Gerstmann syndrome

Gerstmann syndrome is a very rare neurological disorder characterized by tetrad of symptoms of an impairment in performing calculations (acalculia), discriminating their own fingers (finger agnosia), distinguishing left from right (left-right disorientation) and writing by hands (agraphia), which is supposed to be secondary to a focal subcortical white matter damage in the parietal lobe ¹. Additional cognitive defects may occur in some cases.

Finding a patient with two to three symptoms of Gerstmann syndrome is not rare but finding a patient with the full Gerstmann syndrome is rare ².

Gerstmann syndrome is also described in brain-damaged children, who present with learning disability, where it’s called developmental Gerstmann’s syndrome ³.

Gerstmann syndrome has not been found to run in families ⁴. In extremely rare cases, children who are bright and functioning intellectually at a high level may be affected by Gerstmann syndrome as well as those who suffer brain damage.

Gerstmann syndrome is different from Gerstmann-Sträussler-Scheinker syndrome, a rare genetic degenerative brain disorder.

Gerstmann syndrome causes

Gerstmann’s syndrome is caused by specific brain lesions which affect the posterior lobule of the parietal lobe in the dominant hemisphere ², which is usually in the left hemisphere but in some patients it could be the right ⁵, especially the angular gyrus and adjacent structures (at the confluence of parietal, temporal and occipital lobes) ⁶, some studies showed that Gerstmann’s syndrome caused not only by lesion in the mentioned area but also in the left middle frontal lobe of the dominant hemisphere ⁷.

The cause of Gerstmann syndrome may be from:

• Ischemic stroke ²
• Tumors ⁸
• Carotid artery dissecting aneurysm or stenosis ⁹
• Middle cerebral artery aneurysm ¹⁰
• Progressive multifocal leukoencephalopathy ¹¹
• Chronic subdural hematoma ¹²
• Multiple sclerosis ¹³
• Cortical atrophy ¹⁴

Other diffuse causes are:

• Alcoholism ¹⁵
• Carbon monoxide poisoning ¹⁶
• Lead intoxication ¹⁵
• Anaphylactic shock ¹⁵
• Systemic lupus erythematosus (SLE) ¹⁵

Other reported cases presented with Gerstmann’s syndrome, like cystic lesions with dilated perivascular spaces ¹⁷, as a complication of cerebral angiography ¹⁸, as a complication of the contrast which is used in angiography ¹⁹, as a complication of endovascular treatment of dural arteriovenous malformation ²⁰, posterior leukoencephalopathy syndrome ²¹, necrotizing granulomatous inflammation of small-medium sized subarachnoid vessels ²², adverse drug reaction of Acetazolamide and embolism from left atrial myxoma ²³.

Transient symptoms of Gerstmann syndrome may occur as a result of parietal lobe epilepsy ²⁴.

Because of the variation of the causes, Gerstmann syndrome can occur in children, young and elderly patients, where it has been called “developmental Gerstmann’s syndrome” in children ³.

Gerstmann syndrome pathophysiology

The symptoms of Gerstmann’s syndrome can be explained by a disorders affecting specific areas of the brain, but some disagreements between the studies about the exact localization of the disease ²⁵, for an example finger agnosia and acalculia responsible sites were found in the supramarginal gyrus or close to the intraparietal sulcus, and involvement of the superior parietal gyrus was founded to be responsible for agraphia, further studies are needed to know whether the syndrome occurs as a result of angular lesion with or without subcortical extension ²⁵.

Several hypotheses have been mentioned in order to explain Gerstmann’s syndrome, but none of this hypothesis has been confirmed ²⁵.

Several authors have suggested the defect which causes some symptoms (acalculia, right-left disorientation, and finger agnosia) of this syndrome is in the mental spatial rotations, it’s thought that the problem in this syndrome is in restricting the degree ’s freedom of spatial rotation; especially defective horizontal mapping ²⁶.

It is thought also that pure form of Gerstmann’s syndrome is caused by disconnection from a subcortical parietal white matter lesion which affects crossing fiber tracts and disconnects separate cortical networks ²⁵.

Gerstmann’s syndrome symptoms

The symptoms of Gerstmann’s syndrome should include the loss of four specific neurological functions:

1. the inability to identify one’s own or another’s fingers (finger agnosia),
2. the loss of the ability to do mathematics (acalculia), in which patients understand the numbers but cannot perform a simple addition,
3. inability to make the distinction between the right and left side of the body (left-right disorientation)
4. inability to write (dysgraphia or agraphia).

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 Gerstmann 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.

A study suggested that acalculia is always associated with semantic aphasia ²⁷, which has a significant involvement in the angular gyrus, and the paper suggested that the syndrome should include semantic aphasia instead of agraphia, the later may occur in case of superior parietal gyrus involvement ²⁵, symptoms of Gerstmann syndrome may be complete or partial ¹⁷ and may be attributed to other cerebral symptoms and can occur in elderly and children ⁷.

Gerstmann’s syndrome may be transient and caused as an ictal symptom in partial epilepsy, but it may occur as three symptoms out of 4 ²⁸.

The symptoms and signs of Gerstmann syndrome in children may appear late in the years after perinatal asphyxia and may only suffer from other symptoms in the neonatal period; seizures ²⁹.

Other symptoms and signs which may accompany Gerstmann syndrome differ according to the cause of this syndrome such as; apraxia ³⁰, optic ataxia ³¹, cognitive decline ¹⁵, numbness or weakness ³². Gesture imitation defects and toe agnosia may be seen as an association with finger agnosia so it may be named as digit agnosia ³³.

