Journal of , Neurosurgery, and 1997;63:399–403 399 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.63.3.399 on 1 September 1997. Downloaded from

SHORT REPORT

Toe in Gerstmann

Oliver Tucha, Anne Steup, Christian Smely, Klaus W Lange

Abstract debate concerning the The following case report presents a has been described elsewhere.10 14–16 We de- patient exhibiting Gerstmann syndrome scribe a patient who had a focal lesion in the accompanied by toe agnosia. A 72 year old of the left hemisphere which was right handed woman had a focal lesion in caused by a glioblastoma multiforme. The the angular gyrus of the left hemisphere patient exhibited Gerstmann syndrome ac- which was caused by a glioblastoma companied by toe agnosia. multiforme. The first symptom she had complained of was severe . Case report Standardised neuropsychological tests of A 72 year old right handed woman who had intelligence, memory, attention, fluency, had no history of neurological or psychiatric , and language functions as well as diseases was admitted to the Department of tests for the assessment of , acal- Neurosurgery for treatment of a glioblastoma culia, right-left disorientation, and digit multiforme in the left . The first agnosia were performed. The patient symptom she had complained of was severe displayed all four symptoms of the Gerst- headache. The patient had worked as an accountant until the age of 64.

mann syndrome—namely, agraphia, acal- copyright. culia, right-left disorientation, and finger A cranial CT scan disclosed a tumour agnosia. The patient did not display apha- situated in the areas P01, P02, and P05 sia, , or any other according to the classification of Damasio and neuropsychological impairment. In addi- Damasio and which included the angular tion to the four symptoms of the Gerst- gyrus, and extended to mann syndrome an agnosia of the toes was the of the left hemi- found. Further studies should determine sphere (figure).17 whether finger agnosia in Gerstmann syn- On neurological examination no deficits drome is usually accompanied by toe were found. On neuropsychological examin- agnosia. Finger agnosia in the context of ation, the patient was alert, cooperative and this syndrome may be better named digit well oriented. Her intellectual functions were agnosia. average (four subtests of the Wechsler adult http://jnnp.bmj.com/ intelligence scale18 ). She showed no deficits in (J Neurol Neurosurg Psychiatry 1997;63:399–403) biographical memory, everyday memory, short, medium and long-term memory or in working Keywords: Gerstmann syndrome; finger agnosia; toe memory for verbal material (subtests of the agnosia; digit agnosia Wechsler memory scale19). Her performance in a recognition task (recurring figures20)aswell as her short term memory and working

Department of In the 1930s Josef Gerstmann described a syn- memory for non-verbal material were also nor- on September 24, 2021 by guest. Protected drome consisting of four symptoms: finger mal (subtests of the Wechsler memory scale19 ). Oliver Tucha agnosia, right-left disorientation, agraphia and She had no diYculty in copying the complex Anne Steup 21 22 Klaus W Lange , which is attributed to dominant figure of Rey or in drawing a cube and clock hemisphere lesions aVecting the angular faces. Her motor speed and performance in a 1–3 Department of gyrus. Subsequently numerous case reports complex conceptual tracking task were normal Neurosurgery, based upon this description were published.2–9 (trail making test23), and she showed no bucco- University of Freiburg, The facts that not all observed cases with the facial apraxia or of the limbs Freiburg, Germany Gerstmann syndrome displayed all four com- 24 Christian Smely and body. In a detailed examination of ponents of the quadrima, that the patients language functions (Aachen test25), Correspondence to: showed other clinical manifestions—for exam- including spontaneous , token test, rep- Professor KW Lange, ple, constructive apraxia or aphasic etition of syllables, words and sentences, read- Institute of Psychology, disturbances—that the number of symptoms University of Freiburg, ing and words, words and sen- 79085 Freiburg, Germany seemed to depend on the size of the lesion and tences with cards on which letters and words that some patients with lesions in the left pos- were printed, naming objects, colours and situ- Received 8 January 1997 and terior parasylvian area were free of any of the ations as well as tests of comprehension, no in revised form 20 March 1997 Gerstmann symptoms raised doubts as to the deficits were found. Only her performance in Accepted 3 April 1997 existence of a distinct syndrome.5 9–13 The writing sentences was impaired: spelling mis- 400 Tucha,Steup, Smely, Lange J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.63.3.399 on 1 September 1997. Downloaded from

