Issue # 39

Lesser Known Disorders

Each issue of this series contains at least three lesser known disorders. Some of these disorders may contain subtypes which will also be presented. You will also notice that each disorder has a code. These codes represent the coding system for all disabilities and disorders listed in the Educator’s Diagnostic Manual(EDM) Wiley Publications.

SL 6.05-Landau-Kleffner Syndrome

(also known as Infantile Acquired Aphasia, Acquired Epileptic Aphasia, or Aphasia with Compulsive Disorder)

Disability Category: Speech and Language

Definition

Landau-Kleffner syndrome (also called infantile acquired aphasia, acquired epileptic aphasia, or aphasia with convulsive disorder) is a language disorder. It frequently occurs in normally-developing children, usually between three and seven years of age, and is characterized by the gradual or sudden loss of the ability use or comprehend spoken language (New York Presbyterian Hospital, 2003).

 

Explanation

LKS occurs most frequently in normally developing children who are between 3 and 7 years of age. For no apparent reason, these children begin having trouble understanding what is said to them. Doctors often refer to this problem as auditory agnosia or “word deafness.” The auditory agnosia may occur slowly or very quickly. Parents often think that the child is developing a hearing problem or has become suddenly deaf. Hearing tests, however, show normal hearing. Children may also appear to be autistic or developmentally delayed (National Institute of Neurological Disorders and Stroke, 2005; National Institute on Deafness and Other Communication Disorders National Institutes of Health, 2002d; New York Presbyterian Hospital, 2003).

 

The inability to understand language eventually affects the child’s spoken language which may progress to a complete loss of the ability to speak (mutism). Children who have learned to read and write before the onset of auditory agnosia can often continue communicating through written language. Some children develop a type of gestural communication or sign-like language.

 

The loss of language may be preceded by an epileptic seizure that usually occurs at night. At some time, 80 percent of children with LKS have one or more seizures. The seizures usually stop by the time the child becomes a teenager. All LKS children have abnormal electrical brain activity on both the right and left sides of their brains (National Institute of Neurological Disorders and Stroke, 2005; National Institute on Deafness and Other Communication Disorders National Institutes of Health, 2002d).

 

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    SL 6.06-Transcortical Aphasia

    Disability Category: Speech and Language

    Definition

    A type of aphasia specifically associated with either partial or total loss of the ability to communicate verbally or using written words that does not affect an individual’s ability to repeat words, phrases, and sentences (National Institute on Deafness and Other Communication Disorders, 2002a). Transcortical aphasia is caused by damage to the language areas of the left hemisphere that are outside the primary language areas (Penn State-Milton Hershey Medical Center College of Medicine, 2005).

     

    Explanation

    People with Transcortical Aphasia suffer partial or total loss of the ability to communicate verbally or use written words, but can still repeat words, phrases, or sentences (Penn State-Milton Hershey Medical Center College of Medicine, 2005). Other language functions may also be impaired to varying degrees, depending on the extent and particular location of brain damage.

     

    There are three types of Transcortical Aphasia:

    1. Transcortical Motor Aphasia
    2. Transcortical Sensory Aphasia
    3. Mixed Transcortical Aphasia.

     

    SL 6.06a-Transcortical Motor Aphasia

    Transcortical motor aphasia is different than Broca’s aphasia in that repetition ability is intact. Patients who demonstrate transcortical motor aphasia have the same halting, agrammatic speech of Broca’s, but may be echolalic, or able to repeat complex words and phrases (DaVanzo, 2005).

     

    SL 6.06b-Transcortical Sensory Aphasia

    This occurs when the speech areas are disconnected from the posterior association cortex. Patients can recognize words and they can talk, but they cannot understand what people are saying to them and have no spontaneous speech of their own (DaVanzo, 2005)

     

    SL 6.06c-Mixed Transcortical Mixed Aphasia

    A rare aphasic disorder involves the isolation of both Broca’s and Wernicke’s areas. The patient has a virtual compulsion to repeat utterances to the point of appearing echolalic. Other language abilities, such as comprehension, naming, expression, and reading are impaired. The individual may not utter any language unless spoken to (DaVanzo, 2005)

     

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      SL 6.07-Wernicke’s Aphasia

      Disability Category: Speech and Language

      Definition

      A type of aphasia specifically associated with damage to the temporal lobe of the brain (typically the left hemisphere) thought to be important for the comprehension of spoken language (Hedge & Maul, 2006).

       

      Explanation

      Individuals with Wernicke’s Aphasia may speak in long sentences that have no meaning, add unnecessary words, and even create new “words” (Anderson & Shames, 2006). For example, someone with Wernicke’s Aphasia may say, “You know that smoodle pinkered and that I want to get him round and take care of him like you want before,” meaning, “The dog needs to go out so I will take him for a walk.” Individuals with Wernicke’s aphasia usually have great difficulty understanding speech and are therefore often unaware of their mistakes. These individuals usually have no body weakness because their brain injury is not near the parts of the brain that control movement (National Institute of Neurological Disorders and Stroke, 2005).

       

      People with Wernicke’s Aphasia often speak with words that aren’t needed, difficult to understand, or simply made up. They are often completely unaware of their language difficulties, and may not even understand what other people say at all. While they may still be able to write, what they write may not be understandable. People with Wernicke’s aphasia also have difficulty reading (Penn State-Milton Hershey Medical Center College of Medicine, 2005).

       

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