Gerstmann Syndrome
Overview
Gerstmann's syndrome is a cognitive impairment that results from damage to a specific area of the brain known as the left parietal lobe in the region of the angular gyrus. It may occur after a stroke or in association with damage to the parietal lobe. It is characterized by four primary symptoms:
A writing disability (agraphia or dysgraphia)
A lack of understanding of the rules for calculation or arithmetic (acalculia or dyscalculia)
An inability to distinguish right from left
An inability to identify fingers (finger agnosia)
Many adults also experience aphasia, (difficulty in expressing oneself when speaking, in understanding speech, or in reading and writing).
The disorder should not be confused with Gerstmann-Sträussler-Scheinker disease, a type of transmissible spongiform encephalopathy.
There are few reports of the syndrome, sometimes called developmental Gerstmann's 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
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 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.
There is no cure for Gerstmann's syndrome. Treatment is symptomatic and supportive. Occupational and speech therapies may be beneficial. Calculators and word processors may help school children cope with the symptoms.
In adults, many 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.
SYNONYMS
Developmental Gerstmann Syndrome
Gerstmann Tetrad
GS
SIGNS & SYMPTOMS
Gerstmann syndrome is a rare disorder characterized by the loss of four specific neurological functions: Inability to write (dysgraphia or agraphia), the loss of the ability to do mathematics (acalculia), the inability to identify one’s own or another’s fingers (finger agnosia), and inability to make the distinction between the right and left side of the body. It is very rare for a person with learning disabilities to have all four of these neurologic dysfunctions. Only when all four symptoms appear together without mental retardation is the classic syndrome present.
When affected individuals have all four of the characteristic symptoms of Gerstmann syndrome without other cognitive defects, the condition may be referred to as “pure” Gerstmann syndrome. However affected individuals usually have other defects in addition to the classic four findings of Gerstmann syndrome. In addition, many individuals have only two or three of the four key findings in combination with other types of cognitive defects.
In such cases in addition to the four classical symptoms, affected individuals may also have difficulty expressing themselves through speech, and/or difficulty understanding another person’s speech (aphasia). They may experience difficulty in reading and spelling as well.
A few cases have been reported in children and called developmental Gerstmann syndrome. These cases usually become apparent when children begin school. Affected children may demonstrate poor handwriting, spelling and math skills (e.g., difficulty adding, subtracting, dividing and multiplying). Some children have difficulty reading or understanding written words (alexia) and difficulty copying or tracing simple objects (constructional apraxia).
Some researchers suggest that developmental Gerstmann syndrome is not a true, unique syndrome, but rather a group of symptoms caused by another, underlying disorder.
CAUSES
In adults, the syndrome can arise in adults as a result of impaired blood flow to the brain (cerebrovascular disease) such as a stroke or other damage to the brain. The parietal lobes (upper side lobes) of the brain are affected in Gerstmann syndrome. The parietal lobes are involved with sensation and perception as well as understanding sensory input.
In rare cases, traumatic brain injury or a brain tumor in the same region of the brain can cause the various symptoms associated with Gerstmann syndrome.
The cause of Gerstmann syndrome in children is often unknown. Although in some cases it may be linked to brain damage, children without brain damage can also be affected.
AFFECTED POPULATIONS
Gerstmann syndrome affects males and females in equal numbers. The incidence of Gerstmann syndrome in the general population is unknown. The disorder was first described by Dr. Josef Gerstmann, a Viennese neurologist, in 1924.
DISORDERS WITH SIMILAR SYMPTOMS
Symptoms of the following disorders can be similar to those of Gerstmann syndrome. Comparisons may be useful for a differential diagnosis:
Alzheimer disease is a progressive condition of the brain that affects memory, thought, and language. The degenerative changes of Alzheimer disease lead to patches or plaques in the brain and the entanglement of nerve fibers (neurofibrillary tangles). Memory loss and behavioral changes occur as a result of these changes in brain tissue. The characteristic findings associated with Gerstmann syndrome can occur due to Alzheimer disease. (For more information on this disorder, choose “Alzheimer’s” as your search term in the Rare Disease Database).
Additional disorders may cause brain dysfunction and could potentially cause the four characteristic findings associated with Gerstmann syndrome. These characteristic findings have also been seen in alcoholics, lupus, carbon monoxide poisoning, and lead poisoning.
DIAGNOSIS
The presence in the adult of all four neurological symptoms suggests a diagnosis of Gerstmann syndrome, especially when other causes of these symptoms are ruled out. Among children, most cases are recognized at school age when the affected person has difficulty in math and writing. Affected children may also have problems in spelling, performing the basic four mathematical calculations, and distinguishing left from right. Also, they generally fail the finger identification test. Many, but not all such children will find it difficult to copy simple drawings (constructional apraxia).
STANDARD THERAPIES
Treatment
Treatment of Gerstmann syndrome in developmental cases will involve special education and related rehabilitation and counseling services. Neurological examination is necessary to tell the difference between the two causes of the condition. In adults, treatment of the underlying neurological condition is necessary. When brain injury or tumor is involved, surgery may be used to alleviate the condition. In some cases, the symptoms affecting adults with Gerstmann syndrome diminish over time.
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