Acquired Epileptic Aphasia Differential Diagnoses

  • Author: Eli S Neiman, DO; Chief Editor: Amy Kao, MD   more...
 
Updated: Apr 27, 2011
 
 

Diagnostic Considerations

The differential diagnosis of acquired epileptic aphasia (AEA) is extensive, including the conditions shown in Table 3, below. Table 3. Differential Diagnosis of Syndromes of Language and/or Cognitive and Behavioral Regression

(Open Table in a new window)

DiagnosisDeteriorationEEG Patterns
Autistic epileptiform regressionExpressive language, RL, S, verbal and nonverbal communicationCentrotemporal spikes
Autistic regressionExpressive language, RL, S, verbal and nonverbal communicationNormal
Acquired epileptic aphasiaRL, possibly behavioralLeft or right temporal or parietal spikes, possibly ESES
Acquired expressive epileptic aphasiaExpressive language, oromotor apraxiaCentrotemporal spikes
ESESExpressive language, RL, possibly behavioralESES
Developmental dysphasia (developmental expressive language disease)No; lack of expressive language acquisitionTemporal or parietal spikes
Disintegrative epileptiform disorderExpressive language, RL, S, verbal and nonverbal communication, possibly behavioralESES
EEG = electroencephalographic; ESES = electrical status epilepticus of sleep; RL = receptive language; S = sociability.



* Continuous spike and wave of slow-wave sleep (>85% of slow-wave sleep).



Acquired aphasia in children may be secondary to head trauma, brain tumors, stroke, or neurocysticercosis. As with acquired epileptic aphasia, head injury, brain neoplasms, and cerebrovascular thromboembolism may be associated with an epileptiform electroencephalogram (EEG) and seizures. Other neurologic deficits, such as hemiparesis or signs of increased intracranial pressure, may be a clue of an underlying structural lesion. Imaging studies, especially magnetic resonance imaging (MRI), can help clarify the diagnosis. Patients with brain tumors may develop an acquired aphasia secondary to seizures and epileptic discharges generated around the neoplasm.

Deaf children may have many of the symptoms of acquired epileptic aphasia. Conversely, patients with acquired epileptic aphasia do not always have convulsions, and, even when present, the seizures may be missed. Some children with acquired epileptic aphasia may initially be thought to be deaf and are referred for audiologic evaluation.

Hyperactivity and decreased attention span are common in acquired epileptic aphasia. Patients in whom attention deficit disorder is suspected should be carefully examined for aphasia. The resemblance of acquired epileptic aphasia and attention deficit disorder is superficial, and differentiation of the 2 should not be difficult. Likewise, patients with aggressive (eg, rage attacks), oppositional, or psychotic behavior should be examined for signs of language impairment, because these symptoms can be present in patients with acquired epileptic aphasia.

Complex and bizarre behaviors, such as interpersonal contact avoidance, gestural stereotypies, and frankly autistic behavior can be seen in acquired epileptic aphasia. A careful history is necessary in patients with autism or pervasive developmental disorders (PDDs). When in doubt, a sleep EEG is required.

Patients who never develop language or have a poor acquisition of language must be differentiated from children with hearing deficits and mental retardation. Acquired epileptic aphasia and mental retardation (congenital without intellectual deterioration) should be differentiated on the basis of information from the history, neuropsychologic testing, and EEG. Patients with acquired epileptic aphasia typically have normal hearing threshold on formal testing.

On occasion, patients with neurodegenerative diseases may present with problems in language comprehension. This is especially true for patients with adrenoleukodystrophy in whom the initial complaint can be difficulty in processing auditory information due to dysmyelination of the white matter in the temporoparietal region. At this stage, patients do not necessarily have corticospinal tract signs; however, over time, spasticity, increased deep tendon reflexes, and upgoing toes become apparent. Seizures are infrequent (about 10%) in adrenoleukodystrophy. The age of onset of the childhood form of adrenoleukodystrophy is similar to that of acquired epileptic aphasia. T2-weighted MRIs of the brain show increased signal intensity in the affected areas.

