Trakia Journal of Sciences, No 3, pp 239-241, 2018 Copyright © 2018 Trakia University Available online at: http://www.uni-sz.bg ISSN 1313-7050 (print) ISSN 1313-3551 (online) doi:10.15547/tjs.2018.03.011

Case Report GERSTMANN IN A YOUNG MAN: A CASE REPORT

Iv. Dimitrov1*, S. Atanasova2, A. Kaprelyan2, B. Ivanov3, V. Nestorova4, K. Drenska2, Zh. Chuperkova5, Iv. Aleksandrov1

1Department of Nursing, Sliven Affiliate, Prof. Paraskev Stoyanov Medical University, Varna, Bulgaria 2Department of and Neuroscience, Faculty of Medicine, Prof. Paraskev Stoyanov Medical University, Varna, Bulgaria 3Department of Clinical Medical Sciences, Faculty of Dental Medicine, Prof. Paraskev Stoyanov Medical University, Varna, Bulgaria 4Department of Physiotherapy, Rehabilitation, Thalassotherapy, Occupational Diseases, Faculty of Public Health, Prof. Paraskev Stoyanov Medical University, Varna, Bulgaria 5Child Neurology Sector, Second Pediatric Clinic, MHAT St. Marina, Varna, Bulgaria

ABSTRACT Gerstmann syndrome is a classical cerebral syndrome in neurology, named after Joseph Gerstmann, a Jewish Austrian-born American neurologist. Patients present with a tetrad of cognitive symptoms, including , , finger and left-right disorientation. The syndrome is known to result from a lesion of the posterior portion of the dominant and is usually due to or to developmental problems. We describe the case of a 35-year-old man whose illness debuted about 9 months before the initial presentation to the neurology clinic, with memory complaints, anxiety, verbal aggression, sleeping problems, as well as subjective word finding difficulty and depressed mood. The patient had 3 out of the 4 classic symptoms of Gerstmann syndrome, among other, mostly neuropsychiatric symptoms. Initially, structural lesions were sought for, but were not found on magnetic resonance imaging. Psychiatric conditions were discussed but not confirmed by the consulting psychiatrist. We are prone to accepting a non-organic reason for the condition of the patient, but follow-up of the clinical course and repeated assessments, including neuropsychological and psychiatric evaluations, structural and possibly functional neuroimaging will be required to verify and confirm this presumption.

Key words: Gerstmann syndrome, agraphia, acalculia, finger agnosia, left-right disorientation

INTRODUCTION known to result from a lesion of the posterior Gerstmann syndrome is a classical cerebral portion of the dominant parietal lobe (3). The syndrome in neurology. It was named after affected area is usually the (4) Joseph Gerstmann, a Jewish Austrian-born which is responsible for processing the visual American neurologist (1). Patients present with images of letters and words in meaningful a tetrad of cognitive symptoms, including information, as is done during . Such agraphia, acalculia, finger agnosia and left- lesions in adults are usually seen in stroke, right disorientation (2). The syndrome is while in children the syndrome can be related ______to developmental problems (Developmental *Correspondence to: Ivan Dimitrov, Department Gerstmann syndrome). In the latter cases the of Nursing, Sliven Affiliate, Prof. Paraskev aetiology remains unknown (5). The clinical Stoyanov Medical University, Sveta Marina manifestations that constitute the syndrome are University Hospital, First Clinic of Neurology, 1 heterogeneous. That is why some authors Hristo Smirnenski st., 9010 Varna, Bulgaria, Telephone: +359 52 978 524, E-mail: consider unlikely the possibility that damage to [email protected] the same population of neurons should account for all of the four symptoms, and explain the

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DIMITROV IV., et al. syndrome with lesions causing disconnection hydrochloride in usual doses was prescribed. of co-localized tracts in the subcortical white He had not carried out the treatment, stating matter of the parietal lobe (6). “personal reasons”.

