Gerstmann syndrome

Neuropsychological disorder caused by damage to the inferior parietal lobule Not to be confused with Gerstmann–Sträussler–Scheinker syndrome, which is a transmissible spongiform encephalopathy. Medical condition

Gerstmann syndrome

The inferior parietal lobule, with damage in this area most often associated with Gerstmann syndrome
Specialty	Neurology, neuropsychology
Symptoms	Dysgraphia, dyscalculia, finger agnosia, left-right disorientation, constructional apraxia, aphasia
Causes	Idiopathic, stroke, dementia

Gerstmann syndrome is a neurological disorder that is characterized by a constellation of symptoms that suggests the presence of a lesion usually near the junction of the temporal and parietal lobes at or near the angular gyrus. Gerstmann syndrome is typically associated with damage to the inferior parietal lobule of the dominant hemisphere. It is classically considered a left-hemisphere disorder, although right-hemisphere damage has also been associated with components of the syndrome.

It is named after Jewish Austrian-born American neurologist Josef Gerstmann.

Symptoms

Gerstmann syndrome is characterized by four primary symptoms, collectively referred to as a tetrad:

Dysgraphia/agraphia: deficiency in the ability to write
Dyscalculia/acalculia: difficulty in learning or comprehending mathematics
Finger agnosia: inability to distinguish the fingers on the hand
Left-right disorientation

Causes

This disorder is often associated with brain lesions in the dominant (usually left) hemisphere including the angular and supramarginal gyri (Brodmann area 39 and 40 respectively) near the temporal and parietal lobe junction. There is significant debate in the scientific literature as to whether Gerstmann syndrome truly represents a unified, theoretically motivated syndrome. Thus its diagnostic utility has been questioned by neurologists and neuropsychologists alike. The angular gyrus is generally involved in translating visual patterns of letters and words into meaningful information, such as is done while reading.

In adults

In adults, the syndrome may occur after a stroke. In addition to exhibiting the above symptoms, many adults also experience dysphasia or aphasia, which is difficulty in expressing oneself when speaking, in understanding speech, or in reading and writing.

In children

There are few reports of the syndrome, sometimes called developmental Gerstmann syndrome, in children. The cause is not known. Most cases are identified when children reach school age, a time when they are challenged with writing and mathematic exercises. Generally, children with the disorder exhibit poor handwriting and spelling skills, and difficulty with math functions, including 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 an impairment in reading. Children at any level of intelligence may be affected with the disorder.

Diagnosis

Diagnosis may be clinical if associated with dementia and other etiologies. In cases caused by stroke, MRI will show a corresponding stroke in the inferior parietal lobule. In the acute stage, this will be bright (restricted diffusion) on the DWI sequence and dark at the corresponding area on the ADC sequence.

Treatment

There is no cure for Gerstmann syndrome. Treatment is symptomatic and supportive. Occupational and speech therapies may help diminish the dysgraphia and apraxia. In addition, calculators and word processors may help school children cope with the symptoms of the disorder.

Prognosis

In adults, many of the symptoms diminish over time. Although it has been suggested that a similar diminishing of symptoms occurs in children as well, it appears more likely that most do not overcome their deficits, but instead simply learn to adjust.

References

João RB, Filgueiras RM, Mussi ML, de Barros JE (June 2017). "Transient Gerstmann syndrome as manifestation of stroke: Case report and brief literature review". Dementia & Neuropsychologia. 11 (2): 202–205. doi:10.1590/1980-57642016dn11-020013. PMC 5710689. PMID 29213512.
^ Heimburger RF, Demyer W, Reitan RM (February 1964). "Implications of Gerstmann's syndrome". Journal of Neurology, Neurosurgery, and Psychiatry. 27 (1): 52–57. doi:10.1136/jnnp.27.1.52. PMC 495679. PMID 14123925.
synd/2267 at Who Named It?
^ Vallar G (July 2007). "Spatial neglect, Balint-Homes' and Gerstmann's syndrome, and other spatial disorders". CNS Spectrums. 12 (7): 527–536. doi:10.1017/S1092852900021271. PMID 17603404. S2CID 45201083.
^ Carota A, Di Pietro M, Ptak R, Poglia D, Schnider A (2004). "Defective spatial imagery with pure Gerstmann's syndrome". European Neurology. 52 (1): 1–6. doi:10.1159/000079251. PMID 15218337. S2CID 22897847.
^ Miller CJ, Hynd GW (April 2004). "What ever happened to developmental Gerstmann's syndrome? Links to other pediatric, genetic, and neurodevelopmental syndromes". Journal of Child Neurology. 19 (4): 282–289. doi:10.1177/088307380401900408. PMID 15163095. S2CID 26407185.
^ Office of Communications and Public Liaison. "Gerstmann's Syndrome Information". U.S. National Institute of Neurological Disorders and Stroke (NINDS). Bethesda, MD. Archived from the original on 2012-06-24. Retrieved 2012-06-27.

