Morvan syndrome and related disorders associated with CASPR2 antibodies
Jan. 18, 2022
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In this clinical article, the author describes the basics of aphasic seizures. As an inhibitory form of epileptic seizure, thus, not causing positive symptoms but disrupting ongoing cognitive tasks involving language, aphasic seizures may present a challenging diagnosis. Transient ischemic attacks or stroke could be first evoked by clinicians not aware of this possibility. Careful examination, medical history, and EEG should help the neurologist in making the correct diagnosis and introducing adequate therapeutics.
• Aphasic seizure is a specific type of inhibitory seizure.
• Aphasic status epilepticus should be considered in patients with transient and sustained aphasia not better explained by acute acquired brain lesion (stroke).
• EEG and brain imaging (including functional brain imaging) could help differentiate an aphasic seizure from aphasia due to other causes.
Although the functional anatomy of speech processing has been investigated intensively for almost 150 years, the neural organization of speech perception remains highly debated, even in gross anatomical terms, as pointed out in a reference article (21). “Aphasia” could be defined as “language impairment caused by a permanent or transient injury to the brain, in a previously normal language user.” The deficit may range from very limited problems in exact word finding to more pervasive deficits impacting on all language modalities. Speech production, verbal comprehension, word finding, and repetition can be affected to varying degrees, with or without reading and writing abnormalities.
In the vast majority of left-handed people and almost all right-handers, left hemispheric dysfunction is responsible for language disorders. However, right hemisphere lesions or transient impairment could produce some language problems such as dysprosody. After Paul Broca’s description of the loss of articulate speech following ischemic damage located in the posterior inferior frontal gyrus of the left hemisphere, several other aphasia syndromes have been described, including anomic aphasia, global aphasia, transcortical motor aphasia, Wernicke aphasia, conduction aphasia, and transcortical sensory aphasia. Each aphasic syndrome is presumed to be associated with specific brain area damage, although this remains controversial, even with respect to very ancient observations, as in Broca aphasia (18).
As already mentioned on what should be considered as a “true” aphasic seizure, there are numerous potential causes for a patient’s inability to speak, and thus, “aphasia” is not synonymous with “not speaking.” A comatose patient or a patient refusing to communicate with doctors or caregivers is not aphasic. Evidence of inappropriate use of language should be collected by examiners to document the diagnosis of aphasia: inability to correctly name objects or point to objects named by others, incorrect comprehension and/or repetition of sentences, words, and non-words, disturbed syntax or grammar, paraphasic errors, etc.
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