Gerstmann syndrome

Gerstmann syndrome
Principal fissures and lobes of the cerebrum viewed laterally. (Parietal lobe is shown in yellow)
ICD-10	F81.2
ICD-9	784.69
DiseasesDB	30728

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Gerstmann syndrome is a rare neurological disorder that is characterized by a tetrad of symptoms which include finger agnosia, agraphia, acalculia, and left - right disorientation. Also known as Angular Gyrus Syndrome as it occurs due to lesion or pathology involving the angular gyrus of the inferior parietal lobule in the dominant hemisphere. (It should not be confused with Gerstmann-Straussler syndrome, which is a transmissible spongiform encephalopathy.)

Primary symptoms

Gerstmann syndrome is characterized by four primary symptoms:

1. Dysgraphia/agraphia
2. Dyscalculia/acalculia
3. Finger agnosia
4. Left-right disorientation

Causes

This disorder is often associated with brain lesions in the dominant (usually left) side of the angular and supramarginal gyri near the temporal and parietal lobe junction. There is significant debate in the scientific literature as to whether Gerstmann Syndrome truly represents a unified, theoretically motivated syndrome. Thus its diagnostic utility has been questioned by neurologists and neuropsychologists alike.

In adults

In adults, the syndrome may occur after a stroke or in association with damage to the parietal lobe.

In addition to exhibiting the above symptoms, many adults also experience aphasia, which is a difficulty in expressing oneself when speaking, in understanding speech, or in reading and writing.

In children

There are few reports of the syndrome, sometimes called developmental Gerstmann syndrome, in children. The cause is not known. Most cases are identified when children reach school age, a time when they are challenged with writing and math exercises. Generally, children with the disorder exhibit poor handwriting and spelling skills, and difficulty with math functions, including adding, subtracting, multiplying, and dividing. An inability to differentiate right from left and to discriminate among individual fingers may also be apparent. In addition to the four primary symptoms, many children also suffer from constructional apraxia, an inability to copy simple drawings. Frequently, there is also an impairment in reading. Children with a high level of intellectual functioning as well as those with brain damage may be affected with the disorder.

Treatment

There is no cure for Gerstmann syndrome. Treatment is symptomatic and supportive. Occupational and speech therapies may help diminish the dysgraphia and apraxia. In addition, calculators and word processors may help school children cope with the symptoms of the disorder.

Prognosis

In adults, many of the symptoms diminish over time. Although it has been suggested that in children symptoms may diminish over time, it appears likely that most children probably do not overcome their deficits, but learn to adjust to them.

Sources

• Template:NINDS

External links

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