: Recognizing Your Symptoms

At a Glance

Familial Adult Myoclonic Epilepsy (FAME) is characterized by a triad of symptoms: cortical tremor (fine hand shaking), myoclonus (sudden muscle jerks), and occasional convulsive seizures. Symptoms often begin in early adulthood and can flare up due to stress, lack of sleep, or flickering lights.

Understanding the symptoms of Familial Adult Myoclonic Epilepsy (FAME) often brings a sense of relief to patients who have spent years searching for an explanation for their “shaky hands.” Doctors identify FAME through a specific “triad” or group of three symptoms that typically appear in a predictable order over a person’s life [1][2][3].

The Symptom Triad

Most patients with FAME experience these three features at different stages:

  1. Cortical Tremor: Usually the first sign, appearing in the late teens or early 20s [2][4]. It is a fine, rhythmic trembling of the hands [3].
  2. Myoclonus: These are brief, involuntary “shocks” or jerks of the muscles [3]. While the tremor itself is a form of tiny myoclonus, patients may also experience larger, sudden jerks in the limbs or torso [3][5].
  3. Occasional Seizures: Convulsive seizures (often called generalized tonic-clonic seizures) usually develop later than the tremor, often in a person’s 30s or 40s [6][4]. These seizures are typically rare and may be separated by many years [1][3]. It’s important to note that some patients with FAME may only ever experience the tremor and never develop convulsive seizures [2].

Understanding “Cortical Tremor”

The most common symptom of FAME is the cortical tremor, but it is frequently misdiagnosed because it looks like other common tremors [7]. However, it has unique characteristics that set it apart:

Feature FAME (Cortical Tremor) Essential Tremor Parkinson’s Tremor
Origin The brain’s cortex (the outer layer) [8]. The cerebellum or brainstem. The basal ganglia.
What it feels like A fine “tremulousness” that is actually a series of tiny, rapid muscle jerks (myoclonus) [3][2]. A rhythmic back-and-forth shaking. A slow “pill-rolling” motion.
When it happens When holding a posture (like holding out your hands) or moving [2]. Mostly during movement (like reaching for a cup). Mostly at rest (when hands are in your lap).
Associated Signs May include light sensitivity or “giant” responses on specialized brain tests (SEPs) [9][10]. No seizure or myoclonus association. Often includes stiffness and slow movement.

Common Triggers

FAME symptoms are not always constant; they can “flare up” or become more noticeable due to external factors. Because the brain in FAME is hyperexcitable (meaning its nerves are more easily “startled”), certain triggers can lower the threshold for tremors or seizures [9][11]:

  • Photosensitivity: Many patients are sensitive to flickering lights or specific visual patterns, which can trigger tremors or muscle jerks [10][12].
  • Sleep Deprivation: Lack of sleep is a well-known trigger for many types of epilepsy, including FAME.
  • Stress and Fatigue: Emotional stress or physical exhaustion can make the rhythmic hand shaking more prominent.
  • Caffeine and Alcohol: While not unique to FAME, excessive caffeine can exacerbate any tremor, and alcohol withdrawal can lower the seizure threshold [11].
  • Action and Posture: The tremor is often most visible when you are trying to use your hands for precise tasks, such as writing or holding a utensil [2].

A Life-Long Perspective

For most people, FAME follows a slow, non-progressive course [13]. While the hand tremors may become slightly more noticeable as you age, the condition does not typically lead to the severe neurological decline seen in other movement disorders [13][3]. Some families notice genetic anticipation, where children may start experiencing symptoms slightly earlier than their parents did [1][14]. Knowing this allows for early identification and better management of the condition.

Common questions in this guide

What are the three main symptoms of FAME?
The classic symptom triad of FAME includes cortical tremor (fine hand trembling), myoclonus (sudden muscle jerks or shocks), and occasional convulsive seizures. The tremor usually appears first in early adulthood, while seizures typically develop later in a person's 30s or 40s.
How do I know if my hand shaking is a cortical tremor or an essential tremor?
A cortical tremor feels like a fine trembling but is actually a series of rapid, tiny muscle jerks originating in the brain's cortex. Unlike essential tremor, which originates in a different part of the brain, cortical tremor is often associated with larger muscle jerks or seizures.
What can trigger my FAME symptoms to flare up?
FAME symptoms can be worsened by sleep deprivation, emotional stress, physical fatigue, and excessive caffeine. Many people are also sensitive to flickering lights or specific visual patterns, which can trigger tremors or muscle jerks.
Will my FAME symptoms get severely worse over time?
For most people, FAME follows a slow, non-progressive course. While hand tremors may become slightly more noticeable with age, the condition typically does not lead to the severe neurological decline seen in other movement disorders.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Based on my symptoms, how do we confirm that this is a 'cortical tremor' rather than a more common essential tremor?
  2. 2.Can we perform an electrophysiological test, like a somatosensory evoked potential (SEP), to look for 'giant' responses in my brain?
  3. 3.Does my pattern of symptoms suggest a specific genetic subtype of FAME?
  4. 4.How can we best manage my specific triggers, especially if I have photosensitivity?
  5. 5.Since my tremors might change as I get older, what signs of progression should I be watching for?

