Historical Neurology

historical information Gersmann's s

2010-01-30T14:00:00.001-05:00

Pearce, J. M. S.Gerstmann's syndrome. Journal of Neurology, Neurosurgery & Psychiatry ≫ 61(1) July 1996

Author Information
304 Beverley Road, Anlaby Hull HU10 7BG, UK.
In the 19th century, there flourished the concept of localising organic function to discrete areas or "centres" in the brain. It was extended to attempts to provide accurate localisation for the more problematical higher cortical and psychological dysfunctions. Of many such syndromes described at the turn of the 20th century, the syndrome of Gerstmann is a good and oft cited example:

"The subject of this paper ... I first described several years ago (1924) [1] under the name of `finger agnosia'. It manifests itself as an isolated disturbance in the recognition, naming, choosing, and differential exhibition of the various fingers of both hands--one's own fingers as well as those of another person.... Furthermore, I will discuss the association that I noted between this symptom and a disturbance in right-left orientation (in one's own as well as in another's body), agraphia and acalculia.

...in some it existed as an independent condition from the beginning, while in others--though seldom--it was a residual syndrome after regression of a more complex deficit.

... In its selective form ... aphasia, apraxia, agnosia have been lacking. Furthermore, the other signs that have sometimes been associated with the syndrome (such as right hemianopsia, diminution of opticokinetic nystagmus, amnestic disturbance of word-finding, impairment in reading ability,...) can be characterised as neighbouring or bordering symptoms because of their variable appearance and mildness. The phenomenon of finger agnosia itself always appeared as an essential disturbance of recognition and orientation.

... It has become evident that the syndrome of finger agnosia, agraphia etc can be related to a focal disturbance in the area of transition between the angular and second occipital convolution.... the syndrome ... is caused by a unilateral lesion in the left hemisphere in right-handed individuals." [2] Gerstmann referred to corroborative cases of Potzl and Herrmann, Schilder, Kroll, and Lange; the syndrome found acceptance in the contemporary corpus of neurological teaching, but was later seriously challenged.

The entity proclaimed by Gerstmann has been seriously arraigned, notably by Critchley in a classic and critical review. [3] He demurred at the idea of an autonomous, independent syndrome, and at its alleged localising value. Benton too, harshly but probably justly, regarded it: "an artifact of defective and biased observation having little support for its alleged focal diagnostic significance." [4] Further, the description was not original: Anton (1899) and Hartmann (1902) described similar cases (though with bilateral pathology and therefore not strictly comparable). Critchley assigned priority to Jules Badal, ophthalmologist in Bordeaux, who in 1888 accurately reported the essential features in a patient named Valerie, suffering the posteclamptic state. [5]

Josef Gerstmann (1887-1969) of Vienna was a pupil of the Nobel prize winner Wagner von Jauregg; he became professor of neurology and psychiatry and director at the Maria-Thereisen-Schlossel. He fled the oncoming Nazi regime in 1938, but continued his work in the United States and wrote several papers on the variation of symptoms, pathology, and the localising value of his syndrome.

J M S PEARCE

304 Beverley Road, Anlaby

Hull HU10 7BG, UK

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REFERENCES
1. Gerstmann J. Fingeragnosie: Eine umschriebene Storung der Orientierung am eigenen Korper. Wien Klin Wschr 1924;37:1010-2. [Context Link]

2. Gerstmann J. Zur Symptomatologie der Hirnlasionen im Ubergangsgebiet der unteren Parietal--und mittleren Occipitalwindung. (Das Syndrom: Fingeragnosie, Rechts-Links-Storung, Agraphie, Akalkulie). Nervenarzt 1930;3:691-5. [Context Link]