Developmental Gerstmann’s syndrome occurs in children with the tetrad of the mentioned symptoms, with or without dyspraxia as a fifth symptom ³⁴.

Gerstmann syndrome diagnosis

To diagnose Gerstmann syndrome a complete history and physical examination should be done to guide the physician toward the cause of it. 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).

Some special neurological examination should be done to diagnose the 4 components of Gerstmann’s syndrome:

1. Finger Agnosia: The examiner should cover the patient eyes and ask him to stretch his fingers, then the examiner should touch the patient fingers lightly and the patient should respond by identifying the finger as soon as it was touched, this examination should be done firstly with opened eyes to eliminate the misunderstanding or lack of alertness, this test may be affected if the patient has tactile insensitivity, a minimum 20% false recognition by the patient is needed to diagnose the patient with finger agnosia.
2. Right-left disorientation: physician should give the patient a card with written instruction such as “ place left hand to right ear” and the patient should be asked to read it loudly, if the patient read it incorrectly, the physician should contact with the patient verbally, then the patient should be asked to do similar instruction.
3. Dysgraphia: physician should show a clock to the patient, and the patient should write the name of it without saying it firstly, then physician should show the word “SEVEN” to the patient, and after having read it, and presenting it orally, patient should be asked to write it, then the patient should repeat the sentence “ He shouted the warning” and should explain it and write it.
4. Dyscalculia: a patient should be given a card which has this simple equation written on it “85-27” and he should be asked to write it and calculate it, then the patient should be asked to do a multiplying equation in his head without writing it or seeing it.

As the number of Gerstmann’s syndrome component increased the responsible brain lesion tend to be larger ³⁵.

Imaging studies

Any patient with the symptoms and signs of Gerstmann’s syndrome should be evaluated by neuroimaging MRI/CT scan ³⁶, abnormalities should be seen in the dominant angular gyrus with or without the involvement of the surrounding areas, abnormalities that should be seen in the imaging differs according to the cause of the syndrome.

In infarction it may be detected in CT scan as a low-density focus, but also a high-density focus in the parietal lobe has been reported in one patient with Gerstmann’s syndrome ³⁷, MRI in the acute phase will show an abnormally reduced diffusivity , technetium-99 bicisate single proton emission computed tomography will also show a decreased cerebral blood flow with an increased lactate in MR spectroscopy and slowed waves in the affected hemisphere in electroencephalography ¹⁷ and angiography may show stenosis in the middle cerebral artery ¹⁷.

Studies showed the cerebral blood flow would be decreased in the non-infarcted areas also ⁷.

In patients with chronic subdural hematoma computerized tomographic (CT) scan will see an extra-axial hypodense fluid collection ¹².

In multiple sclerosis, brain MRI will show plaques in the mentioned areas, and in posterior leukoencephalopathy syndrome, MRI T2-weighted images will show an abnormal high-intensity lesion in the gray and white matter ¹³.

In necrotizing granulomatous inflammation will cause a lymphocytic pleocytosis in the cerebrospinal fluid and marked meningeal enhancement on MRI with dilated cortical venules ²².

Other lesions from other disorders in the parietal lobe may be detected by MRI such as JC virus, the lesions may not be limited to the parietal lobe, but may involve other lobes and other areas ¹¹.

Diagnosis of Gerstmann’s syndrome in children may be impossible at the early stages because parietal lobes have slow functional development during childhood ³⁸.

Gerstmann syndrome differential diagnosis

The differential diagnosis for Gerstmann’s syndrome includes:

• Posterior cortical atrophy which shares the symptom of agraphia with Gerstmann’s syndrome, other symptoms includes; hemineglect, optic ataxia, and verbal alexia ³⁹
• Some occipital lobe lesions which include agraphia with alexia ⁴⁰
• Alzheimer disease, which causes degeneration of the networks in the brain, it’s considered a differential to Gerstmann’s syndrome and may have an association with it, also the Syndrome of progressive posterior cortical dysfunction may cause similar symptoms, and sometimes causing the syndrome itself ⁴¹
• The possibility of disconnection syndrome should be considered as a diagnosis in some cases ²

As a differential for developmental Gerstmann’s syndrome is the disorders which cause learning disabilities, such as developmental dyscalculia ⁴².

Gerstmann’s syndrome 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.

Some causes of Gerstmann’s syndrome may be reversible and can be treated such as, removing the tumor ⁴³, the hemorrhage ¹² or epilepsy focus lesion ⁴⁴ or treating the diffuse etiology such as carbon monoxide poisoning ¹⁰.

Gerstmann syndrome prognosis

As the number of Gerstmann’s syndrome components increase the responsible lesion tend to be larger and the neurological impairment tend to be larger also; every patient with the 4 components of this syndrome is more likely to have severe impairment of brain function and the lesion tend to compromise the survival of the patient ³⁵.

Presence of Gerstmann’s syndrome with other disorder can make the normal life impossible, mainly due to the severe light-left disorientation ⁴⁵. But children who present with developmental Gerstmann’s syndrome may show an improvement with intensive speech training especially when it’s diagnosed early ⁴⁶.

Patient with Gerstmann’s syndrome may show an excellent recovery after intensive rehabilitation and treatment, but acalculia may have a delay in the recovery ¹⁸. Also, children with developmental Gerstmann’s syndrome may exhibit improvement in the syndrome, but this may require early recognition and diagnosis of the syndrome ⁴⁶.

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