Gerstmann syndrome

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ORPHA:221117F81.2
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Overview

Gerstmann syndrome is a rare neurological condition characterized by a distinctive tetrad of cognitive deficits: finger agnosia (inability to recognize or distinguish one's own fingers), agraphia (difficulty with writing), acalculia (difficulty performing mathematical calculations), and left-right disorientation (inability to distinguish left from right). The syndrome results from damage to the dominant (usually left) parietal lobe, specifically the angular gyrus region. In adults, it is most commonly caused by stroke, brain tumors, or other acquired lesions affecting this brain area. A developmental form, known as developmental Gerstmann syndrome (also called Gerstmann tetrad or developmental dysgraphia with dyscalculia), occurs in children and is classified under ICD-10 as F81.2 (specific disorder of arithmetical skills). In the developmental form, children present with the same core tetrad of symptoms without an identifiable structural brain lesion. These children may also exhibit constructional apraxia and difficulty with reading. The developmental variant is thought to reflect atypical maturation of parietal lobe circuits rather than acquired damage. There is no specific cure for Gerstmann syndrome. Treatment is primarily supportive and symptomatic, focusing on educational interventions, occupational therapy, and cognitive rehabilitation tailored to the individual's specific deficits. In acquired cases, treatment of the underlying cause (such as stroke management or tumor removal) may lead to partial or full recovery. For the developmental form, specialized educational strategies addressing writing, arithmetic, and spatial orientation difficulties are the mainstay of management. Prognosis varies depending on the underlying etiology and the severity of symptoms.

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Gerstmann syndrome.

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No actively recruiting trials found for Gerstmann syndrome at this time.

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No specialists are currently listed for Gerstmann syndrome.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Gerstmann syndrome.

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Community

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Latest news about Gerstmann syndrome

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Common questions about Gerstmann syndrome

What is Gerstmann syndrome?

Gerstmann syndrome is a rare neurological condition characterized by a distinctive tetrad of cognitive deficits: finger agnosia (inability to recognize or distinguish one's own fingers), agraphia (difficulty with writing), acalculia (difficulty performing mathematical calculations), and left-right disorientation (inability to distinguish left from right). The syndrome results from damage to the dominant (usually left) parietal lobe, specifically the angular gyrus region. In adults, it is most commonly caused by stroke, brain tumors, or other acquired lesions affecting this brain area. A devel