Gerstmann syndrome

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at October 2, 2016
StartDiseasesGerstmann syndrome

Gerstmann syndrome is named after neurologist Josef Gerstmann for his contributions to the advancements of scientific research and awareness of this neurological disorder and disease. It is a rare disease and it is not to be confused with Gerstmann-Sträussler-Scheinker syndrome which is a genetic disorder and neurogenerative disease. It is sometimes known as developmental Gerstmann's syndrome.

Contents

Definition & Facts

Gerstmann Syndrome is a rare neurological disorder causing cognitive impairment that occurs as a result of a traumatic brain injury or a developmental disorder. This disorder occurs as a result of bruising or brain injury typically to the left cerebral hemisphere of the brain and can be equally found in both children and adults alike. 

The number of those suffering from Gerstmann syndrome remains unknown, and there are questions as to the number of people that are living with Gerstmann’s syndrome around the world. This disorder was first identified and described by Dr. Josef Gerstmann in 1924. Since then, advancements in diagnosing and caring for Gerstmann syndrome have continued to advance. 

Symptoms & Complaints

The syndrome is largely defined and characterized by the loss or absence of four cognitive abilities:

  • The ability to express thoughts in writing (agraphia, dysgraphia)
  • The ability to perform and carry out simple arithmetic or mathematical problems (acalculia)
  • The ability to recognize or identify one's own or another's fingers (finger agnosia)
  • The ability to distinguish between the right and left sides of one's body

Three of the most common recognizable symptoms in affected children are that they may have poor handwriting, less than average spelling abilities and struggle to carry out basic math skills like adding, subtracting, dividing and multiplying. 

Children may also have trouble reading or understanding certain words, remembering their meanings and spelling them out. Basic tasks, like copying a simple object or tracing basic lines also appear to be problematic for children with Gerstmann’s Syndrome. 

When it comes to handwriting, children and adults alike may have difficulty distinguishing between upper and lower case letters, may write pages full of unfinished letters and demonstrate an inconsistency in shape and size of written letters. 

In addition to these symptoms being present, some patients also have speech disorders and language disorders. They may display symptoms of aphasia, which is the difficulty in expressing oneself clearly and thoroughly when speaking.

Patients with aphasia have trouble forming thoughts and expressing them in complete sentences. They may also have trouble understanding the speech of others and in reading and writing. 

Causes

Gerstmann syndrome, scientifically speaking, occurs in the presence of a contusion or bruise in one of the lobes of the brain; specifically it tends to affect the angular gyrus which is in the inferior parietal lobule.

In adults, Gerstmann syndrome is more likely to occur after a stroke and can occur as a result of stroke. Other times, the disease is caused by impaired or restricted blood flow to the brain, which is also known as cerebrovascular disease. Multiple sclerosis can also cause this syndrome.

In even rarer cases, Gerstmann’s syndrome can occur after having suffered from a traumatic brain injury or as a result of a brain tumor in the same part of the brain. Gerstmann syndrome affects both males and females equally.

Diagnosis & Tests

In order to make a proper diagnosis of Gerstmann syndrome many tests need to be performed. Some of these tests are more complicated than others and some indications of a person holding this disease are far more obvious than others. Finger agnosia may be difficult to diagnose among children because many healthy children cannot name their fingers.

An individual's medical history and family history will be inquired about. A physical examination is a standard component of any diagnostic exam. Neurological examinations which assess a person's sensory and motor responses, balance, and coordination.

Certain medical imaging exams such as magnetic resonance imaging (MRI) scans and computed tomography (CT) scans may be used to view the brain and assess its function and structure. A biopsy of the brain may confirm the presence of lesions.

Treatment & Therapy

Unfortunately, at the current moment, there is no cure for Gerstmann's syndrome. Symptoms are tended to as needed and treatment is largely supportive, meaning that it attempts to abate symptoms rather than necessarily cure the underlying disease.

Because the symptoms for Gerstmann’s syndrome are so diverse and affect so many basic and foundational parts of daily living, there has been a variety of treatment options to help prevent further deterioration of certain skills. 

To help diminish the effects of dysgraphia and apraxia it has been proven helpful for patients to participate in some form of occupational therapy and/or speech therapy. Engaging in regular cognitive and speech therapies can help improve one’s ability to express their thoughts in writing and to verbalize them, an important and necessary function for day-to-day life. Special education and the use of calculators may be used to assist children with the syndrome.

In rarer cases, when Gerstmann’s syndrome is present due to a tumor or existing brain injury, surgery may be used to treat the condition. Natalizumab is one medication that has been used to treat Gerstmann syndrome that results from multiple sclerosis.

Prevention & Prophylaxis

For adults who suffer from the syndrome as a result of traumatic brain injury or stroke, certain preventative approaches involve steps to prevent those conditions. Wearing appropriate headgear while engaging in extreme or contact sports and riding motorcycles can help prevent traumatic brain injury. Exercising caution while riding in motor vehicles can include wearing seat belts and can help prevent traumatic brain injury.

To the extent that strokes are preventable through lifestyle factors, adopting a healthy diet and engaging in regular exercise may mitigate the risk. Quitting smoking or never starting to begin with as well as avoiding secondhand smoke may also mitigate the risk of stroke and conditions that result from it such as Gerstmann syndrome.