Gerstmann Syndrome

Definition

Gerstmann’s syndrome abbreviated as GS is also referred as the developmental Gerstmann syndrome or the Gerstmann tetrad, which is a rare neurological problem that affects the dominant parietal lobe of the brain, specifically located at the angular gyrus and supramarginal gyrus near the junction point of the parietal lobe and the temporal lobe. This disorder might involve the superior lobes and inferior lobes of the brain.

GS has not been proved to be noted among families. There are some rare instances though that those children who are performing well and who function on a higher level of intelligence can have the chance to be affected by this problem, the same chance that those with brain damage may also have GS. It can be a problem of both males and females having equal chances.

GS is not associated with Gerstmann-Sträussler-Scheinker syndrome, which is considered as a rare genetic degenerative disorder affecting the brain.

Symptoms of Gertsmann’s Syndrome

Gerstmann’s syndrome is being specifically present and characterized by the loss of four neurological functions:

• Dysgraphia or agraphia pertains to a writing disability
• Finger agnosia is the inability to identify the fingers on the hand
• Acalculia or dyscalculia refers to the lack of understanding in the application for the rules of calculation or arithmetic
• Inability to distinguish the right portion from the left portion

Adult patients may also manifest aphasia which is the difficulty with speech expression or even in understanding speech, or it can be the difficulty involving reading and writing.

Children with GS are often identified when they are in school and having the challenge to apply their writing skills and to utilize the use of numbers.  Aside from the existing four primary symptoms in them, they may also present signs of constructional apraxia which refers to the difficulty to copy simple drawings. Most of the time, they have dyslexia or a problem involving reading.

Causes

GS among adult individuals may happen due to a cerebrovascular disease that impairs the flow of blood going to the brain, such as in cases of a stroke or other damages involving the brain. The parietal lobes of the brain are known to be affected in GS; thus ending up to affect the perception and sensation, and the understanding of sensory input by the patient.

The existence of a brain tumor or any occurrence of a traumatic brain injury that impairs the functions of the parietal lobes of the brain, can also lead to the manifestations of the clinical symptoms of Gertsmann syndrome in some rare cases. The cause of GS among pediatric patients is usually unknown, though in some instances, it may be a result of the presence of a brain damage. Children who do not have any brain damage problem can also possibly have the GS.

Diagnosis

The diagnosis of Gerstmann syndrome is being confirmed by the presence of all the four neurological symptoms among adult patients, making sure to rule out of other possible causes of the stated specific signs. The common differential diagnosis is done between cases of cerebrovascular events versus cases of dementia.

Children are said to have the GS problem after encountering some difficulties of mathematical applications and in writing while being in school. They may also have a spelling difficulty, problems to use the basic four mathematical calculations in some activities in class, and the disorientation to identify the left side from the right side. Constructional apraxia might also be observed in many children who have GS.

Some physicians might recommend an MRI (magnetic resonance imaging) scan which might present a result of a lesion found at the angular gyrus of the brain’s left parietal lobe.

Treatment

GS problem has no specific cure. Its treatment is supportive based on patient manifestations.

• Occupational and speech therapy for apraxia and dysgraphia
• Word processors and calculators for children with school difficulties related to learning
• Training exercises to facilitate mathematical skills of children

Prognosis

GS symptoms may lessen among adults as time pass by; also the same with children, though they can adapt with life. An early diagnosis and treatment of GS problem offers better outcomes among patients.

References:

1. Gerstmann syndrome – Description, Presentation, Differential diagnosis, Investigations, Associated diseases, Management, Prognosis, References at http://patient.info/doctor/Gerstmann’s-Syndrome.htm
2. gerstmann syndrome Standard Therapies, Investigational Therapies at https://rarediseases.org/rare-diseases/gerstmann-syndrome/
3. http://www.rightdiagnosis.com/g/gerstmanns_syndrome/intro.htm#excerpts
4. Reeve R, Humberstone J (2011). “Five- to 7-year-olds’ finger gnosia and calculation abilities”. Front Psychol 2: 359.
5. Vallar G (July 2007). “Spatial neglect, Balint-Homes’ and Gerstmann’s syndrome, and other spatial disorders”. CNS Spectr 12 (7): 527–36.
6. Miller CJ, Hynd GW (April 2004). “What ever happened to developmental Gerstmann’s syndrome? Links to other pediatric, genetic, and neurodevelopmental syndromes”. J. Child Neurol. 19 (4): 282–9.