Overview

What Is Gerstmann Syndrome?

Gerstmann syndrome is a neurological disorder characterised by the loss of four specific cognitive functions, which are:

• Agraphia (inability to write)
• Acalculia (failure to do mathematics) 
• Finger agnosia (inability to recognise fingers of one’s self or of others)
• Left-right disorientation (inability to distinguish the right from the left side of the body)¹

What Causes Gerstmann Syndrome?

Gerstmann syndrome occurs due to damage involving a specific area of the brain responsible for sensation, perception, and understanding of sensory input. This area is present in the parietal lobes of the brain (upper side lobes). The cause of impairment in adults is mostly due to a stroke,  involving this area, other rare causes include brain tumour, or trauma to the brain in this area. In children, the cause is often unknown, known as Developmental Gerstmann syndrome.^1,2

What Are The Core Symptoms of Gerstmann Syndrome and Their Effects on Daily Life

Agraphia (Inability to write)

Impact: Difficulty writing results in problems with writing tasks and written communication.

Examples: writing letters, doing homework, signing documents, and filling forms.³

Acalculia (Inability to do mathematics)

Impact: difficulties processing mathematical functions and calculations, whether in written or verbal context. 

Examples: Managing money, organising activities and employment, managing medications,  and shopping.⁴

Finger Agnosia (Inability to recognise fingers)

Impact: difficulty recognising and moving fingers results in problems with hand function and manual tasks.

Examples: Difficulty moving fingers selectively, difficulty pointing or naming others’  fingers, and difficulty typing. ⁵

Left-Right Disorientation

Impact: difficulty following directions and navigation, difficulty determining right from left of others.

Examples: Taking wrong directions, difficulty driving, difficulty using maps, and problems when dealing with patients in healthcare settings.⁶

Cognitive and Emotional Impact

Rare diseases can cause considerable emotional impact on patients and caregivers.

According to a survey conducted across the United Kingdom and United States, patients with rare disease reported that their disease caused: 

• Depression 
• Anxiety and stress 
• Isolation from friends and family 
• Less interaction with friends and family
• Worry based on the future of their disease 
• Worry based on lack of information available on disease

In addition to patients, caregivers of rare disease patients also experienced similar psycho-social concerns including feelings of:

• Depression 
• Anxiety and stress 
• Isolation from friends and family 
• Less interaction with friends and family 
• Worry based on the future outlook of the disease 
• Worry based on lack of information available on disease⁷

In Gerstmann syndrome, patients are subjected to the psychological impact of the disorder, these include frustration and anxiety, difficulties with social interactions, problems with work and independence 

Frustration and Anxiety

The symptoms of inability to write, calculate, use fingers accurately, and problems with directions cause difficulty performing daily routine tasks leading to feelings of frustration.

Social Implications

Patients with Gerstmann syndrome experience difficulties with communication with others which can affect their social relationships.

Work and Independence

Patients with Gerstmann syndrome can experience difficulties and limitations in performing their jobs and living independently.

Evaluation of patients with Gerstmann Syndrome 

Clinical examination 

In adults, the presence of all four symptoms suggests the diagnosis of Gerstmann syndrome after excluding other causes. 

In children, it is usually identified at school age when affected children experience difficulties in doing math, writing, and spelling.¹ 

Imaging studies 

All patients having symptoms suggesting Gerstmann syndrome need to undergo brain imaging either with computed tomography (CT) or magnetic resonance imaging (MRI). Interpretation of brain images to detect any abnormalities involving the parietal lobe (upper side lobes) especially an area called “angular gyrus” and the surrounding areas. Different abnormalities affecting this area are responsible for the various causes of Gerstmann syndrome.²

Management and Support Strategies

In adults with Gestmann syndrome, the treatment is focused on treating underlying conditions like the removal of the brain tumour or haemorrhage. On the other hand, children with developmental Gerstmann syndrome usually need special education and rehabilitation according to the symptoms experienced by each patient.^1,2 

Patients can exhibit an excellent recovery after rehabilitation and treatment, only acalculia may have delayed recovery. In children, early recognition of the condition is associated with better outcomes and improvement.²

The form of rehabilitation and support measures needed for the patient is tailored according to the patient’s condition 

Occupational Therapy

 To improve functional independence in everyday tasks at work. 

Speech and Language Therapy

Special forms of speech therapy and exercises to assist patients with their speech and communication abilities.

Assistive Technologies and Support Networks 

Patients can use various tools to simplify daily tasks such as calculators and speech-to-text devices. In addition, having a family, caregiver, or support group surrounding patients is important to assist patients with their needs and provide them with emotional support. 

Summary 

Gerstmann syndrome is a rare neurological condition characterised by the presence of loss or difficulty in performing four neurological functions, including an inability to write (agraphia), failure to do math (acalculia), difficulty with recognising and using fingers (finger agnosia), and difficulty with distinguishing right from left sides (left-right disorientation). The underlying cause is an abnormality involving a specific area of the brain in the upper side lobes of the brain (parietal lobes) whether due to a stroke in this area, trauma, or a brain tumour. Another form present in children is known as developmental Gerstmann syndrome and the underlying cause is unknown, it is usually diagnosed when children enter school and experience difficulties with math and writing. The difficulties patients experience in performing daily tasks can affect their work, jobs, daily living, family, and social relationships, in addition to the emotional impact and anxiety on patients themselves. The management includes correcting the underlying abnormality when present and utilising rehabilitation and support techniques to improve the functional ability of these patients.