Gerstmann syndrome is a
neuropsychological
Neuropsychology is a branch of psychology concerned with how a person's cognition and behavior are related to the brain and the rest of the nervous system. Professionals in this branch of psychology often focus on how injuries or illnesses of ...
disorder that is characterized by a constellation of symptoms that suggests the presence of a
lesion
A lesion is any damage or abnormal change in the tissue of an organism, usually caused by disease or trauma. ''Lesion'' is derived from the Latin "injury". Lesions may occur in plants as well as animals.
Types
There is no designated classifi ...
usually near the junction of the
temporal and
parietal lobes at or near the
angular gyrus
The angular gyrus is a region of the brain lying mainly in the posteroinferior region of the parietal lobe, occupying the posterior part of the inferior parietal lobule. It represents the Brodmann area 39.
Its significance is in transferring vis ...
. Gerstmann syndrome is typically associated with damage to the
inferior parietal lobule
The inferior parietal lobule (subparietal district) lies below the horizontal portion of the intraparietal sulcus, and behind the lower part of the postcentral sulcus. Also known as Geschwind's territory after Norman Geschwind, an American neurolo ...
of the
dominant hemisphere
The lateralization of brain function is the tendency for some neural functions or cognitive processes to be specialized to one side of the brain or the other. The median longitudinal fissure separates the human brain into two distinct cerebra ...
. It is classically considered a left-hemisphere disorder, although right-hemisphere damage has also been associated with components of the syndrome.
It is named after
Jewish Austrian-born American
neurologist
Neurology (from el, νεῦρον (neûron), "string, nerve" and the suffix -logia, "study of") is the branch of medicine dealing with the diagnosis and treatment of all categories of conditions and disease involving the brain, the spinal c ...
Josef Gerstmann
Josef Gerstmann (July 17, 1887, Lemberg – March 23, 1969, New York City) was a Jewish Austrian-born American neurologist.
Gerstmann studied Medicine at the Medical University in Vienna, then capital of the Austro-Hungarian Empire, between 1906 ...
.
Symptoms
Gerstmann syndrome is characterized by four primary symptoms, collectively referred to as a tetrad:
#
Dysgraphia
Dysgraphia is a learning disability of written expression, which affects the ability to write, primarily handwriting, but also coherence. It is a specific learning disability (SLD) as well as a transcription disability, meaning that it is a wri ...
/agraphia: deficiency in the ability to write
#
Dyscalculia
Dyscalculia () is a disability resulting in difficulty learning or comprehending arithmetic, such as difficulty in understanding numbers, learning how to manipulate numbers, performing mathematical calculations, and learning facts in mathematics. ...
/acalculia: difficulty in learning or comprehending mathematics
#
Finger agnosia Finger agnosia, first defined in 1924 by Josef Gerstmann, is the loss in the ability to distinguish, name, or recognize the fingers—not only the patient's own fingers, but also the fingers of others, and drawings and other representations of finge ...
: 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
The supramarginal gyrus is a portion of the parietal lobe. This area of the brain is also known as Brodmann area 40 based on the brain map created by Korbinian Brodmann to define the structures in the cerebral cortex. It is probably involved wit ...
(
Brodmann area 39 and 40 respectively) near the
temporal and
parietal lobe
The parietal lobe is one of the four major lobes of the cerebral cortex in the brain of mammals. The parietal lobe is positioned above the temporal lobe and behind the frontal lobe and central sulcus.
The parietal lobe integrates sensory informa ...
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 letters and words into meaningful information, such as is done while reading.
In adults
In adults, the syndrome may occur after a
stroke
A stroke is a medical condition in which poor blood flow to the brain causes cell death. There are two main types of stroke: ischemic, due to lack of blood flow, and hemorrhagic, due to bleeding. Both cause parts of the brain to stop functionin ...
.
In addition to exhibiting the above symptoms, many adults also experience dysphasia or
aphasia
Aphasia is an inability to comprehend or formulate language because of damage to specific brain regions. The major causes are stroke and head trauma; prevalence is hard to determine but aphasia due to stroke is estimated to be 0.1–0.4% in th ...
,
which is 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 mathematic 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 have
constructional apraxia Constructional apraxia is characterized by an inability or difficulty to build, assemble, or draw objects. Apraxia is a neurological disorder in which people are unable to perform tasks or movements even though they understand the task, are willing ...
, an inability to copy simple drawings. Frequently, there is also an impairment in reading. Children at any level of intelligence may be affected with the disorder.
Diagnosis
Diagnosis may be clinical if associated with
dementia
Dementia is a disorder which manifests as a set of related symptoms, which usually surfaces when the brain is damaged by injury or disease. The symptoms involve progressive impairments in memory, thinking, and behavior, which negatively affe ...
and other etiologies. In cases caused by stroke,
MRI
Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio waves ...
will show a corresponding stroke in the
inferior parietal lobule
The inferior parietal lobule (subparietal district) lies below the horizontal portion of the intraparietal sulcus, and behind the lower part of the postcentral sulcus. Also known as Geschwind's territory after Norman Geschwind, an American neurolo ...
. In the acute stage, this will be bright (restricted diffusion) on the DWI sequence and dark at the corresponding area on the ADC sequence.
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 a similar diminishing of symptoms occurs in children as well, it appears more likely that most do not overcome their deficits, but instead simply learn to adjust.
See also
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Pure alexia
Pure alexia, also known as agnosic alexia or alexia without agraphia or pure word blindness, is one form of alexia which makes up "the peripheral dyslexia" group. Individuals who have pure alexia have severe reading problems while other language-r ...
, another left sided lesion that does not result in agraphia, despite being next to the area where lesions cause Gerstmann syndrome.
References
Further reading
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External links
{{dyslexia
Specific developmental disorders
Aphasias
Syndromes affecting the nervous system
Rare syndromes