3. Critchley M. The enigma of Gerstmann's syndrome. Brain 1966;89:183-98. [Context Link]

4. Benton AL. The fiction of Gerstmann's syndrome. J Neurol Neurosurg Psychiatry 1961;24:176-9. [Context Link]

5. Badal J. Arch Ophthal (Paris) 1888, 8:97 cited by Critchley. *RF 3* [Context Link]

Historical signs of neurologic syphilis

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1. Abadie's sign-- loss of pain with squeezing Achilles tendon, presumptive sign of tabes
2. Argyll-Robertson pupil-- occurs not just in syphilis but occassionally in alcoholism and epidemic encephalitis and includes the following features a. loss of light reflex b. retained accomodation reflex c. miosis present d. loss of ciliospinal reflex e. imperfect dilatation of eye with atropine
3. Biernacki's sign of tabes-- loss of pain with pressure of ulnar nerve behind albow
4. Westphal sign-- abolished patellar knee jerk, characteristic of tabes but not pathognomonic

Classic signs of tetany

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1. Chvostek's facial sign-- tapping over parotid causes contraction of homolateral facial muscle
2. Trousseau's sign-- spasm of hand and forearm (accoucheur's hand) by compressing brachial artery for one to five minutes
3. Erb's sign of tetany-- hyperexcitable peripheral motor nerve to galvanic current with a current less than 5 mamperes
4. Hoffmann's sign of tetany-- hyperexcitability or tetanic muscular spasms due to mechanical or electrical stimulation of a sensory nerve, usually the ulnar nerve
5. Kashida's thermic sign of tetany-- hyperesthesia and spasms after applying hot and cold irritants
6. Pool's arm and leg signs of tetany-- a. tension on the brachial plexus through forcible abduction of the arm resulting in spasms of muscles of hand and arms b. tension on sciatica by forcible flexion of thigh on trunk with the leg extended causes spasms of the muscles of the leg and foot.
7. Schlesinger's lef sign of tetany-- If the hip is flexed and the knee extended for one to three minutes, spasms occur in the extremity.
8. Schultze's tongue dimpling sign of tetany-- Tapping the protruded tongue causes dimpling at the site of contact.

Classical abnormal gaits

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Tabetic (ataxic) gait

Hemiplegic gait
Steppage gait
Scissors gait
Drunken or ataxic gait
Waddling or clumsy gait
Festinating gait
Hysterical gait
astasia - abasia

Babinski-like signs

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Extensor toe signs

Babinski response- extension of big toe, fanning of small toes with stroking plantar surface
Chaddock response- same response except stimulation is of lateral foot from lateral malleolus
Gordon leg sign-- same with stimulation of squeezing leg muscle
Oppenheim's sign-- same result with downward stroking of tibia and tibialis anterior muscle
Gonda's reflex--upward movement of big toe by moving another toe down and releasing with snap
Shaefer's sign-- Babinski response by squeezing Achilles' reflex

Non extensor toe long tract signs
Rossolimo's sign-- same response with striking or tapping the ball of the foot
Mendel-Bechterew sign- flexion of 4 outer toes by striking dorsum of foot
Hoffman's sign-- Clawing of the thumb and all fingers by flicking distal phalanx of index finger
Gordon's finger sign-- flexion of fingers or thumb/index finger with pressure over pisiform bone
Chaddock's wrist sign- wrist flexion, extension & fanning of fingers with stimulation of ulnar side of hand
Hirschberg's sign- adduction and internal rotation while stroking the inner border of foot
Ankle clonus-- may be normal if unsustained
Patellar clonus (trepidation sign)-- pushing knee suddenly looking for clonus

Other pathologic signs
Beevor's sign-- with lesion at T10, the patient tenses abdominal muscles and ombilicus moves upwa rds, doe to paralyzed
Mass reflex of Riddoch-sudden emptying of bowel and bladder, flexion of lower limbs and sweating. Its released pathologically by severing spinal cord and striking skin below

Quotes and current spectrum of PSP

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"The postmortem room has become the temple of truth" (Donald Calne, re PSP) (Neurology 2008)

Current nomenclature

"Richardson disease" corresponds with initial descriptions. There is a gradual onset of postural instability and falls within the first two years, with vertical supranuclear gaze palsy, a frontal dysexecutive syndrome, rigidity and bradykinesia that is not responsive to leveodopa, and a life expectancy of six years.

