All over the world, stroke is a leading cause of death and disability.1 Alongside this, stroke can lead to numerous other complications including brain oedema, pneumonia, urinary tract infections, seizures, deep vein thrombosis (DVT), and Alzheimer’s disease.
Like stroke, Gerstmann syndrome is a severe neurological condition. It is rare and controversial within the medical community due to several factors, ranging from its symptoms to the location of the lesions. The type of lesions present in Gerstmann syndrome indicate that stroke can play a role in its development. In this article, we are going to explore the connection between Gerstmann syndrome and stroke.
Understanding the Connection Between Gerstmann Syndrome and Stroke
Overview of Gerstmann Syndrome and Stroke
What is Gerstmann Syndrome?
Joseph Gerstmann first saw a rare condition in 1924: Gerstmann syndrome, a neurological condition targeting the brain's angular gyrus region.2 The lesions in the area produce the following tetrad of symptoms:
- Finger agnosia
- Agraphia
- Acalculia
- Right-left disassociation
In total, it causes you to lose the ability to:
- Write
- Name your fingers
- Ability to do basic math functions
- Distinguish between left and right
These symptoms appear because of the location of the angular gyrus region and its responsibility. The angular gyrus lies in the middle of the parietal, occipital, and temporal lobes. This location makes it a key player in integrating, processing, and connecting the different functional areas. Hence, it has a hand in the following brain functions:
- Spatial cognition
- Language processing
- Attention
- Number processing
- Memory retrieval
- Social cognition
- Conflict resolution
Gerstmann syndrome can also appear in children — when this occurs, a paediatrician diagnoses it as developmental Gerstmann syndrome. You can notice it through its effects on the child's following abilities:
- Spelling
- Handwriting
- Basic maths skills
The nature of Gerstmann syndrome will cause a massive reduction in the following activities listed above.
What is stroke?
Stroke is a syndrome — it is a collection of acute focal and neurological deficits caused by vascular injuries, such as infarction or haemorrhage. You cannot compare stroke with diseases like malaria because stroke abides by a different rule — it has no definitive cause, risk factor, disease process, or mechanism.
There are three types of stroke:
Ischaemic stroke is the cause of 85% of all stroke cases and cardiovascular conditions often cause it.3
Regardless of the type, when a stroke occurs, the blood vessels in the brain can become blocked or burst open. This is bad because the brain relies on these blood vessels to receive nutrients and oxygen. Therefore, brain cells can starve without them, leading to their death. The death of brain cells can impact brain function and the extent of the impact depends on factors such as:
- The type of stroke
- Severity of the stroke
- Location of the stroke
What is the connection between Gerstmann Syndrome and stroke?
Stroke leaves lesions when it occurs — Gerstmann syndrome happens if it appears in the angular gyrus area. We can see this pattern in the man highlighted in 4's case study. The man came into the hospital experiencing a stroke — he had a history of cardiovascular issues and diabetes mellitus.
After medical intervention post-ischaemic stroke, the physician carried out a neuropsychological assessment to check the extent of damage done by the stroke. The evaluation found that the patient had difficulty with:
- Ability to read
- Ability to identify simple objects
- Repeating things, such as naming simple objects
- Writing
- Performing basic mathematics functions
- Recognising his fingers
- Differentiating between left and right
All these symptoms indicated one thing: Gerstmann syndrome had set in due to the ischaemic stroke the man suffered.4 This study is not the only one with this finding — another notable study evaluated over 194 acute stroke patients and found 30.4% positive with a combination of the symptoms associated with Gerstmann syndrome.5
What makes you vulnerable to developing Gerstmann Syndrome after a stroke?
To recap, stroke is not a single disease with one risk factor or disease mechanism but rather a spectrum. You can say the same thing about developing Gerstmann syndrome after a stroke. Despite this, studies suggest that a specific type of lesion, ischaemic lesions, can lead to Gerstmann syndrome.4,5 This is because ischaemic stroke causes these lesions and this type of stroke is a risk factor for Gerstmann syndrome. Other risk factors include:
- Carotid artery dissecting aneurysm
- Middle cerebral artery aneurysm
- Chronic subdural haematoma
- Multiple sclerosis
- Cortical atrophy
The following can also put you at risk for developing an ischaemic stroke and, by extension, Gerstmann syndrome:
- Diabetes mellitus
- Hypertension
- Atrial fibrillation
- Use of anabolic steroids
- Coronary artery bypass graft surgery
- Mitral valve replacement
- Older age
- High cholesterol levels
- Sickle cell anaemia
- Cardiomyopathy
- Heart valve diseases
- A clotting disorder
- History of stroke
How to Diagnose Gerstmann Syndrome Post Stroke?
There are three things involved for a physician to diagnose you with Gerstmann syndrome post-ischaemic stroke:
Neuropsychological assessment
It is the first examination carried out by physicians post-ischaemic stroke. The physician’s target is to assess for the tetrad symptoms of Gerstmann syndrome.
