Introduction

Adult-onset Gertsmann Syndrome is not necessarily a common issue among older people as it is mostly diagnosed among children during school age. It is not hereditary meaning it doesn’t run in the family. It comes on as a co-morbidity to other diseases that occur due to age such as strokes, tumours, and many others we will go through later.¹

With this article, we will investigate what this disease is, how it can be diagnosed within this population, and even the differential diagnosis that can occur with the Gerstmann syndrome.

Disease discovery

This syndrome was discovered by an Austrian Neuroscientist in 1924 named Josef Gerstmann.² He initially observed some of the symptoms in a few of his patients. Some of these symptoms range from:

• An impairment in performing calculations³
• Finger agnosia⁴
• Agraphia¹

This tetrad of symptoms leads Gerstmann to the conclusion that they were due to a lesion of the dominant parietal lobe of the brain.²

Why learn about Adult-onset Gerstmann Syndrome?

The symptoms of this syndrome are capable of critically and holistically affecting the lives of the people affected. These disruptions can come from something like someone not knowing their left to right, having difficulties writing, conducting simple math equations like additions and subtractions, and many other issues.⁵ Therefore, knowing more about this disease and its effects can aid in increasing awareness of the syndrome and can contribute to reducing under/misdiagnosing this disease.

Additionally, a deeper understanding of this syndrome can identify links to more serious conditions such as brain injuries, neurodegenerative diseases and/or stroke. This knowledge could facilitate earlier treatments, minimising potential complications from delayed interventions and ultimately reducing mortality rates. Raising awareness of this syndrome can improve the quality of life for those affected, while also advancing medical knowledge and research. 

Symptoms of Adult-onset Gertsmann Syndrome

Gertsmann syndrome can be identified through the following symptoms, unlike when it affects children these symptoms are caused by various issues. It is also important to note that in adults, many symptoms of adult-onset Gertsmann syndrome may diminish over time.

These symptoms are:⁵

• Agraphia or Dysgraphia, a writing disability
• Acalculia or Dyscalculia, a lack of understanding of the rules for calculation or arithmetic
• An inability to distinguish left to right and vice-versa
• Finger agnosia, an inability to identify fingers
• Aphasia, is a difficulty in expressing oneself when speaking, or in understanding speech or in reading and writing. This symptom is not always experienced by patients with adult-onset Gertsmann syndrome
• Additional cognitive defects may occur in some patients

Causes of Adult-onset Gertsmann Syndrome

Adult-onset Gertsmann Syndrome can be caused by the following:⁵

• Ischemic stroke
• Tumours
• Stenosis
• Middle cerebral artery aneurysm
• Progressive multifocal leukoenuphalopathy
• Multiple sclerosis
• Cortical atrophy
• Alcoholism
• Carbon monoxide poisoning
• Lead intoxication
• Anaphylactic shock
• Systemic lupus

Risk factors of Adult-onset Gertsmann Syndrome

Age and underlying health conditions are the most coveted risk factors associated with adult-onset Gertsmann Syndrome. Furthermore, an increase in this syndrome’s susceptibility is due to the diseases that come with ageing as mentioned above, with lifestyle factors having some effects as well. 

Diagnosis

The easiest and most effective way to diagnose adult-onset Gertsmann syndrome is through neuroimaging; MRI or a CT scan. These methods will easily detect any abnormalities in the dominant angular gyrus with or without the involvement of surrounding areas, with abnormalities differing from cause to cause.

The role of imaging techniques when diagnosing adult-onset gertsmann syndrome

Magnetic Resonance Imaging (MRI)

When a patient is going through an acute phase, an MRI can detect the functioning of certain brain regions. This method can detect multiple sclerosis, which can be a cause of Gertsmann Syndrome, which appears as patches of damage in the brain.⁶ The MRI can detect changes in the meninges (outer layer of the brain), blood vessels and the formation of lesions.

Computed Tomography (CT) scan

These are used to detect any low-density areas in the brain or any damaged tissue in the brain that can lead to Gertsmann Syndrome. A CT scan can also detect a brain bleed, also called Chronic Subdual Hematoma.⁷

A CT scan can help spot an infarction in the brain which can lead to symptoms of Gertsmann Syndrome.