A second milder group at pm (post-mortem) have PSP tau pathology that is more restricted and less severe are called PSP-P (PSP-parkinsonism). They have assymmetric bradykinesia of the limbs, an initial response to levodopa, tremor and limb dystonia without early falls, eye movement problems, or cognitive dysfunction. Most patients with "atypical PSP" are in this category.

The third rarer category is pure akinesia with gait freezing (PAGF). There is gradual onset of unsteady or slow gait and hypophonia progressing to gair freezing and start hesitation, without limb rigidity or tremor. There is no response to levodopa and there is no dementia or opthalmoplegia in the first five years. In types 2 and 3 the median duration of the disease is around ten years.

Other patients with similar tau-PSP pathology present with corticobasal ganglionic degeneration, progressive nonfluent aphasia, or apraxia of speech.

References up to date:
Williams DR, de Silva R, Pavour DC et al. Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson' syndrome and PSP - parkinsonism. Brain 2005; 128:1247-1258.

Williams DR, Holton JL, Strand C. et al. Pathological tau burden and distribution distinguishes progressive supranuclear palsy-parkinsonism from Richardson's syndrome. Brain 2007; 130: 1566-1576.

Mizusawa H, Mochizuki A , Ohkoshi N, et al. Progressive supranuclear palsy presenting with pure akinesia. Adv Neurol 1993; 60: 618-621.

Josephs KA, Duffy JR, Strand EA et al. Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain 2006; 129: April 13.

Tsuboi Y, Josephs KA, Boeve BF et al. Increased tau burden in the cortices of progressive supranuclear palsy presenting with corticobasal syndrome. Mov Disord 2005; 20: 982-988.

Localization debate

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Thomas Willis-- sensation occurred in the corpus striatum and perceptual integration in the corpus callosum and memory in the cortex

La Peyronie-- corpus callosum was the seat of intellect

Hughlings Jacksonb (1878) "to locate the lesion which destroyes speech and to locate speech are two different things."

Lashley (1950) -- fruitless "search for the engram"

Obsolete terms in neuropsychology

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Neuropsychology is a field that "began" after World War II. Activities that preceded that time that would today be called neuropsychology were then caught by the terms "Psychoneurology" (Bekhterev) or brain pathology (Kleist). Until the 1820's, the gyri of the cortex were called "enteroid processes. " "Asymbolia" was proposed by Finkelnburg as an explanation for aphasia that was antilocalizationist.

Jack Ruby

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Gutmann L, Neurology 2007; 68:707-708.
Frank Forster diagnosed Ruby with TLE. Ruby's EEG showed short burst of 6/sec activity from the the temporal lobes. Frederick Gibbs testified for the defense that Ruby had psychomotor epilepsy. Marvin Belli made the case that Ruby had seizures and could not tell right from wrong. Guttmacher, from Johns Hopkins testified Ruby was in a "fugue" state when he shot Oswald. The jury convicted Ruby anyway and sentenced him to death, the conviction was overturned, and Ruby died waiting for a second trial. "Psychomotor variant" is now called rhythmic temporal theta bursts of drowsiness and is not considered epileptic.

US neurology: Hoover sign

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Hoover was an Ohio internist, graduate of Harvard medical school, student of Pierre Marie.

See Hoover CF. A new sign for detection of malingering and functional paresis of the lower extremities. JAMA. 1908; 51:746.

Hoover observed that normal, lifting one leg, will dig the contralateral heel into the couch with equal force. Conversely, if asked to move one leg downward, the other leg will elevate. The Hoover sign was present if the alleged paralyzed leg dug into the couch when lifting the healthy leg. He felt this was superior to the Babinski sign. It was the only maneuver to differentiate hysterical and organic paralysis without relying on the affected limb.