CASE REPORT As the symptoms persisted, the patient was We describe the case of a 35-year-old man sent to the neurology clinic for admission, whose illness debuted about 9 months before further assessment and treatment. The the initial presentation to the neurology clinic, neurological exam was normal. Only elevated with memory complaints, anxiety, verbal ALAT (77.0 U/L) was found on laboratory aggression, sleeping problems, as well as assessment. Abdominal ultrasonography subjective word finding difficulty. According showed no pathological changes. to his wife, he had become depressed in the Neuropsychological assessment was repeated. recent months and had developed the habit to MMSE score was 27 points with evidence of ask the same questions multiple times. For the . Isaacs set test showed normal last month he had had 2 collapses with category verbal fluency with a score of 36 measured close values of systolic and diastolic points. Clock drawing test was normal, with a blood pressure, with no convulsions. Ten years maximal score of 10 points according to earlier, he had suffered a head trauma – Sunderland’s scoring method. Luria’s memory whiplash injury with no fractures or loss of test showed fixation of 66% (lower normal consciousness. The patient had no other level) and reproduction of 75% (middle normal relevant medical history. He denied alcohol or level). Finger agnosia and left-right other . disorientation were present. Psycho-emotional instability and fluctuations of cognitive In the outpatient care, due to the described processing were noted. complaints, he was consulted by a neurologist and a psychiatrist, electroencephalography and The consultant psychiatrist reported that the carotid/vertebral Doppler ultrasonography patient was calm, orientated, euthymic, with were performed, which were normal. mild alterations of thinking, but no psychiatric Neuropsychological assessment showed mild illness was diagnosed. magnetic to moderate frontal dysfunction, impaired resonance imaging (MRI) was performed in attention, mild disturbance of immediate recall order to seek for structural lesions, but no and moderate of delayed recall, emotional and pathology was detected (Figure 1). The patient behavioral disturbances. Mini- Mental State was discharged in good general condition, with Exam (MMSE) score was 29 points. The recommendations for neurologic, psychiatric, patient was classified as “neurasthenic” and neuropsychological and, if needed, treatment with valproate and hydroxyzine neuroimaging follow-up.

Figure 1. Normal MRI of the brain in axial, sagittal and coronal planes

DISCUSSION vascular risk factors, which additionally The described case shows the presence of 3 out reduced the likelihood of vascular disease to be of the 4 classic symptoms of Gerstmann the reason for the clinical manifestations. In syndrome: dyscalculia, finger agnosia and left- line with the statement that Gerstmann right disorientation, among other, mostly syndrome may result from intraparietal neuropsychiatric symptoms. Initially, structural disconnection after damage to a focal region of lesions were sought for, but were not found on subcortical white matter (7, 8), and taking into MRI. The patient was young and had no account the age of the patient, we also

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DIMITROV IV., et al. considered a demyelinating disease, which, lesion. Archives of Neurology, 40(1):46-47, again, was not confirmed by MRI. The history 1983. of whiplash injury was taken into account, but 3. Wilkins, RH and Brody, IA. Gerstmann’s evidences for connection with the current syndrome. Archives of Neurology, condition were not convincing. As 24(5):475-475, 1971. psychiatric/neuropsychiatric symptoms were 4. João, RB, Filgueiras, RM, Mussi, ML, dominating the clinical picture, a psychiatric Barros, JEFd. Transient Gerstmann disease was an important differential syndrome as manifestation of stroke: Case diagnostic option. For instance, it has been report and brief literature review. stated in the literature that the symptoms of & Neuropsychologia, 11:202-205, 2017. Gerstmann syndrome can be present in 5. Kinsbourne, MM, Warrington, EK. The patients, even in the early stages developmental Gerstmann syndrome. (9). Some mild alterations of thinking were Archives of Neurology, 8(5):490-501, 1963. noted in our patient, but the consultant 6. Rusconi, E, Pinel, P, Dehaene, S, psychiatrist did not diagnose a psychiatric Kleinschmidt, A. The enigma of condition. Nevertheless, in this case we are Gerstmann’s syndrome revisited: a telling prone to accepting a non-organic reason for the tale of the vicissitudes of . condition of the patient. Malingering was Brain, 133(2):320-332, 2010. discussed, but considered unlikely. Follow-up 7. Rusconi, E, Pinel, P, Eger, E, et al. A of the clinical course and repeated disconnection account of Gerstmann assessments, including neuropsychological and syndrome: functional neuroanatomy psychiatric evaluations, structural and possibly evidence. Ann Neurol, 66(5):654-662, functional neuroimaging will be required to 2009. verify and confirm this presumption. 8. Rusconi, E. Gerstmann syndrome: historic and current perspectives. Handb Clin ACKNOWLEDGEMENT Neurol, 151:395-411, 2018. No funding was obtained for this study. 9. Liu, X, Zhuo, C, Qin, W, et al. Selective functional connectivity abnormality of the REFERENCES transition zone of the inferior parietal 1. Triarhou, LC. Josef Gerstmann (1887- lobule in schizophrenia. NeuroImage: 1969). J Neurol. Apr 2008;255(4):614-615. Clinical, 11:789-795, 2016. 2. Roeltgen, DP, Sevush, S, Heilman, KM. Pure Gerstmann’s syndrome from a focal

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