Other problems to be considered include the following:

  • Acquired expressive epileptic aphasia
  • Adrenoleukodystrophy
  • Childhood disintegrative disorder
  • Developmental dysphasia or developmental expressive language disorder
  • Disintegrative epileptiform disorder
  • Electrical status epilepticus of sleep (ESES)

Differential Diagnoses

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Contributor Information and Disclosures
Author

Eli S Neiman, DO  Assistant Professor of Neurology, Seton Hall University School of Graduate Medical Education, Comprehensive Epilepsy Center, New Jersey Neuroscience Institute-JFK Medical Center, Edison, NJ

Eli S Neiman, DO is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, and American Osteopathic Association

Disclosure: UCB Pharma Honoraria Review panel membership; UCB Pharma Honoraria Speaking and teaching; Cyberonics, Inc Honoraria Speaking and teaching

Coauthor(s)

Michael Seyffert  MD, Assistant Professor or Neurosciences, Seton Hall University School of Graduate Medical Education; Project Advisor, Biomedical Engineering, New Jersey Neuroscience Institute, Neurology and Sleep Medicine, JFK Medical Center

Michael Seyffert is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, and Child Neurology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Robert Stanley Rust Jr, MD, MA  Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-Director of FE Dreifuss Child Neurology and Epilepsy Clinics, Director, Child Neurology, University of Virginia School of Medicine; Chair-Elect, Child Neurology Section, American Academy of Neurology

Robert Stanley Rust Jr, MD, MA is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Headache Society, American Neurological Association, Child Neurology Society, International Child Neurology Association, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Chief Editor

Amy Kao, MD  Attending Neurologist, Children's National Medical Center

Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author Marcio Sotero de Menezes, MD, to the development and writing of the source article.

References

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Table 1. Long-Term Follow-up of Acquired Epileptic Aphasia
StudyNumber of PatientsMean Follow-up, yNumber of Patients with Normal or Mild Language Problems
Soprano et al[10] (1994) 1283
Mantovani and Landau[15] (1980) 9226
Paquier[16] (1992) 68.13
Rossi[17] (1999) 119.72
Robinson et al[11] (2001) 185.63
Duran et al[13] (2009) 79.51
Total6318 (28.6%)
Table 2. Epileptiform EEG Findings in Autism, Dysphasia, and Epilepsy
SourceDiagnosisNumber of PatientsNumber of Patients with EEGsPatients with Abnormal EEGs (%)
Tuchman et al (1991)Autism with epilepsy424075
Autism without epilepsy1601398
Dysphasia with epilepsy191958
Dysphasia without epilepsy218669
Tuchman and Rapin[22] (1997) PDD or autism585392*NA
With epilepsyNA6659
Without epilepsyNA6659
Without epilepsy but with history of regressionNA15514
Without epilepsy and without history of regressionNA3646
EEG(s) = electroencephalogram(s); NA = not applicable; PDD = personality developmental disorder.



* Sleep EEGs.



Table 3
DiagnosisDeteriorationEEG Patterns
Autistic epileptiform regressionExpressive language, RL, S, verbal and nonverbal communicationCentrotemporal spikes
Autistic regressionExpressive language, RL, S, verbal and nonverbal communicationNormal
Acquired epileptic aphasiaRL, possibly behavioralLeft or right temporal or parietal spikes, possibly ESES
Acquired expressive epileptic aphasiaExpressive language, oromotor apraxiaCentrotemporal spikes
ESESExpressive language, RL, possibly behavioralESES
Developmental dysphasia (developmental expressive language disease)No; lack of expressive language acquisitionTemporal or parietal spikes
Disintegrative epileptiform disorderExpressive language, RL, S, verbal and nonverbal communication, possibly behavioralESES
EEG = electroencephalographic; ESES = electrical status epilepticus of sleep; RL = receptive language; S = sociability.



* Continuous spike and wave of slow-wave sleep (>85% of slow-wave sleep).



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