US physician: Chaddock

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from Ann Arbor, later St Louis U, visited Babinski in Europe.

Chaddock sign-- extension of one or more, or all oo the toes,with or without fanning, when the external inframalleolar skin is irritated in cases of organicdisease of the spinocortical reflex paths. C called it "external malleolar sign."

Advantage was absence of reflex withdrawal in cases.

This sign was simultaneous described by the Japanese neurologist Kisaku Yoshimura in Hiroshima Hospital in 1906.

The reverse Chaddock sign (reversing the direction of the stimulus) is also sensitive. Chaddock sign is considered more sensitive than Babinski sign.

Upper limb corollary-- scratching the skin at the ulnar side over the tendons of the long palmar and radial carpal flexor muscles caused a short lasting contraction of the hypothenar muscles. He called this the "wrist sign."

Signs of organic hemiplegia nearly a century old

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Raimiste sign-- during the period of flaccidity, the elbow is placed vertically on the table and rapidly flexes and pronates. (1809)

Souques interossei phenomena (1909) When the patient raises the affected arm, the fingers extend and abduct.

Klippel Weil sign 1909- involuntary flexion of the thumb with passive straightening of the flexed fingers.

Strumpell tibialis anticus phenomenon-- voluntary flexion of the limb causes dorsiflexion and adduction of the foot.

Rainiste associated adductionand abduction (1909)-- When the patient lies on back and adducts or abducts the unaffected leg, the affected leg mirrors the movement

Marie-Foix associated movements-- (1916)-- general synkinesis-- contraction of all the muscles on the affected side with effort; imitation synkinesis-- involuntary movements on affected side reproduce voluntary movements on unaffected side; coordination synkinesis-- voluntary contraction of certain muscle groups on affected side give rise to involuntary contractions of functionally synergistic muscles

Neri sign (1907) flexion of knee accompanies flexion of trunk on affected side

Dorsal reflex of Mendel-Bekhterev-- (1904)-- percussion of lateal dorsal cuboid produces flexion of the toes

Reflex hyperkinesia of Claude- painful pricking, sticking, pinching or pressure causes reflex movements of paralyzed upper limb

Raichline. Marie and Meige adduction reflex of the foot-- stimulation of the skin of the foot on inside causes adduction.

Pithiatism (instead of hysterical paralysis)

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means persuasion and curable, Babinski's term, from Greek, never was accepted. Signs of, according to Babinski, included: paralysis not always unilateral; unconscious voluntary movements unaffected (associated movements normal); no exaggerated flexion of forearm; no reflex changes, cutaneous reflexes intact; the form of contracture can be reproduced voluntarily; capricious disease course, relapses and remissions.

In organic disease, by contrast, paralysis is unilateral, platysma sign is present with bending head or opening mouth; tongue slightly deviated; lack nasolabial fold; at onset, absent reflexes, later clonus;loss cutaneous reflexes (abdominal/cremasteric)with Babinski sign (extensor toe with fanning of other toes); contracture non reproducible voluntarily, increases with passive extension; claw hand; regular disease course.

Babinski disagreed with others who felt there were special signs present in hysteria.

The Babinski Trunk-thigh test

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IBID. 1897. Babinski noted that a recumbent patient with organic paralysis of a leg was asked to sit up with his arms crossed across his chest, the weak leg would involuntarily flex due to absent gluteal maximus extension, and in paraplegia, both llgs would flex. In normals, extension of the leg would allow flexion of the trunk (the opposite). Only the healthy leg would flex in hysterical hemiparesis and often neither leg would flex. Also, retention of some movement indicated organic disease.

Babinski, Chaddock and Hoover signs

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citation paid or by subscription

Okun MS , Koehler, PJ. Babinski's clinical differentiation of organic paralysis from hysterical paralysis. Effect on US neurology. Arch Neurol 2004; 61:778-783.

Joseph Babinski,a student of Charcot, moved from the Salpetriere Hospital to the Pitie Hospital. He had several signs.

The trunk -thigh test