Acquired Epileptic Aphasia Differential Diagnoses

Updated: Oct 19, 2017
  • Author: Eli S Neiman, DO, FACN; Chief Editor: Amy Kao, MD  more...
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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

Table. (Open Table in a new window)

Diagnosis

Deterioration

EEG Patterns

Autistic epileptiform regression

Expressive language, RL, S, verbal and nonverbal communication

Centrotemporal spikes

Autistic regression

Expressive language, RL, S, verbal and nonverbal communication

Normal

Acquired epileptic aphasia

RL, possibly behavioral

Left or right temporal or parietal spikes, possibly ESES

Acquired expressive epileptic aphasia

Expressive language, oromotor apraxia

Centrotemporal spikes

ESES

Expressive language, RL, possibly behavioral

ESES

Developmental dysphasia (developmental expressive language disease)

No; lack of expressive language acquisition

Temporal or parietal spikes

Disintegrative epileptiform disorder

Expressive language, RL, S, verbal and nonverbal communication, possibly behavioral

ESES

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