External links

Classification	D ICD-10: F81.2 ICD-9-CM: 784.69 MeSH: D005862 DiseasesDB: 30728
External resources	Orphanet: 221117

Mental disorders (Classification)

Adult personality and behavior

Sexual	Ego-dystonic sexual orientation Paraphilia Fetishism Voyeurism Sexual anhedonia Sexual anorexia Sexual maturation disorder Sexual relationship disorder
Other	Factitious disorder Munchausen syndrome Fear of intimacy Gender dysphoria Intermittent explosive disorder Dermatillomania Kleptomania Pyromania Trichotillomania Personality disorder

Childhood and learning

Emotional and behavioral	ADHD Conduct disorder ODD Emotional and behavioral disorders Separation anxiety disorder Movement disorders Stereotypic Social functioning DAD RAD Selective mutism Speech Cluttering Stuttering Tic disorder Tourette syndrome
Intellectual disability	X-linked intellectual disability Lujan–Fryns syndrome
Psychological development (developmental disabilities)	Pervasive Specific

Mood (affective)
Bipolar Bipolar I Bipolar II Bipolar NOS Cyclothymia Depression Atypical depression Dysthymia Major depressive disorder Melancholic depression Seasonal affective disorder Mania

Neurological and symptomatic

Autism spectrum	Autism Asperger syndrome High-functioning autism PDD-NOS Savant syndrome
Dementia	AIDS dementia complex Alzheimer's disease Creutzfeldt–Jakob disease Frontotemporal dementia Huntington's disease Mild cognitive impairment Parkinson's disease Pick's disease Sundowning Vascular dementia Wandering
Other	Delirium Organic brain syndrome Post-concussion syndrome

Neurotic, stress-related and somatoform

Adjustment

Adjustment disorder with depressed mood

Anxiety

Phobia	Agoraphobia Childhood phobia Social anxiety Social phobia Anthropophobia Specific social phobia Specific phobia Claustrophobia
Other	Generalized anxiety disorder OCD Panic attack Panic disorder Stress Acute stress reaction PTSD

Dissociative

Somatic symptom

Physiological and physical behavior

Eating	Anorexia nervosa Bulimia nervosa Rumination syndrome Other specified feeding or eating disorder
Nonorganic sleep	Hypersomnia Insomnia Parasomnia Night terror Nightmare REM sleep behavior disorder
Postnatal	Postpartum depression Postpartum psychosis
Sexual desire	Hypersexuality Hypoactive sexual desire disorder

Psychoactive substances, substance abuse and substance-related
Drug overdose Intoxication Physical dependence Rebound effect Stimulant psychosis Substance dependence Withdrawal

Schizophrenia, schizotypal and delusional

Delusional	Delusional disorder Folie à deux
Psychosis and schizophrenia-like	Brief reactive psychosis Schizoaffective disorder Schizophreniform disorder
Schizophrenia	Childhood schizophrenia Disorganized (hebephrenic) schizophrenia Pseudoneurotic schizophrenia Simple-type schizophrenia
Other	Catatonia

Symptoms and uncategorized
Impulse-control disorder Klüver–Bucy syndrome Psychomotor agitation Stereotypy

Signs and symptoms, and syndromes associated with lesions of the brain and brainstem

Cerebral cortex

ACA syndrome
MCA syndrome
PCA syndrome
Aphasia
Frontal lobe
- Expressive aphasia
- Abulia
Parietal lobe
Occipital lobe
Temporal lobe
- Cortical deafness
- Prosopagnosia

Subcortex

Basal ganglia
Thalamic syndrome

Cerebellum

Lateral
Medial
- Cerebellar ataxia

Brainstem

Medulla	Lateral medullary syndrome/Wallenberg PICA Medial medullary syndrome/Dejerine ASA
Pons	Upper dorsal pontine syndrome/Raymond–Céstan syndrome Lateral pontine syndrome (AICA) (lateral) Medial pontine syndrome/Millard–Gubler syndrome/Foville's syndrome (basilar) Locked-in syndrome Internuclear ophthalmoplegia One and a half syndrome
Midbrain	Weber's syndrome ventral peduncle, PCA Benedikt syndrome ventral tegmentum, PCA Parinaud's syndrome dorsal, tumor Claude's syndrome
Other	Alternating hemiplegia

Other

Conditions

Speech, language, and communication	Expressive language disorder Infantile speech Landau–Kleffner syndrome Language disorder Lisp Mixed receptive-expressive language disorder Specific language impairment Speech and language impairment Speech disorder Speech error Speech sound disorder Stuttering Tip of the tongue
Learning disability	Dyslexia Dyscalculia Dysgraphia Disorder of written expression
Motor	Developmental coordination disorder Developmental verbal dyspraxia
Sensory	Auditory processing disorder Sensory processing disorder

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