Journal of Neurology, Neurosurgery, and Psychiatry 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 agnosia in Gerstmann syndrome Oliver Tucha, Anne Steup, Christian Smely, Klaus W Lange Abstract debate concerning the Gerstmann syndrome 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 angular gyrus 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 headache. 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 apraxia, and language functions as well as diseases was admitted to the Department of tests for the assessment of agraphia, acal- Neurosurgery for treatment of a glioblastoma culia, right-left disorientation, and digit multiforme in the left parietal lobe. 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, constructional apraxia, 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, supramarginal gyrus and extended to mann syndrome an agnosia of the toes was the inferior parietal lobule 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 Neuropsychology 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 acalculia, 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 ideomotor apraxia 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 aphasia test25), Correspondence to: showed other clinical manifestions—for exam- including spontaneous speech, 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 writing words, spelling 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 reading, 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.