Gerstmann Syndrome - Causes, Symptoms, Diagnosis, and Management

Introduction:

Gerstmann syndrome is a brain disorder characterized by four classical cognitive defects. The syndrome usually occurs as a result of brain injury or developmental disorders. The condition affects both men and women and can develop at any age. When the disease affects children, it is known as developmental Gerstmann syndrome. However, according to some researchers, developmental Gerstmann syndrome is not a unique syndrome, but it is more likely to be a collection of symptoms resulting from another underlying condition. Moreover, the diagnosis procedure involves neuroimaging to determine the cause of the condition, and the treatment usually involves removing the underlying cause, rehabilitation, and counseling.

What Is Gerstmann Syndrome?

Gerstmann syndrome is a neurological disorder resulting from developmental disorders, trauma, or injury to the brain. This syndrome is characterized by a set of four hallmark symptoms, which include:

• Agraphia or Dysgraphia: Inability to write or express thoughts in the form of writing.

• Acalculia: Inability to perform simple mathematic calculations.

• Finger Agnosia: Inability to recognize their own or others’ fingers.

• Left-Right Disorientation: Inability to differentiate between left and right sides.

In addition, apart from these four symptoms, other systemic symptoms and cognitive defects may also be present. Josef Gerstmann, an Austrian neuroscientist, in the year 1924, first recognized this medical condition. The syndrome is also known as developmental Gerstmann's syndrome and is documented in seemingly normal but brain-damaged children who present with learning disabilities. Gerstmann syndrome is distinct from Gerstmann-Sträussler-Scheink, an uncommon and inherited degenerative brain disorder.

What Are the Other Names for Gerstmann Syndrome?

Other names of Gerstmann syndrome are:

• GS.

• Developmental Gerstmann syndrome.

• Gerstmann tetrad.

What Causes Gerstmann Syndrome?

Adults who have experienced a stroke or other type of brain damage that impairs blood flow to the brain may develop the syndrome. Gerstmann syndrome affects the parietal lobe of the brain, which is part of the brain responsible for sensation, perception, and recognizing sensory output. Gerstmann syndrome, in rare cases, may arise due to brain trauma or brain tumors. In children, the exact cause of Gerstmann syndrome is unknown. However, some of the common causes of Gerstmann syndrome are:

• Ischemic stroke (a condition in which the blood supply to certain brain parts is decreased or interrupted).

• Brain tumors.

• Carotid artery dissecting aneurysm (a medical condition in which the layers of the carotid artery are separated).

• Stenosis (narrowing of a passage in the body).

• Middle cerebral artery aneurysm (abnormal focal dilation of a brain artery).

• Progressive multifocal leukoencephalopathy (disease affecting the white matter of the brain occurring due to viral infection).

• Chronic subdural hematoma (collection of blood and its breakdown products between the brain surface and its outermost covering).

Other symptoms may include:

• Chronic alcoholism.

• Carbon monoxide toxicity.

• Lead poisoning.

• A severe allergic reaction that is frequently life-threatening in response to an antigen to which the body becomes sensitive (anaphylactic shock).

• A complication of cerebral angiography.

• A complication of contrast is used in angiography.

• Adverse reaction of the drug Acetazolamide.

Is Gerstmann Syndrome a Common Condition?

• Gerstmann syndrome is a rare condition. However, the exact incidence rate of the condition in the general population is still unknown.

• It is not unusual to encounter a patient who exhibits two to three of the characteristic symptoms of this syndrome, but it is rare to find a patient who exhibits all four symptoms of the syndrome.

• Gerstmann syndrome affects both men and women equally.

• The condition can affect individuals of all ages.

Is Gerstmann Syndrome an Inherited Condition?

Gerstmann is not an inherited condition which means parents cannot genetically pass down the condition to their children. Therefore, Gerstmann syndrome does not run in families.

What Are the Symptoms of Gerstmann Syndrome?

