eMedicine Specialties > Neurology > Seizures and Epilepsy

Epilepsy, Juvenile Myoclonic: Differential Diagnoses & Workup

Author: Jose E Cavazos, MD, PhD, FAAN, Associate Professor with Tenure, Departments of Neurology, Pharmacology, and Physiology, University of Texas Health Science Center at San Antonio; Co-Director, South Texas Comprehensive Epilepsy Center; Director of the Epilepsy Center, Audie L Murphy Veterans Affairs Medical Center
Coauthor(s): Mark Spitz, MD, Professor, Department of Neurology, University of Colorado Health Sciences Center
Contributor Information and Disclosures

Updated: Jan 7, 2010

Differential Diagnoses

Absence Seizures
Benign Childhood Epilepsy
Frontal Lobe Epilepsy
Tonic-Clonic Seizures

Other Problems to Be Considered

Epilepsy with generalized tonic-clonic seizures on awakening
Nocturnal generalized tonic-clonic seizures
Myoclonic absence epilepsy
Myoclonus
Partial seizures with secondary generalization
Progressive myoclonic epilepsies

Workup

Imaging Studies

  • Neuroimaging studies are typically normal in juvenile myoclonic epilepsy (JME). Many clinicians believe that, in the presence of an adequate supportive history, EEG abnormalities, normal intelligence, and normal neurologic findings, neuroimaging studies are unnecessary. However, the clinical scenario might not be as clear as the classical description.
  • MRIs of the brain are usually unremarkable. This observation reflects the fact that JME is an idiopathic generalized epilepsy and not caused by conditions such as brain tumors or encephalitis. However, quantitative morphometric studies using a voxel-based technique have shown some differences among patients with JME. For example, decreased gray matter volume was found in thalami, insula cortices, and cerebellar hemispheres bilaterally in patients with JME. An increase in gray matter volume was observed in the right superior frontal, orbitofrontal, and medial frontal gyri of patients with JME as compared with age-matched controls. Patients with JME who are photosensitive have decreased gray matter volume in the visual cortex as compared with the control group; this was not found in patients with JME who were not photosensitive.27
  • Some patients with brain MRIs, particularly if the MRIs are high definition, or high Tesla studies, have shown minor abnormalities of cortical development. Tae et al reported reduction in the cortical thicknesses of frontal and temporal gyri in patients with JME.28 However, these observations were not confirmed in the study by Roebling et al.29 Progressive thalamic atrophy was also reported in patients with JME by the same group.30 The decreased thalamic volume has been confirmed by several other groups and might be related to executive function impairment.31,32 Furthermore, studies using diffusion tension imaging (DTI) have also confirmed that abnormalities in the degree of thalamocortical fiber orientation and tissue anisotropy correlate with the frequency of generalized tonic clonic seizures.33
  • Despite these minor quantitative differences, the guidelines of the International League Against Epilepsy (ILAE) do not recommend routine neuroimaging studies in patients with JME.34
  • Other neuroimaging studies
    • Magnetic resonance spectroscopy (MRS) has also confirmed abnormalities in the thalamus and thalamocortical system of patients with JME.35,36
    • Studies using transcranial magnetic stimulation show abnormalities in cortical excitability in patients with JME.37
    • Functional MRI (fMRI) studies have not shown significant abnormalities in patients with JME.29

Other Tests

  • Study of choice to confirm the clinical diagnosis is sleep-deprived EEG with activation procedures (ie, hyperventilation, photic stimulation). A normal study does not rule out epilepsy or JME. Typical EEG abnormalities are highly supportive of the clinical diagnosis (see Media file 1).

  • Findings in a man with a history of generalized t...

    Findings in a man with a history of generalized tonic-clonic seizures (mostly nocturnal) and myoclonic jerks (mostly in the morning) since the age of 14 years. Carbamazepine exacerbated his myoclonic seizures. Sleep-deprived EEG was digitally recorded and then plotted by using an analog paper machine. The patient was getting drowsy when this burst of polyspike and slow wave was recorded.

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    Findings in a man with a history of generalized t...

    Findings in a man with a history of generalized tonic-clonic seizures (mostly nocturnal) and myoclonic jerks (mostly in the morning) since the age of 14 years. Carbamazepine exacerbated his myoclonic seizures. Sleep-deprived EEG was digitally recorded and then plotted by using an analog paper machine. The patient was getting drowsy when this burst of polyspike and slow wave was recorded.