Single Photon Emission Computed Tomography (SPECT) scan

SPECT scan utilises a special tracer, known as technetium-99 which detects areas of the brain with blockages or low blood flow.⁵

MR spectroscopy

 This measures oxygen levels in the brain to see if there’s an increase in lactate which can lead to symptoms of Gertsmann syndrome in adults.⁵

Electroencephalograph (EEG)

This scan measures brain waves, whereby patients with Gertsmann syndrome, have slow brain waves.⁵

Angiography

This scan looks at blood vessels in the brain and can help detect Stenosis; the narrowing of the middle cerebral artery.⁵

Cerebral Fluid Analysis (CSF)

This can aid in detecting increased white blood cells in the fluid around the brain hence indicating an infection or inflammation in the brain. Inflammation in the brain can lead to symptoms of Gertsmann syndrome.⁵

Differential diagnosis

For Adult-onset Gertsmann syndrome, additional tests are critical before making a diagnosis, due to a chance of misdiagnosis Several other diseases and syndromes share similar symptoms and could be mistaken for Gerstmann syndrome, including: 

• Progressive posterior cortical atrophy, symptom shared is atrophy⁸
• Occipital lobe lesions⁹
• Disconnection syndrome⁴
• Alzheimer’s disease, due to the degeneration of brain networks¹⁰

Treatment and management options

With Adult-onset Gertsmann Syndrome, treatment is symptomatic and supportive because the syndrome itself has no cure.⁴ It is also important to note that with this syndrome, symptoms among adults tend to diminish and fade over time. Causes and risk factors such as tumours, strokes, or carbon monoxide poisoning can be treated for recovery and other causes such as alcoholism need cognitive behavioural therapy.¹¹

Occupational and speech therapy is also an option to combat some of the symptoms of Gertsmann syndrome such as agraphia.

Managing daily life and maintaining the quality of life of patients with Adult-onset Gertsmann Syndrome

Due to this syndrome affecting adults that already have an established way of living, it is very difficult to adjust to this disease and its symptoms as they have the capability of changing your way of living,¹² whereby there are many tools to make speech and mathematical challenges easier

For patients with finger agnosia, you may incorporate visual aids for finger identification and left-to-right confusion. Some people also wear bracelets on each arm with an indication of which wrist is left or right to make distinguishing easier.

Adding cognitive behavioural therapy can also be beneficial to aid in managing anxiety or depression due to the rapid changes in one’s life. This may aid the patient with acceptance of the onset of symptoms during adulthood and the lifestyle change one must adopt.

Summary

Adult-onset Gertsmann syndrome is not a common occurrence but due to some very common diseases, it can occur to certain people. The myriads of symptoms range from agraphia, acalculia, an inability to distinguish right to left, and finger agnosia. This syndrome does not have a cure, but treatment can come in multiple forms of cognitive therapy and treating the causes of these diseases.

Adult-onset Gertsmann Syndrome is not hereditary but, the symptoms fade over time. It can be primarily diagnosed using an MRI and CT scan.

Knowing more about this disease creates awareness that will aid health practitioners during diagnosis and reduce any kind of panic patients diagnosed with this syndrome may have. This is why more research needs to be done to ensure more information about Gertsmann syndrome is available to healthcare providers and the public.

Gertsmann Syndrome is a rare occurrence among adults. It is caused by various issues and diseases that can affect the body and the brain such as strokes, tumours, multiple sclerosis, carbon monoxide poisoning, and many others. Adult-onset Gertsmann Syndrome has no cure, so cognitive and speech therapy is one of the most efficient methods that aid in reducing this syndrome’s symptoms. Together with treating the causal diseases can aid in diminishing the disease's symptoms altogether.

Adult-onset Gertsmann Syndrome is primarily diagnosed using an MRI or CT scan. SPECT scans, MR spectroscopy, EEG, Angiography, and a CSF can also be used to diagnose Adult-onset Gertsmann Syndrome if the primary methods do not work.

Keep in mind that with this syndrome it is incredibly important that it is diagnosed well as it has a couple of differential diagnoses. These range from Alzheimer’s disease to disconnection syndrome and a couple more neurological diseases. This is why spreading correct and evidence-based information about Adult-onset Gertsmann syndrome is very important to ensure awareness among health professionals and the public.