Questions For You

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References

References (14)
  1. 1

    TTTCA repeat expansion causes familial cortical myoclonic tremor with epilepsy.

    Lei XX, Liu Q, Lu Q, et al.

    European journal of neurology 2019; (26(3)):513-518 doi:10.1111/ene.13848.

    PMID: 30351492
  2. 2

    Familial adult myoclonic epilepsy: A new expansion repeats disorder.

    Lagorio I, Zara F, Striano S, Striano P

    Seizure 2019; (67()):73-77 doi:10.1016/j.seizure.2019.03.009.

    PMID: 30928698
  3. 3

    Familial Adult Myoclonic Epilepsy: Clinical and Genetic Approach to an Under-recognized Disease.

    Uzun GA, Baykan B

    Noro psikiyatri arsivi 2023; (60(2)):174-177 doi:10.29399/npa.28252.

    PMID: 37287551
  4. 4

    Familial Adult Myoclonus Epilepsy: A Non-Coding Repeat Expansion Disorder of Cerebellar-Thalamic-Cortical Loop.

    Cuccurullo C, Striano P, Coppola A

    Cells 2023; (12(12)) doi:10.3390/cells12121617.

    PMID: 37371086
  5. 5

    Clinical and neurophysiological features of familial cortical myoclonic tremor with epilepsy.

    Cen Z, Huang C, Yin H, et al.

    Movement disorders : official journal of the Movement Disorder Society 2016; (31(11)):1704-1710 doi:10.1002/mds.26756.

    PMID: 27613677
  6. 6

    Familial cortical myoclonic tremor with epilepsy: TTTCA/TTTTA repeat expansions and expanding phenotype in two Chinese families.

    Zhang Y, Xiong W, Lu L, Zhou D

    Brain research 2020; (1737()):146796 doi:10.1016/j.brainres.2020.146796.

    PMID: 32194077
  7. 7

    Autosomal dominant cortical tremor, myoclonus and epilepsy.

    Striano P, Zara F

    Epileptic disorders : international epilepsy journal with videotape 2016; (18(S2)):139-144 doi:10.1684/epd.2016.0860.

    PMID: 27618961
  8. 8

    Familial adult myoclonus epilepsy: Neuroimaging and neuropathological findings.

    van Rootselaar AF, Cocozza S, Aronica E, Striano P

    Epilepsia 2023; (64 Suppl 1()):S47-S51 doi:10.1111/epi.17628.

    PMID: 37096373
  9. 9

    A Biomarker for Benign Adult Familial Myoclonus Epilepsy: High-Frequency Activities in Giant Somatosensory Evoked Potentials.

    Tojima M, Hitomi T, Matsuhashi M, et al.

    Movement disorders : official journal of the Movement Disorder Society 2021; (36(10)):2335-2345 doi:10.1002/mds.28666.

    PMID: 34050549
  10. 10

    Aberrant visual-related networks in familial cortical myoclonic tremor with epilepsy.

    Wang H, Wang B, Cen Z, et al.

    Parkinsonism & related disorders 2022; (101()):105-110 doi:10.1016/j.parkreldis.2022.07.001.

    PMID: 35870251
  11. 11

    Amplitude of Somatosensory Evoked Potentials (SEPs) Recorded in Short-Latency SEP Condition Is 80% of That in Giant SEP Condition.

    Demura A, Demura Y, Sato K, Kinoshita M

    Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society 2024; (41(3)):285-290 doi:10.1097/WNP.0000000000000966.

    PMID: 36173293
  12. 12

    Characteristics of visual sensitivity in familial cortical myoclonic tremor and epilepsy.

    Wang X, Wang B, Wang J, et al.

    Epileptic disorders : international epilepsy journal with videotape 2021; (23(2)):366-375 doi:10.1684/epd.2021.1278.

    PMID: 33935025
  13. 13

    Familial cortical myoclonic tremor and epilepsy: Description of a new South African pedigree with 30 year follow up.

    van Coller R, van Rootselaar AF, Schutte C, van der Meyden CH

    Parkinsonism & related disorders 2017; (38()):35-40 doi:10.1016/j.parkreldis.2017.02.016.

    PMID: 28237853
  14. 14

    (TTTCA)exp Drives the Genotype-Phenotype Correlation and Genetic Anticipation in FCMTE1.

    Chen X, Wang B, Xia H, et al.

    Movement disorders : official journal of the Movement Disorder Society 2025; (40(2)):315-323 doi:10.1002/mds.30057.

    PMID: 39569876

This page explains the symptoms of Familial Adult Myoclonic Epilepsy (FAME) for educational purposes. Always consult a neurologist for a proper diagnosis and to evaluate your specific tremors or seizures.

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