  • The typical interictal EEG abnormality consists of a generalized 4- to 6-Hz spike or polyspike and slow-wave discharges lasting 1-20 seconds (see Media file 1). Usually, 1-3 spikes precede each slow wave. When absence seizures are also present, 3-Hz spike-and-wave activity may be seen in addition to the polyspike-and-wave pattern.
  • The ictal EEG associated with myoclonic jerks typically reveals 10- to 16-Hz polyspike discharges. These may be preceded by spike and wave activity and are often followed by 1- to 3-Hz slow waves. The number of spikes is typically 5-20 and tends to be proportionately correlated with the clinical intensity of the seizure. These epileptic discharges may briefly persist, even after clinical activity has ceased. Seizures in patients with JME tend to be associated with polyspikes and disorganization of the paroxysm.38
  • Absence seizures of JME may be associated with ictal EEG patterns consisting of 3-Hz spike-and-wave activity. Sometimes, these are preceded by 4- to 6-Hz polyspike-and-wave discharges, which slow to 3 Hz as the patient loses consciousness.
  • Background activity of the EEG is normal in JME. Hyperventilation and photic stimulation often facilitate the appearance of epileptiform discharges. Photic stimulation frequently precipitates spike-and-wave patterns. This occurs in approximately 30% of patients with JME, compared with 18% of patients with childhood absence epilepsy, 13% of patients with epileptic seizures on awakening, and 7.5% of patients with juvenile absence epilepsy. Photosensitivity of the EEG in patients with JME has been reported to be as high as 50% of the cases.39 The EEG background has also been studied quantitatively in small groups of patients with JME.40
  • Treatment with medications clinically effective in JME might also reduce the frequency of interictal abnormalities. Levetiracetam adjunctive therapy in patients with JME increased the likelihood of a normal EEG from 8% to 53% after achieving maintenance therapy. There was a decrease in frequency of interictal discharges and suppression of the paroxysms induced by photic stimulation.41
  • Ictal and interictal polyspike-and-wave discharges are not pathognomonic of JME; they may be seen in other primary generalized epilepsies as well as in myoclonic epilepsies of early childhood.
  • In addition to generalized epileptiform discharges, focal abnormalities may be found in 20-55% of patients with JME. These include focal slow waves, generalized discharges that evolve from a focal onset, and focal spikes or spike-and-wave discharges. Ignorance of these changes may lead to one's mistakenly ruling out the syndrome. The etiology of these focal abnormalities is unclear. A possible explanation is structural changes in the cerebral cortex secondary to recurrent seizures or head injury; another is fluctuation in the refractoriness of the cortex to the influence of a spike/wave generator.
  • A morning EEG has been proposed as a superior strategy to detect generalized epileptiform discharges in patients with JME. In this particular study, a morning awake EEG detected interictal epileptiform discharges in 69% of patients while an afternoon awake EEG in the same patients demonstrated epileptiform discharges in fewer than 20% of patients.42
  • Video EEG monitoring in patients with atypical features of JME might be needed. In a one study, most people with JME only required no more than 2 days of stay to demonstrate diagnostic abnormalities in the EEG.43
  • A combined magnetoencephalography and EEG study demonstrated interictal spikes with localizations mainly in the central and premotor regions (Stefan et al., 2009) in patients with JME as compared with other absence syndromes.44

More on Epilepsy, Juvenile Myoclonic

Overview: Epilepsy, Juvenile Myoclonic
Differential Diagnoses & Workup: Epilepsy, Juvenile Myoclonic
Treatment & Medication: Epilepsy, Juvenile Myoclonic
Follow-up: Epilepsy, Juvenile Myoclonic
Multimedia: Epilepsy, Juvenile Myoclonic
References
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References

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Further Reading

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Keywords

JME, idiopathic generalized epileptic syndrome, myoclonic jerks, generalized tonic-clonic seizures, GTCSs, absence seizures

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Contributor Information and Disclosures

Author

Jose E Cavazos, MD, PhD, FAAN, Associate Professor with Tenure, Departments of Neurology, Pharmacology, and Physiology, University of Texas Health Science Center at San Antonio; Co-Director, South Texas Comprehensive Epilepsy Center; Director of the Epilepsy Center, Audie L Murphy Veterans Affairs Medical Center
Jose E Cavazos, MD, PhD, FAAN is a member of the following medical societies: American Academy of Neurology, American Clinical Neurophysiology Society, American Epilepsy Society, American Neurological Association, and Society for Neuroscience
Disclosure: Nothing to disclose.

Coauthor(s)

Mark Spitz, MD, Professor, Department of Neurology, University of Colorado Health Sciences Center
Mark Spitz, MD is a member of the following medical societies: American Academy of Neurology, American Clinical Neurophysiology Society, and American Epilepsy Society
Disclosure: pfizer Honoraria Speaking and teaching; ortho-mcneil Honoraria Review panel membership

Medical Editor

Ramon Diaz-Arrastia, MD, PhD, Assistant Professor, Department of Neurology, Comprehensive Epilepsy Center, University of Texas Southwestern
Ramon Diaz-Arrastia, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, New York Academy of Sciences, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

 
 
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