Finger agnosia
To test for finger agnosia, the physician will ask you to identify the finger being touched, first with your eyes open, and then with your eyes closed. If you can locate your fingers in both instances, the physician will rule out finger agnosia.
Agraphia
Testing for agraphia involves three phases:
- The physician shows you simple everyday objects and asks you to name them
- The physician presents a simple sentence, asks you to read it, and then asks you to write it down after repeating it to you
- The physician asks you to repeat a simple phrase and explain it
If you can do all these, the physician can rule out agraphia.
Acalculia
To test for acalculia, the physician takes you through the following steps:
- The physician will ask you to perform basic maths, like subtraction, on paper
- The physician will ask you to do simple multiples without paper or written instructions
If you can do these, the physician can rule out acalculia.
Right-to-left disassociation
For right-to-left disassociation, the physician takes you through the following steps:
- The physician gives you written instructions
- The physician asks you to verbalise them to avoid misunderstandings
- The physician asks you to perform the action
The instructions should involve a simple hand movement that includes both hands. If you can perform this, the physician will rule out right-to-left disassociation.
Magnetic Resonance Imaging / Computed Tomography (MRI/CT)
The physician does these imaging techniques to view the ischemic lesions in the angular gyrus region with or without the involvement of the surrounding areas.
Rule out other conditions with similarities
The physician rules out similar conditions to confirm the diagnosis of Gerstmann syndrome after the neuropsychological assessment and MRI or CT. The physician also aims to rule out the following conditions:
- Alzheimer’s disease
- Posterior cortical atrophy
- Disconnection syndrome
- Occipital lobe lesion
How to Live with Gerstmann Syndrome Post-stroke?
Gerstmann syndrome has no cure, but you can manage it with help from professionals. The target of Gerstmann syndrome management is symptomatic and supportive. Symptomatic management involves your physician treating you based on your present symptoms. Since ischaemic stroke is the underlying cause, the physician may give you warfarin (a medication to prevent blood clots) to prevent reoccurrence.
Supportive care aims to help you cope and improve your present difficulties. Some supportive care options are outlined here.
Neuropsychological rehabilitation
This involves improving your current cognitive, behavioural, psychosocial and emotional issues caused by the stroke. This rehabilitation could be facilitated by:
- Occupational therapy: this helps you develop compensatory techniques to assist you with your current difficulties
- Cognitive therapy: this enables you to improve numerical processing skills
- Speech and language therapy helps with speech difficulties through techniques like Copy and Recall Treatment (CART)
Use of assistive technologies
Like our cars and phones, they are technologies that can help improve your daily life with these symptoms. Some of these include:
- Calculators
- Word processing software
- Electronic spell checker
- Speech cognition
Finally, supportive educational intervention tailored to your needs can also be useful in rehabilitation from Gerstmann syndrome and stroke.
Summary
Gerstmann syndrome is a rare condition that has some past controversies. Despite this, it is a neurological condition that presents with a tetrad of symptoms that can occur after an ischemic stroke. Thus, the best way to prevent Gerstmann syndrome is through the prevention of the stroke itself. This means you should take active measures such as:
- Eating a healthy diet
- Keeping a healthy weight
- Stop smoking
- Limiting your alcohol intake
- Controlling your cholesterol levels
- Watch your blood pressure
- Monitor and follow up on your existing heart conditions like hypertension
- Watch and follow up on your diabetes
- Take your medications as prescribed
References
- Feigin VL, Brainin M, Norrving B, Martins S, Sacco RL, Hacke W, et al. World Stroke Organization (WSO): Global Stroke Fact Sheet 2022. International Journal of Stroke [Internet]. 2022 Jan 5;17(1):18–29. Available from: https://journals.sagepub.com/doi/10.1177/1747493021106591
- Rusconi E. Chapter 20 - Gerstmann syndrome: historic and current perspectives [Internet]. Vallar G, Coslett HB, editors. Vol. 151, ScienceDirect. Elsevier; 2018. p. 395–411. Available from: https://www.sciencedirect.com/science/article/abs/pii/B9780444636225000206
- Murphy SJX, Werring DJ. Stroke: Causes and clinical features. Medicine. 2020 Sep;48(9):561–6.
- Tekgol Uzuner G, Ubur A, Erten M, Uzuner N. A Rare Clinical Antity; Pure Gerstmann Syndrome. Journal of Stroke and Cerebrovascular Diseases. 2020 Oct;29(10):105161.
- Zukic S, Mrkonjic Z, Sinanovic O, Vidovic M, Kojic B. GERSTMANN’S SYNDROME IN ACUTE STROKE PATIENTS. Acta Informatica Medica [Internet]. 2012 Dec 1;20(4):242–3. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3558292/