What is Gerstmann Syndrome?
In 1924, an Austrian brain scientist named Josef Gerstmann discovered an unusual brain disorder. This condition was noted in a small number of his patients and is characterized by four specific symptoms. These include difficulties with doing math calculations (a condition called acalculia), identifying their own fingers (known as finger agnosia), writing by hand (a disability named agraphia), and differentiating left from right (referred to as left-right disorientation).
Interestingly, this collection of symptoms, now called Gerstmann’s syndrome, is also seen in children who appear to have no physical abnormalities but do have brain damage. These children often struggle with learning, and Gerstmann’s syndrome is referred to as developmental Gerstmann’s syndrome in these cases.
What Causes Gerstmann Syndrome?
Gerstmann’s syndrome is a medical condition that is caused by a specific damage to certain parts of the brain. The brain damage usually occurs in the posterior lobule of the parietal lobe in the dominant hemisphere. The dominant hemisphere is typically the left side of the brain, but for some people, it could be the right side. The region most affected is the angular gyrus, which is located where the parietal, temporal and occipital lobes meet.
Some studies have concluded that Gerstmann’s syndrome can also be caused by a lesion in the left middle frontal lobe of the dominant hemisphere, which is another region of the brain.
The factors that could lead to this brain damage and cause Gerstmann’s syndrome include:
– Ischemic stroke, which happens when blood supply to a part of your brain is reduced.
– Tumors in the brain.
– Higher-than-normal pressure or a blood clot in the carotid artery, which is a major artery that carries blood from the heart to the brain.
– A middle cerebral artery aneurysm, which is a weak or thin spot on an artery in the brain that balloons out and fills with blood.
– Progressive multifocal leukoencephalopathy, which is a rare and usually fatal viral disease.
– Chronic subdural hematoma, which consists of a pool of blood forming between the brain and its outermost covering.
– Multiple sclerosis, a chronic disease that affects the brain and spinal cord.
– Cortical atrophy, which involves changes in the brain’s cortex due to aging.
Other widespread causes can include alcoholism, carbon monoxide poisoning, lead intoxication, anaphylactic shock (severe allergic reaction), and systemic lupus erythematosus, which is an inflammatory disease.
Some other cases of Gerstmann’s syndrome have been reported as a result of cystic lesions with enlarged perivascular spaces, as a complication of cerebral angiography (a procedure to see blood flow in the brain), as a result of the contrast used in angiography, as a complication of endovascular treatment of dural arteriovenous malformation (an abnormal connection between blood vessels in the brain), and posterior leukoencephalopathy syndrome. It can also be caused by necrotizing granulomatous inflammation of small-medium-sized subarachnoid vessels, adverse reaction to the drug Acetazolamide, and embolism from a type of noncancerous heart tumor called left atrial myxoma.
In some instances, transient or temporary symptoms of Gerstmann’s syndrome may occur as a result of a condition known as parietal lobe epilepsy.
Risk Factors and Frequency for Gerstmann Syndrome
Gerstmann’s syndrome can affect people of all ages, including children and the elderly. It is often referred to as “developmental Gerstmann’s syndrome” when found in children. Patients often exhibit two or three signs of the syndrome, but it’s rare for someone to have all the symptoms of the full syndrome.
Signs and Symptoms of Gerstmann Syndrome
Gerstmann’s syndrome is a condition with various symptoms. These include difficulty identifying fingers (finger agnosia), trouble doing simple math calculations (acalculia), disorientation between left and right, and difficulty writing (agraphia). In some cases, agraphia may occur due to involvement of the superior parietal gyrus. Acalculia is often related to semantic aphasia, which involves the angular gyrus. These symptoms can occur partially or fully in both elderly and children. Additionally, one might face other cerebral symptoms. Interestingly, Gerstmann’s syndrome might occur as a temporary condition due to partial epilepsy.
In children, symptoms and signs of this condition may appear later in life due to perinatal asphyxia (lack of oxygen at birth), with seizures being the only symptom during infancy. Other accompanying symptoms can vary based on the cause, such as apraxia (difficulty performing purposeful movements), optic ataxia (poor hand-eye coordination), cognitive decline, numbness or weakness. Also, the syndrome might manifest in children as developmental Gerstmann’s syndrome, possibly with or without dyspraxia as a fifth symptom.
To make a diagnosis, doctors perform a thorough history and physical examination. Certain specific neurological tests are carried out to diagnose the four aspects of Gerstmann’s syndrome:
- Finger Agnosia: Patients are asked to identify which finger is being touched while their eyes are covered.
- Right-left disorientation: Patients are tested on following written instructions involving the left and right sides of the body.
- Dysgraphia: Patients’ writing abilities are tested by asking them to write objects, numbers, and sentences as they read them.
- Dyscalculia: Patients’ ability to perform basic calculations and mental math is evaluated.
The severity of Gerstmann’s syndrome can be linked to the size of the brain lesion causing it.
