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Posted by on Jun 6, 2013 in Disease & conditions | 0 comments

Gerstmann syndrome

Gerstmann syndrome

 

 

Gerstmann syndrome is a neurological disorder that is characterized by a constellation of symptoms that suggests the presence of a lesion in a particular area of the brain. (It should not be confused with Gerstmann-Sträussler-Scheinker syndrome, which is a transmissible spongiform encephalopathy.)

It is named for Josef Gerstmann.

In 1924, Gerstmann was observed in some patients an amazing neurological problem where victims of brain injury became unable to name even differentiate their fingers apart.This loss of ability to distinguish his own fingers (called finger agnosia) was accompanied by dysgraphia , dyscalculia and an inability to distinguish left from right . He defined from these four symptoms syndrome that bears his name on the assumption that these disabilities are linked to an injury of the parietal lobe dominant. Since then, the syndrome Gerstmann has been  an enigma for neuropsychologists .

Primary symptoms 

  • Gerstmann syndrome is characterized by four primary symptoms:
  • Dysgraphia/agraphia: deficiency in the ability to write
  • Dyscalculia/acalculia: difficulty in learning or comprehending mathematics
  • Finger agnosia: inability to distinguish the fingers on the hand
  • Left-right disorientation

Causes 

This disorder is often associated with brain lesions in the dominant (usually left) hemisphere including 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. The angular gyrus is generally involved in translating visual patterns of letter and words into meaningful information, such as is done while reading.

In adults & In children 

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.

Among the adult victims of Acute Cerebral Vascular Accident s dyscalculia, only 3% are affected by both dysgraphia, by finger agnosia and disorientation left-right . Gerstmann Syndrome is aclinical entity rare but precisely associate haslesions located in gyrus angle of the dominant hemisphere .

The occurrence of these symptoms may be due to the fact that the brain areas affected are local ones. The angular gyrus is a region of association and multimodal brain receives stimuli both auditory, visual and somatosensory. Neurons in this region are very well placed to deal with the phonological and semantic aspects of language that allows the identification and categorization of objects.

The finger agnosia resulting in the inability to associate the correct name for each finger. The person is not able to appoint another hand on the same finger pointed at his own hand.

Dysgraphia syndrome Gertsmann results in specific disorders handwriting spontaneous or dictation. The letters are sometimes performed correctly but are not assembled in the form of words. There is in this case no error word for another: it is a disorder of the arrangement of letters. In other cases the construction of letters itself is difficult.

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 impairment in reading. Children with a high level of intellectual functioning as well as those with brain damage may be affected with the disorder.

In children, Gerstmann is in a slightly different form, which includes dyslexia, finger agnosia, right-left confusion (of self and others), dysgraphia and dyscalculia. It is called the developmental Gerstmann. A tetralogy of classic symptoms dysgraphia, finger agnosia, dyscalculia, often adds a constructive dyspraxia, sometimes with a disorder of the geographical spatial orientation. While Gerstmann in adults is often marked by speech disorders (aphasia kind of), they are very rare in the child syndrome. The relationship between syndromes in adults and children are still research topics.

 

It is estimated that a large number of children suffering from developmental Gerstmann are undiagnosed; these children compensate by various strategies the main effects of the syndrome.

Different methods for assessing the finger agnosia have been proposed by different authors (Benton, Kinsbourne, Lefford, Birch and Green). Kinsbourne method consists of three tests: first, the differentiation of fingers, then the subject must say how many fingers are intermediate between the two fingers are affected, and finally the subject wearing a blindfold on eyes, receives an object placed in his hand, and once the mask removed, must recognize the object among others.

These three tests can be administered in two ways: in the first, the show about the finger that had been requested by the examiner in the second, he will appoint the finger touched by the examiner. Kinsbourne had however recommended not insisting on the appointment of the fingers, to avoid confusion with a lack of learning the names of the fingers, for example. Sometimes adds an appreciation of graphesthésie (recognizing, without seeing signs painted on the fingers or palm).

For children Gerstmann syndrome can cause great difficulties in school learning, not only because of the impact of dysgraphia on their cursive writing. Problems left right distinction, constructive difficulties and deficits in mental arithmetic have a significant impact in their daily lives. The disorder is misunderstood by the children themselves, but also by parents and teachers. We regularly says that these young patients lack motivation, are uncooperative, are stubbornly clumsy or inadequate intelligence. This negative stigma generates anxiety, family tensions, and is conducive to the installation of a poor self-image. This is why the diagnosis and understanding of the syndrome and its implications are important for these children and their families.

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. When a student suffers from its writing, the problem is adysgraphia proven or just a passing disorder, it is important to act as soon as possible.

First, we must understand that a child in trouble with writing is necessarily suffering in school if nothing is done to help. Let him suffer harm in pursuit of his studies, so we have a vested interest in not letting the problem persist.

In the case of a learning disability, rehabilitation of writing should help resolve the problem. This intervention will be early in the life of the child, the shorter will be suffering in school, and fewer will be the consequences of this bad start with the writing.

For dysgraphia proved more we take the problem earlier the chances of a successful rehabilitation are good. This does not mean that we cannot fix the problem at any age, but with a teenager’s risk of failure due to lack of follow up on a few months of rehabilitation are more important than a young child. For Also, do not forget that if it comes to adaptations to the school because of dysgraphia, it is easier to succeed with a single teacher (which is usually the primary case) only by a multiple educational team (ten teachers per school level at least).

A rehabilitation of writing well done takes less than eight sessions, spaced 3 to 5 weeks (for people with no particular disability, and by scrupulously writing exercises requested). I personally observed the firm that rehabilitations last on average between 4 and 5 sessions. However, the duration of therapy varies greatly depending on many parameters:

Motivation: it is essential to apply themselves to complete assignments each day, including weekends and holidays. A equal problems, a motivated person will always be someone better drag force for rehabilitation and who do not see the point

The problem: it is sometimes possible that work on a specific point (holding the pen, followed by the line of writing, for example) unlocks all writing. Rehabilitation can be sometimes very fast. In other cases, dysgraphia, it will work every node that blocks the action before restoring fluid and legible writing that one is entitled to expect.

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.

References:

Miller CJ, Hynd GW (April 2004). “What ever happened to developmental Gerstmann’s syndrome? Links to other pediatric, genetic, and neurodevelopmental syndromes”. J. Child Neurol. 19 (4): 282–9.

 

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