Experience | News | Products | Other
This is a PatientPlus article. PatientPlus articles are written for doctors and so the language can be technical. However, some people find that they add depth to the articles found in the other sections of this website which are written for non-medical people.
Gerstmann's Syndrome
Synonyms: Developmental Gerstmann syndrome (when it occurs in children), Gerstmann tetrad
The condition should not be confused with Gerstmann-Sträussler syndrome or Gerstmann-Sträussler-Scheinker syndrome - a transmissible spongiform encephalopathy.
This is a condition arising as a result of disease of the dominant parietal lobe at the angular gyrus.1 Possibly both superior and inferior lobes need to be affected.2 The specific effect of lesions of various lobes is discussed in the article on Space Occupying Lesions but in Gerstmann's syndrome in particular, the result is characterised by 4 components:
- Agraphia or dysgraphia
- Acalculia or dyscalculia
- Finger agnosia
- Left-right disorientation
Pure Gerstmann syndrome is said to be without aphasia. It generally presents as either a congenital or learning disorder or as a feature of a stroke of the middle cerebral artery. Both forms are rare, especially the childhood form. It can also be a feature of neurodegenerative diseases such as Alzheimer's disease or as a result of head injury.
The childhood type may occur in those with brain damage or in isolation with otherwise good mental function; the pathophysiology in children is not understood.3 It does not seem to have a genetic component and is not listed in OMIM. It has been argued that developmental Gerstmann's syndrome is not a unique entity but a feature of other neurodevelopmental disorders.4 This has also been disputed.5 In adults, the risk factors appear to be the same as those for a cerebrovascular event.
There is loss or absence of four sensory abilities.
- Loss of the ability to express thoughts in writing (agraphia, dysgraphia)
- Inability to perform simple arithmetic calculations (acalculia)
- Inability to recognise or indicate one's own or another's fingers (finger agnosia)
- Inability to distinguish between right and left
In addition, many adults also experience aphasia, (difficulty in expression with speech, in understanding speech or in reading and writing).
- The speech area is in the dominant hemisphere that is on the left in over 95% of right handed people. It is also on the left in 75% of left handed people but in the other 25% it appears to be bilateral.
Most paediatric cases are identified when children start school and they are challenged with writing and numbers. Generally, children with the disorder exhibit poor handwriting and spelling and difficulty with mathematical functions (adding, subtracting, multiplying and dividing). An inability to differentiate right from left and to discriminate among individual fingers may also be apparent. In addition to the four primary symptoms, many children also suffer from constructional apraxia, an inability to copy simple drawings. Frequently, there is also impairment in reading (dyslexia). Children with good intellectual function as well as those with brain damage may be affected.
Eliciting features of Gerstmann's syndrome
- Agraphia
- Illegible or very poor writing
- Inconsistencies in forming letters
- Mixture of upper and lower case letters or print and cursory writing
- Irregular letter sizes and shapes
- Unfinished letters
- Acalculia
This is tested by asking the patient to do serial subtraction of 7 from 100. This means 100, 93, 86, 79, 72, etc. It must be interpreted in the light of the educational level of the patient including the age of a child. An easier test may be applicable, especially for children. - Finger agnosia
Finger agnosia is difficulty in distinguishing fingers on the hand. It is tested by asking questions like, "Touch my index finger with your index finger" and "Touch your nose with your little finger." - Left-right disorientation
This is confusion of the right and left limbs and indicates a lesion in the dominant parietal lobe. It is tested by questions like, "Show me your left hand. Touch your right foot" and "Touch your left ear with your right hand." A positive test is the inability to obey these commands in the presence of otherwise normal sensory and motor function.
In adults, differential diagnosis is that of stroke and includes dementia.6 In children, it is with more global brain damage and learning difficulties.
MRI scan will usually show a lesion of the angular gyrus in the left parietal lobe.
