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Neurological Examination, clinical cases and neuropsychological ...

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23/07/54<br />

Gerstmann syndrome is a neurological disorder that is characterized by<br />

a constellation of symptoms that suggests the presence of a lesion in a particular area of<br />

the brain.<br />

Gerstmann syndrome is characterized by four primary symptoms:<br />

►Dysgraphia/agraphia: deficiency in the ability to write<br />

► Dyscalculia/acalculia: difficulty in learning or comprehending mathematics<br />

► Finger agnosia: inability to distinguish the fingers on the h<strong>and</strong><br />

► Left‐right disorientation<br />

Causes<br />

This disorder is often associated with brain lesions in the dominant (usually left)<br />

hemisphere including the angular <strong>and</strong> supramarginal gyri near the temporal <strong>and</strong> parietal<br />

lobe junction. There is significant debate in the scientific literature as to whether<br />

Gerstmann Syndrome truly represents a unified, theoretically motivated syndrome. Thus<br />

its diagnostic utility has been questioned by neurologists <strong>and</strong> neuropsychologists alike.<br />

The angular gyrus is generally involved in translating visual patterns of letter <strong>and</strong> words<br />

into meaningful information, such as is done while reading.<br />

In adults<br />

In adults, the syndrome may occur after a stroke or in association with damage to the<br />

parietal lobe. In addition to exhibiting the above symptoms, many adults also experience<br />

aphasia, which is a difficulty in expressing oneself when speaking, in underst<strong>and</strong>ing<br />

speech, or in reading <strong>and</strong> writing.<br />

In children<br />

There are few reports of the syndrome, sometimes called<br />

Developmental Gerstmann syndrome, in children. The<br />

cause is not known. Most <strong>cases</strong> are identified when children reach<br />

school age, a time when they are challenged with writing <strong>and</strong> math<br />

exercises. Generally, children with the disorder exhibit poor<br />

h<strong>and</strong>writing <strong>and</strong> spelling skills, <strong>and</strong> difficulty with math functions,<br />

including adding, subtracting, ti multiplying, l i <strong>and</strong> dividing. idi An inability<br />

to differentiate right from left <strong>and</strong> to discriminate among individual<br />

fingers may also be apparent.<br />

In addition to the four primary symptoms, many children also suffer<br />

from constructional apraxia, an inability to copy simple drawings.<br />

Frequently, there is also an impairment in reading. Children with a<br />

high level of intellectual functioning as well as those with brain<br />

damage may be affected with the disorder.<br />

Bálint's syndrome is an uncommon <strong>and</strong> incompletely understood triad<br />

of severe <strong>neuropsychological</strong> impairments involving space representation<br />

(visuospatial processing). Its three major components are<br />

1) Simultanagnosia, i.e., the inability to perceive the visual field as a whole,<br />

2) Ocular apraxia, a deficit of visual scanning, <strong>and</strong> Apraxia—inability to carry out<br />

familiar movements when asked to do so<br />

3) Optic ataxia, an impairment of pointing <strong>and</strong> reaching under visual guidance.<br />

The syndrome was named in 1909 for the Austro‐Hungarian neurologist Rezső Bálint who<br />

had been the first to identify it. Since it represents impairment of both visual <strong>and</strong><br />

language functions, it is a significant disability that can affect the patient's safety even in<br />

one's own home environment, <strong>and</strong> can render the person incapable of maintaining<br />

employment. Lack of awareness of this syndrome may lead to a misdiagnosis <strong>and</strong><br />

resulting inappropriate or inadequate treatment. Therefore, clinicians should be familiar<br />

with Bálint's syndrome <strong>and</strong> its various etiologies.<br />

Balint's syndrome occurs most often with an acute onset as a consequence of<br />

multiple bilateral strokes. The most frequent cause of complete Balint's syndrome is<br />

said by some to be sudden <strong>and</strong> severe hypotension, resulting in bilateral<br />

borderzone infarction in the occipito‐parietal region. More rarely, <strong>cases</strong> of<br />

progressive Balint's syndrome have been found in degenerative disorders such as<br />

Alzheimer's disease or certain other traumatic brain injuries at the border of the<br />

parietal <strong>and</strong> the occipital lobes of the brain.<br />

Acalculia<br />

7/23/2011 NEUROPSYCHIATRY 226<br />

Body Schema Disturbance<br />

415703 Cognitive Neuropsychology<br />

Week 6:<br />

The Temporal lobes<br />

7/23/2011 NEUROPSYCHIATRY 227<br />

Naiphinich Kotchabhakdi, Ph.D.<br />

Naiphinich Kotchabhakdi, Ph.D.<br />

Director, Salaya Stem Cell R & D Project,<br />

Research Center for Neuroscience,<br />

Institute of Molecular Biosciences,<br />

Mahidol University Salaya Campus,<br />

999 Phutthamonthol 4 Road, Salaya, Phutthamonthol,<br />

Nakornpathom 73170 Thail<strong>and</strong><br />

Email: scnkc@mahidol.ac.th or naiphinich@gmail.com<br />

Web: www.neuroscience.mahidol.ac.th<br />

38

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