takes, especially omissions of single letters, numbers, to insert arithmetic operators (for were found. Her performance in the other example, +, −, ×, ÷) into simple calculations writing tasks was good; her overall performance and to do simple mental arithmetic as well as in writing was at a normal level. Her perform- written calculations. The patient was requested ance in a verbal (S-words) and figural fluency to add, subtract, multiply, and divide. (5-point-test26 ) task was normal, although she The patient had no diYculty in counting, in showed a raised rate of repetitions in the figural pointing to numbers called by the examiner or fluency task. In a computerised examination of in discriminating, grading, and copying num- handwriting movements which was performed bers. However, deficits in writing dictated with a digitising tablet (WACOM IV),27 28 no numbers, in , and in repeating numbers impairment was revealed. Her reaction time in were found. Furthermore, she displayed severe a computerised reaction task (TAP-alertness29) impairments in calculating. In writing dictated was reduced. On further neuropsychological numbers and in repeating numbers she had examination she displayed all four symptoms of diYculty with numbers which contained a zero the Gerstmann syndrome. All of these symp- and which had more than three digits—for toms could be seen throughout the entire example, when the examiner said 3011 she duration of her stay in hospital. repeated 3111. In reading numbers she made some mistakes concerning the first digit of the AGRAPHIA numbers—for example, she said 148 instead of The patient was asked to write down dictated 248. In reading as well as in repeating numbers letters and words, to name words which were she made the mistake several times of splitting spelt by the examiner either orally or with the numbers into thousands, hundreds, and lettered cards, to spell and read words, to write digits, eg. she said 9000-0-9 instead of 9009. In a short report of her relatives’ last visit, to recite arithmetic she made mistakes in more than the alphabet and to search for spelling mistakes 50% of the problems, even in simple calcula- in a short story. tions. The deficits aVected adding, subtracting, While the patient displayed no deficits in multiplying and dividing equally. In inserting reading or spelling, in correcting spelling arithmetic operators into calculations her mistakes, in reciting the alphabet, in writing performance was much better than in the other dictated letters, or in naming words which were arithmetical tasks. spelt by the examiner, frequent omissions, additions and substitutions could be seen when RIGHT-LEFT DISORIENTATION she was asked to write down dictated words, to In order to investigate a possible right-left copyright. copy words and to write a report. These disorientation, tests were chosen considering findings were supported by the results of the the reflections of Benton and Sivan.30 The examination of language functions. patient was asked to name the parts of her body which were touched by the examiner, to state ACALCULIA which side of her body was being touched, and The patient was asked to count to 25, to point to point to parts of her body as well as their side to numbers printed on cards (the number (for example, left leg). These requests were being determined by the examiner), to copy based on one-stage commands. Another set of and read numbers, to repeat numbers which two-stage commands was also used consisting were called by the examiner, to discriminate of crossed (for example, “touch your right ear and grade numbers, to write down dictated with your left hand”) and uncrossed com- http://jnnp.bmj.com/ on September 24, 2021 by guest. Protected

MRI T1W in transverse and coronal view showing a multigyral contrast enhancing tumour mass with perifocal oedema. Slight compression of ipsilateral lateral ventricle. Toe agnosia in Gerstmann syndrome 401 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.63.3.399 on 1 September 1997. Downloaded from

mands (for example, “touch your left knee with tigation comprised touching and naming toes your left hand”) which were given first by the on request by the examiner with and without examiner and subsequently by the patient her- sight and touching with closed eyes the toe of self. All of these tasks were performed with eyes one foot which corresponded to the toe of the open and closed. Furthermore, she was asked other foot that the examiner had just touched. to perform most of these tasks on a model who It was not possible to perform the tests sat opposite or next to her. described by Kinsbourne and Warrington on On one-stage commands the patient dis- the toes.13 playednodiYculty in distinguishing left from The patient had no problems in naming right referring to her own body. The same parts of the body and in grading strips of requests referring to a model’s body caused papers which diVered in size or which were problems regardless of the model’s spatial rela- labelled with the names of the fingers. How- tionship to the patient. These diYculties were ever, her performance in naming and touching not therefore related to problems with mental her own fingers, the fingers of the examiner or spatial rotation. In all tasks comprised of the model as well as her performance in the two-stage commands the patient demonstrated two-point finger test, in the in-between test and a right-left disorientation. She was, however, in the matchbox test was impaired. While she able to manage tasks that were based on com- made only few mistakes in tasks in which the mands with similar complexity without includ- requested fingers were shown on a model or ing right and left. No impairment of up-down named by the examiner she displayed severe orientation (for example, orientation corre- impairments in the tests without sight, espe- sponding to the vertical body axis) was cially when asked to touch, move, or name the revealed. fingers of one hand which corresponded to those of the other hand that had just been FINGER AGNOSIA touched by the examiner. When the finger had The patient was asked to grade strips of paper been touched on the same hand, less severe of diVering size, to name parts of her own and impairments could be observed. This result of the examiner’s body, to name or touch was due to a strategy of compensation in which fingers of her own, of the examiner and of a the patient held with her thumb the finger cardboard model as indicated by the examiner, which had been touched by the examiner until to move her own fingers on request by the she was asked to touch, move or name this fin- examiner, to touch the same fingers of the ger. To avoid this possibility of compensation examiner’s hand that had just been touched on the patient was asked to shake hands with a copyright. the model’s hand, to touch, move, or name fin- second examiner after the examiner had gers of the hand that had just been touched by touched her finger. She then worked out a the examiner without the aid of vision, and with similar strategy: Instead of shaking hands she closed eyes to touch or move the fingers of one quickly grabbed the finger of the examiner hand which corresponded to the fingers of the which corresponded to the finger of her own other hand which had just been touched by the hand that had just been touched and made a examiner. Furthermore, the finger strip test, gesture like shaking hands. the two-point finger test, the in-between test, The deficits aVected each finger but more and the matchbox test as described by often the index finger, the middle finger and Kinsbourne and Warrington were employed.13 the ring finger. Most frequently she mistook In the finger strip test the patient is requested the ring finger for the index finger and vice