Signs and symptoms of Gerstmann syndrome are:

• Inability to do simple mathematic calculations.

• Inability to distinguish between left and right.

• Inability to recognize fingers.

• Inability to write or express thoughts in the form of writing.

• Two-way disturbance of comprehension and naming (semantic aphasia).

• The presence of all four of these neurologic dysfunctions in a person with learning impairments is quite uncommon. The classic syndrome is only present when all four symptoms co-occur without mental retardation.

• Affected individuals may occasionally experience difficulty in communicating verbally and/or comprehending other people's speech in addition to the four classic symptoms. Additionally, they might have trouble with reading and spelling.

• Affected children exhibit poor handwriting, poor mathematic skills, and spelling problems.

• Depending on the underlying reason, this syndrome may also be accompanied by additional symptoms such as apraxia (inability of the brain to deliver correct instructions of movement to the body), visual ataxia (the inability to reach for or aim accurately at an item under visual guidance), cognitive impairment, numbness, or weakness.

• Toe agnosia is often associated with finger agnosia; therefore, it may be called digit agnosia instead of finger agnosia.

How Is Gerstmann Syndrome Diagnosed?

A thorough medical history and physical examination should be performed to diagnose this syndrome and direct the physician to identify its underlying cause. The diagnostic procedures include:

• Study the Symptoms: The four elements of Gerstmann's syndrome should be identified with a special neurological evaluation.

1. Finger Agnosia: The patient's eyes should be covered, and the doctor should ask them to extend their fingers. The doctor should lightly touch the patient's fingers, and the patient should react by identifying the finger. If the patient has tactile insensitivity, the test may be impacted. A minimum of 20 % false recognition is required to confirm the diagnosis of finger or digit agnosia.

2. Right-Left Disorientation: The doctor should hand the patient a card with written instructions, like "put the left hand to the right ear," and ask them to read it aloud. If the patient fails to read it correctly, the doctor should read it to the patient, and then the patient is asked to perform similar instructions.

3. Dysgraphia: The doctor may show the patient some particular objects and ask them to write their names. Moreover, the patient may also be asked to explain specific instructions.

4. Dyscalculia: The patient should be given a card with a simple equation and asked to write it down and solve it. After that, the patient should be asked to multiply the equation without writing it down or looking at it.

• Imaging Tests: A neuroimaging scan like magnetic resonance imaging (MRI) or CT scan (computed tomography) should be done to diagnose any patient exhibiting the symptoms and signs of Gerstmann's syndrome. Abnormalities are primarily noticed in the posteroinferior region of the brain's parietal lobe, with or without the involvement of the surrounding areas. In the case of an infarct, several abnormalities may be seen in an MRI or CT scan. The abnormalities usually vary depending on the cause of the condition.

How Is Gerstmann Syndrome Treated?

• In the case of developing Gerstmann syndrome, special education, relevant rehabilitation, and counseling can be used as treatment. However, neurological evaluation is crucial to differentiate between the possible causes of the syndrome. In adults, the underlying neurological problem needs to be treated. Surgery may be performed to treat conditions with a brain tumor or injury. In some adults, Gerstmann syndrome symptoms get better with time.

• In some cases, the causes of Gerstmann's syndrome are treatable and reversible, such as when a tumor, hemorrhage, epilepsy focus lesion, or diffuse etiology like carbon monoxide poisoning.

Conclusion:

Gerstmann syndrome is a rare condition with challenging diagnosis and treatment procedures. The severity of the right-left disorientation caused by Gerstmann's disease, combined with other disorders, can make leading a regular life impossible. However, intense speech therapy may be able to help children with developmental Gerstmann's syndrome, especially if they are identified early. After extensive rehabilitation and treatment, a patient with Gerstmann's syndrome may exhibit excellent recovery. However, acalculia may improve at a slower pace. Furthermore, children with developmental Gerstmann's syndrome might show improvement in the syndrome, albeit this necessitates early detection and identification of the syndrome.