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Synonyms: developmental Gerstmann's 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. Possibly both superior and inferior lobes need to be affected. The specific effect of lesions of various lobes is discussed in the separate Space-occupying Lesions of the Brain article. However, in Gerstmann's syndrome in particular, the result is characterised by four components:
- Agraphia or dysgraphia
- Acalculia or dyscalculia
- Finger agnosia
- Left-right disorientation
Pure Gerstmann's 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. It does not seem to have a genetic component and is not listed in Online Mendelian Inheritance in Man (OMIM). It has been argued that developmental Gerstmann's syndrome is not a unique entity but a feature of other neurodevelopmental disorders. This has also been disputed.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 arithmetical 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).
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 have 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
- 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 requests 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 requests 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.
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
- Carbon monoxide poisoning
- Lead poisoning
- Anaphylactic shock
- Systemic lupus erythematosis
There is no cure for Gerstmann's syndrome. Treatment is supportive:
- Dysgraphia and apraxia can be helped by occupational and speech therapy. Calculators and word processors can also help school children cope with their disabilities.
- There has been a recognition of the association between finger recognition and numerical ability and there has been promising work done where improving finger gnosis through training exercises has led to improvements in the mathematical skills of young children.
Symptoms may lessen in adults over time. This is also thought to occur in children but it is more likely that they adapt to life with them. It has been suggested that early diagnosis and intensive treatment give a better outcome.
Further reading and references
Ardila A; A proposed reinterpretation of Gerstmann's syndrome. Arch Clin Neuropsychol. 2014 Dec29(8):828-33. doi: 10.1093/arclin/acu056. Epub 2014 Nov 5.
Rusconi E, Pinel P, Dehaene S, et al; The enigma of Gerstmann's syndrome revisited: a telling tale of the vicissitudes of neuropsychology. Brain. 2010 Feb133(Pt 2):320-32. doi: 10.1093/brain/awp281. Epub 2009 Nov 10.
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 Oct99(4):716-27.
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 Dec103(6):1010-7.
Miller CJ, Hynd GW; What ever happened to developmental Gerstmann's syndrome? Links to other pediatric, genetic, and neurodevelopmental syndromes. J Child Neurol. 2004 Apr19(4):282-9.
Suresh PA, Sebastian S; Developmental Gerstmann's syndrome: a distinct clinical entity of learning disabilities. Pediatr Neurol. 2000 Apr22(4):267-78.
Gerstmann's Syndrome; National Institute of Neurological Disorders and Stroke
Nagaratnam N, Phan TA, Barnett C, et al; Angular gyrus syndrome mimicking depressive pseudodementia. J Psychiatry Neurosci. 2002 Sep27(5):364-8.
Jung RE, Yeo RA, Sibbitt WL Jr, et al; Gerstmann syndrome in systemic lupus erythematosus: neuropsychological, neuroimaging and spectroscopic findings. Neurocase. 20017(6):515-21.
Gracia-Bafalluy M, Noel MP; Does finger training increase young children's numerical performance? Cortex. 2008 Apr44(4):368-75. Epub 2008 Jan 8.
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