to arrange strips of paper on which the names versa. When she was touched three or more http://jnnp.bmj.com/ of the fingers are printed in the correct times in sequence on the same finger she sequence. In the two-point finger test the always gave diVerent responses. While she fingers of the patient are touched in two places. made only few mistakes in the matchbox test The patient judges whether he is being touched and in the two-point test, every second trial of on one or on two fingers. In the in-between test the in-between test was wrong. The wrong two of the patient’s fingers are touched responses never exceeded one digit—for exam- simultaneously by the examiner. The patient is ple, when one finger was between the touched

asked to state the numbers of fingers between fingers she answered either “no finger”, “one on September 24, 2021 by guest. Protected the ones touched (possible answers: 0, 1, 2 or finger“ or “two fingers”. 3). In the matchbox test the patient’s hand is Whereas her performance in naming touched by the examiner in two places, either touched toes and touching toes which had been by one matchbox which is slipped between two named by the examiner was flawless with the fingers, or by two matchboxes each touching aid of vision, she was correct in only 30% of only one finger. The patient is asked to judge trials with closed eyes. In the task in which she whether he is being touched by one or two was touched on one toe and was asked to touch matchboxes. The last three tests were per- the same toe of the other foot she erred in every formed with the patient´s eyes closed. All tests second trial. This was also the case when were performed on both the left and the right responses regarding the big and the little toe hand. were recorded. These deficits aVected the left In addition, the naming and touching of toes and right feet equally. They could not have as instructed by the examiner was examined. been due to loss of or altered sensation since The toes between the big toe and the little toe sensation of the feet had been normal on were named the second, third and fourth toe. neurological examination. of touch, In the examination only the big toe, the third sense of , position sense and of toe and the little toe were touched. The inves- passive and active movement in the toes were 402 Tucha,Steup, Smely, Lange J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.63.3.399 on 1 September 1997. Downloaded from