As well as occurring in strokes, head injuries and developmental disorders, the syndrome has been associated with cerebral atrophy, alcoholism, carbon monoxide poisoning, lead poisoning, anaphylactic shock and systemic lupus erythematosis.7
There is no cure for Gerstmann's syndrome. Treatment is symptomatic and supportive. Occupational and speech therapies may help diminish the dysgraphia and apraxia. There has been a recognition of the association between finger recognition and numerosity and recently, there has been promising work done where improving finger gnosis through training exercises has led to improvements in the mathematical skills of young children.9 Calculators and word processors may also help school children cope with the symptoms of the disorder.
In adults, many of the symptoms diminish with time. Although it has been suggested that in children symptoms may diminish over time, it appears likely that most children probably do not overcome their deficits but learn to adjust to them. It has been suggested that early diagnosis and intensive treatment gives better outcome.4
Document references
- Roux FE, Boetto S, Sacko O, et al; Writing, calculating, and finger recognition in the region of the angular gyrus: a cortical stimulation study of Gerstmann syndrome. J Neurosurg. 2003 Oct;99(4):716-27. [abstract]
- Russell SM, Elliott R, Forshaw D, et al; Resection of parietal lobe gliomas: incidence and evolution of neurological deficits in 28 consecutive patients correlated to the location and morphological characteristics of the tumor. J Neurosurg. 2005 Dec;103(6):1010-7. [abstract]
- Critchley M. The enigma of Gerstmann?s syndrome; Brain. 1966 Jun;89(2):183-98
- Miller CJ, Hynd GW; What ever happened to developmental Gerstmann's syndrome? Links to other pediatric, genetic, and neurodevelopmental syndromes. J Child Neurol. 2004 Apr;19(4):282-9. [abstract]
- Suresh PA, Sebastian S; Developmental Gerstmann's syndrome: a distinct clinical entity of learning disabilities. Pediatr Neurol. 2000 Apr;22(4):267-78. [abstract]
- Nagaratnam N, Phan TA, Barnett C, et al; Angular gyrus syndrome mimicking depressive pseudodementia. J Psychiatry Neurosci. 2002 Sep;27(5):364-8. [abstract]
- Jung RE, Yeo RA, Sibbitt WL Jr, et al; Gerstmann syndrome in systemic lupus erythematosus: neuropsychological, neuroimaging and spectroscopic findings. Neurocase. 2001;7(6):515-21. [abstract]
- NINDS; Gerstmann syndrome. American National Institute of Neurological Disorders and Stroke.
- Gracia-Bafalluy M, Noel MP; Does finger training increase young children's numerical performance? Cortex. 2008 Apr;44(4):368-75. Epub 2008 Jan 8. [abstract]
Internet and further reading
- www.whonamedit.com; Gerstmann's Syndrome
DocID: 2191
Document Version: 21
DocRef: bgp1178
Last Updated: 17 Jan 2009
Review Date: 17 Jan 2011
The authors and editors of this article are employed to create accurate and up to date content reflecting reliable research evidence, guidance and best clinical practice. They are free from any commercial conflicts of interest.
Find out more about updating.
Related pages in Patient UK
Your Experience (^ top of page)
Please add your experience about this condition / medicinePatient UK Newspaper (^ top of page)
Latest Health News
View current health newsRelated Products (^ top of page)
Medical equipment

Books

Other - Useful resources (^ top of page)
Pictures, diagrams, photos, images, etc.Evidence based medicine
Online textbooks and journals
UK Guidelines
Online Videos
Medline
Other good health sites
Disclaimer: Patient UK has no control over the content of any external links above. Inclusion does not imply endorsement by Patient UK.
Want to advertise on this site? Find out how >>
Here you can follow a link to view existing patient experiences on this subject, or to add your own
This will offer you the usual PDF options i.e. document navigation, search, zoom and formatted print
Note: this is the best way to print the document
Note: this will open in a new window
Note: this will open in a new window