Testing for Gerstmann Syndrome
If you’re experiencing signs and symptoms of Gerstmann’s syndrome, your doctor might suggest a brain imaging test called an MRI or CT scan. These tests can help identify any abnormalities in a specific part of your brain, called the dominant angular gyrus. An abnormality in this area could suggest Gerstmann’s syndrome, especially if neighboring areas of the brain are also affected. However, the exact abnormalities in these imaging results can vary, depending on the underlying cause of the syndrome.
If the cause of the syndrome is due to an interruption of blood supply to the brain (infarction), different images might show up on the CT scan like low-density or high-density focus in the parietal lobe of your brain. An MRI, in this case, might show irregularities in the way water molecules are moving in your brain. Furthermore, other medical imaging techniques, like single photon emission computed tomography (which shows how blood flows through your arteries) might be utilized to show decreased blood flow and increased lactate levels in the brain. Other electrical tests could indicate abnormal brain wave activity. Angiography, an X-ray imaging test of blood vessels, might show narrowing in the middle cerebral artery.
Information from research indicates that even the non-affected areas of your brain might have decreased blood flow.
If you have chronic subdural hematoma, a condition where blood collects on the surface of the brain, the CT scan may show a less dense fluid collection around the brain.
In individuals with multiple sclerosis, MRI images could reveal plaques (areas of damage) in certain regions of the brain, while in a condition called posterior leukoencephalopathy syndrome, MRI images could show abnormally high-intensity lesions in the gray and white matter of the brain.
In case of a certain type of inflammation caused by an immune response (necrotizing granulomatous inflammation), there might be an increased number of immune cells in the fluid surrounding the brain and spinal cord. The MRI results might also show swollen blood vessels in the brain.
It’s also possible to detect lesions in the parietal lobe from other disorders, such as lesions caused by the JC virus. However, these may also extend beyond the parietal lobe to other areas of the brain.
It’s important to note that diagnosing Gerstmann’s syndrome can be challenging in children as the parietal lobes of the brain (where this syndrome arises) develop slowly during childhood.
Treatment Options for Gerstmann Syndrome
Gerstmann’s Syndrome, which is a neurological disorder, can sometimes be treated depending on its cause. For instance, if it’s caused by a tumor, a surgical procedure to remove the tumor can help. Similarly, if it’s due to a bleeding inside the brain, handling the bleed can alleviate the symptoms. In instances where the condition is caused by an area of the brain responsible for seizures, treating that area can help. Lastly, if it results from widespread causes like carbon monoxide poisoning, treating the poisoning can lead to improvement.
What else can Gerstmann Syndrome be?
: When looking into Gerstmann’s syndrome, doctors also consider some other conditions that have similar symptoms. These include:
- Posterior cortical atrophy, which also involves difficulty with writing (agraphia) and can show signs of neglecting one side of the body, difficulty with hand-eye coordination, and difficulty reading out loud (verbal alexia).
- Certain damages to the back part of the brain (occipital lobe lesions), which can also involve the same difficulty with writing and reading.
- Alzheimer’s disease, which brings about changes in the brain’s networks. There might be a link between Alzheimer’s disease and Gerstmann’s syndrome. The Syndrome of Progressive Posterior Cortical Dysfunction can also cause similar symptoms and sometimes even leads to Gerstmann’s syndrome.
In some cases, a condition called “disconnection syndrome” could be the correct diagnosis.
For the version of Gerstmann’s syndrome that appears during a person’s development, doctors might also consider learning disorders like developmental dyscalculia.
What to expect with Gerstmann Syndrome
Gerstmann’s syndrome, when present with other health conditions, can greatly disrupt regular life activities, particularly because of the severe light-left disorientation it causes. However, for children diagnosed with developmental Gerstmann’s syndrome, their condition can improve significantly with intensive speech training, especially when the diagnosis is made early on.
Possible Complications When Diagnosed with Gerstmann Syndrome
As the symptoms of Gerstmann’s syndrome increase, it signifies a larger brain lesion, which is a damaged part of the brain, leading to more severe neurological impairment. Patients who show all four symptoms of Gerstmann’s syndrome are more likely to have intense brain function impairment, affecting their life expectancy.
Key Points:
- More symptoms of Gerstmann’s syndrome suggest a larger brain lesion
- Large brain lesions lead to more severe brain function impairment
- Showing all four symptoms of Gerstmann’s syndrome indicates a high likelihood of serious brain function impairment
- Severe impairment can affect life expectancy
Recovery from Gerstmann Syndrome
People suffering from Gerstmann’s syndrome can show significant improvement after thorough treatment and rehabilitation. However, recovering from acalculia, a condition which makes it hard for them to do simple math, may take longer. Also, children who have developmental Gerstmann’s syndrome, a rare childhood disorder, can improve their condition substantially. But, this would need the condition to be identified and diagnosed early on.