undisturbed. Vibration sense was slightly around the angular gyrus of the left reduced at the toes in an age-associated hemisphere.32 More recent case reports sup- fashion. port this view. Roeltgen et al and Varney et al To exclude age-related impairment, six have described non-aphasic patients with focal female subjects aged 67 to 75 years without lesions in the posterior parietal region of the neurological or psychiatric diseases were as- left hemisphere who exhibited bilateral finger sessed on the tests relevant to the diagnosis of agnosia and the other elements of the Gerst- Gerstmann syndrome. These subjects were not mann tetrad.23 The study by Morris et al impaired in any of the tasks employed; in arith- showed that electrical stimulation of one locus metic they made a few mistakes. in the transition zone between the angular and supramarginal gyri produced finger agnosia, Comment agraphia, and acalculia.7 Benton, taking ac- The patient described above displayed all four count of the fact that Morris et al could elicit symptoms of the Gerstmann syndrome— distinctive combinations of deficits by stimula- namely, agraphia, acalculia, right-left disorien- tion of very small areas of the posterior tation and finger agnosia. The nature of the parasylvian cortex, postulated that the Gerst- disturbances corresponds with the findings mann syndrome is only one possible syndrome reported by other authors.25 The patient of focal posterior parietal disease.714 Whether displayed neither aphasia nor constructional the four symptoms of the Gerstmann syn- apraxia. Our findings therefore confirm the drome in combination with toe agnosia form a conclusions of a series of authors who sug- new syndrome as proposed by Benton or gested that no causal relation existed between whether finger agnosia is always accompanied aphasia and the Gerstmann syndrome.2133031 by toe agnosia is not clear.14 The related char- This relation was assumed to be the necessary acter of finger agnosia and toe agnosia in our underlying factor in the Gerstmann syndrome patient points to the latter proposition. If future by Heimburger and collegues as well as Poeck studies demonstrate that toe agnosia is a and Orgass.11 12 Our results also contradict the constant symptom of the Gerstmann syn- assumption made by Benson and Geschwind, drome, finger agnosia in the context of this Sobota and Kinsbourne, and Warrington that syndrome would be better named digit agno- constructional apraxia is an additional compo- sia. Toe agnosia should otherwise be regarded nent of the Gerstmann syndrome.5913 We as a symptom which, in combination with the agree with Roeltgen et al who stated that Ger- Gerstmann’s tetrad, constitutes a new syn- stmann’s tetrad may exist without any drome. copyright. other neurological or neuropsychological symptoms.2 1 Gerstmann J. Syndrome of finger agnosia, disorientation for In addition to the four symptoms of the Ger- right and left, agraphia and acalculia. Archives of Neurology stmann syndrome we found an agnosia of the and Psychiatry 1940;44:398–408. 2 Roeltgen DP, Sevush S, Heilman KM. Pure Gerstmann’s toes related to finger agnosia. The examination syndrome from a focal lesion. Arch Neurol 1983;40:46–7. of the toes of patients with Gerstmann 3 Varney NR. Gerstmann syndrome without aphasia: a longi- tudinal study. Cogn 1984;3:1–9. syndrome has until now seldom been consid- 4 Benson DF, Denckla MB. Verbal as a source of ered. Only Benson and Denckla mentioned calculation disturbance. Arch Neurol 1969;21:96–102. briefly an examination of the toes in which they 5 Benson DF, Geschwind N. Developmental Gerstmann syn- 4 drome. Neurology 1970;20:293–8. found no toe agnosia. Although Critchley 6 Moore MR, Saver JL, Johnson KA, Romero JA. Right pari- remarked that some neurologists include the etal with Gerstmann’s syndrome. Arch Neurol 1991; 48:432–5. toes in their examination he gave no further 7 Morris HH, Lüders H, Lesser RP, Dinner DS, Hahn J. http://jnnp.bmj.com/ information about the authors or their Transient neuropsychological abnormalities (including 10 Gerstmann’s syndrome) during cortical stimulation. Neu- findings. Kinsbourne and Warrington stated rology 1984;34:877–83. in their study of finger agnosia that their 8 Hartje W, Dahmen W, Zeumer H. Spezielle Schreib- und Rechenstörungen bei drei Patienten nach Läsion im linken patients displayed no evidence of autotopagno- parieto-okzipitalen Übergangsbereich. Nervenarzt 1981;53: sia and that they had no problems in naming 159–63. 13 9 Sobota WL, Restum WH, Rivera E. A case report of Gerst- parts of the body other than the fingers. They mann’ syndrome without aphasia. Int J Clin Neuropsychol did, however, emphasise that the toes were not 1985;7:157–64. 10 Critchley M. The enigma of Gerstmann’s syndrome. Brain examined. The neglect of the testing of toes in 1966;89:183–98. on September 24, 2021 by guest. Protected the cortical stimulation study of Morris et al is 11 Heimburger RF, Demeyer W, Reitan RM. Implication of the 7 Gerstmann syndrome. J Neurol Neurosurg Psychiatry 1964; unfortunate. The authors were able to elicit in 27:52–7. a 17 year old epileptic patient the four 12 Poeck K, Orgass B. Gerstmann’s syndrome and aphasia. Cortex 1966;2:421–37. symptoms of the Gerstmann syndrome on 13 Kinsbourne M, Warrington EK. A study of finger agnosia. stimulation of certain loci in the perisylvian Brain 1962;85:47–66. 14 Benton AL. Gerstmann’s syndrome. Arch Neurol 1992;49: area. Furthermore, they could transiently pro- 445–7. duce alexia, anomia, constructional apraxia, 15 Benton AL. The fiction of the Gerstmann syndrome. J Neu- rol Neurosurg Psychiatry 1961;24:176–81. conversation diYculties, and spelling in 16 Benton AL. Reflections on the Gerstmann syndrome. Brain addition to the four symptoms of the Gerst- Lang 1977;4:45–62. 17 Damasio H, Damasio AR. Lesion analysis in neuropsychology. mann syndrome. New York: Oxford University Press, 1989. As we found in our patient a larger in 18 Wechsler, D. Wechsler adult intelligence scale-revised (manual). San Antonio: The Psychological Cooperation, 1981. the identification of both toes and fingers when 19 Wechsler D. WMS-R: wechsler-memory-scale-revised opposite sides of the body were involved, the (manual). San Antonio: The Psychological Corporation, 1987. apparent digit agnosia might at least partly be 20 Kimura D. Right damage. Arch Neurol 1963; influenced by right-left disorientation. Gerst- 8:264–71. mann placed the lesion underlying finger 21 Rey A. L’examen psychologiquedans les cas d’encéphalopathie traumatique. Archives de Psychologie agnosia at the parieto-occipital junction 194;28:286–340. Toe agnosia in Gerstmann syndrome 403 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.63.3.399 on 1 September 1997. Downloaded from

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