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Current and Neuroscience Reports (2020) 20:48 https://doi.org/10.1007/s11910-020-01069-9

BEHAVIOR (H.S. KIRSHNER, SECTION EDITOR)

Gerstmann

Alfredo Ardila1,2

# Springer Science+Business Media, LLC, part of Springer Nature 2020

Abstract Purpose of Review Gerstmann (left angular gyrus) syndrome includes the tetrad of finger (inability to distinguish, name, and recognize the fingers), (acquired disturbance in the ability to write), acalculia (loss of the ability to perform arithmetical operations and use numerical concepts), and right-left disorientation (right-left discrimination defect when using language). There is some disagreement regarding its exact localization, but it most likely involves the left angular gyrus with a probable subcortical extension. This article reviews recent research on the clinical aspects of this syndrome. Recent Findings During the last years, just some few new reports of are found in neurological and neuropsychological literature. Most of the reports are single-case reports. An association between Gerstmann syndrome and the so-called semantic aphasia has been pointed out. Two different explanations to this unusual syndrome have been recently proposed: (1) the pathological process is located in the left parietal white matter disconnecting separate cortical networks and (2) it represents a disturbance in the ability to verbally mediate some spatial knowledge. Summary Although Gerstmann syndrome continues as a controversial syndrome, and most of the reports are single case reports, recently two different explanations have been advance the understanding of this polemic but fascinating syndrome.

Keywords Gerstmann syndrome . Angular gyrus syndrome . Semantic aphasia

Introduction understood as a kind of disturbance in the body scheme. Some authors have overtly opposed to it and have even con- Joseph Gerstmann [1] proposed a clinical syndrome associat- sidered that Gerstmann syndrome is a fiction and it is simply ed to lesions in the left hemisphere posterior an artifact due to incorrect observations (e.g., [3, 4]). which included four different signs: finger agnosia (inability Gerstmann syndrome has been a controversial syndrome in to distinguish, name, and recognize fingers), agraphia (ac- neurology and neuropsychology [5–8]. The controversy is due quired disturbance in the ability to write), acalculia (loss of to (1) the observation that this clinical syndrome usually ap- the ability to perform arithmetical operations and use numer- pears as either, an “incomplete” tetrad or it is found in asso- ical concepts), and right-left disorientation (right-left discrim- ciation to other cognitive deficits, particularly, aphasia, alexia, ination defect when using language). This syndrome has usu- and perceptual disorders [9] and (2) its exact localization (for ally been named as “Gerstmann syndrome” and sometimes as an extensive review of its history see [10••]). “angular gyrus syndrome” [2]. Gerstmann [1](1940)consid- It is accepted that Gerstmann syndrome is an unusual clin- ered that this syndrome represents a disorder of the body sche- ical syndrome. For example, Zukic et al. [11]observedina ma involving the hand and the fingers. Hence, it can be sample of 194 acute patients that 59 (about 30%) had alexia, agraphia, and acalculia or different combinations of these three disorders. However, only two patients (about Behavior This article is part of the Topical Collection on 1%) presented agraphia and acalculia associated with the other two signs of the Gerstmann syndrome: finger agnosia and left- * Alfredo Ardila right disorientation. The conclusion is evident: Gerstmann [email protected] syndrome is a quite unusual clinical syndrome in acute stroke 1 Institute of Linguistics and Intercultural Communication, I.M. patients. Sechenov First Moscow State Medical University, Moscow, Russia Some controversies remain also regarding the exact local- 2 Psychology Doctoral Program, Albizu University, 12230 NW 8th ization of this syndrome in the . The discrepancy refers to Street,Miami,FL33182,USA the specific brain areas involved in it, not to its lateralization; it 48 Page 2 of 5 Curr Neurol Neurosci Rep (2020) 20:48 seems to exist a general agreement that it is usually observed successive subordinate clauses; (b) reversible constructions, in cases of left hemisphere pathology. Seemingly, there is only particularly of the temporal and spatial type (c) constructions one case report in the available literature describing the with double negative; (d) comparative sentences; (e) passive Gerstmann syndrome associated with right parietal hemor- constructions; (f) constructions with transitive verbs; and (g) rhage in an ambidextrous patient [12]. The observation that constructions with attributive relations. He also stated that the Gerstmann syndrome can appear with electrical stimulation in spatial disorders not only incidentally accompany semantic the left posterior parietal area emphasizes its angular localiza- aphasia but that semantic aphasia itself is a defect in the per- tion [13]. Diverse clinical reports have supported the left pos- ception of simultaneous structures transferred to a higher sym- terior parietal localization [14, 15]. Some authors, however, bolic level. In other words, patients with semantic aphasia have reported pure Gerstmann syndrome associated with have difficulty understanding the meaning of words tinged subangular lesions [16, 17]. with spatial or semi-spatial meaning. It is important to empha- At least one paper has reported an unusual and unexpected size that semantic aphasia is strongly associated with localization for the syndrome. Lee et al. [18] reported two acalculia; as a matter of fact, according to Luria, semantic cases of Gerstmann syndrome associated with infarction in aphasia and acalculia share a common underlying defect: un- the left middle aspect of the . The authors sug- derstanding of language-dependent spatial relations. gested that this apparent discrepancy could be explained by Acalculia has been defined as an acquired disturbance in disconnection of functional fibers between the frontal and pa- computational ability [36]. Several classifications of acquired rietal cortex, implicitly assuming that anyhow the syndrome calculation disturbances are found in the literature. Luria [37] was due to the posterior parietal lobe involvement. distinguished three types of acalculia: optic acalculia, frontal Considering its rarity, it has been suggested that the simul- acalculia, and primary acalculia. Hecaen et al. [38]alsorefer taneous occurrence of finger agnosia, right-left disorientation, to three variants of acalculia: alexia and agraphia for numbers, acalculia, and agraphia is simply coincidental. For instance, spatial acalculia, and anarithmetia. However, the most fre- Wingard et al. [19] suggested that the four cognitive functions quent distinction found in contemporary literature includes impaired in the syndrome do not share a common neuronal primary acalculia and secondary acalculias. Ardila and network; they may appear simultaneously in cases of left pos- Rosselli [39] proposed a comprehensive classification of terior parietal pathology because of the anatomical proximity acalculias. They distinguished six different types acalculia: of the different networks mediating these four functions. (a) primary acalculia or anarithmetia, (b) aphasic acalculia, Kleinschmidt and Rusconi [20] consider that a local lesion (c) alexic acalculia, (d) agraphic acalculia, (e) dysexecutive involving the left parietal white matter provides the only rea- (frontal) acalculia, and finally (f) spatial acalculia. Primary sonable explanation for the pure Gerstmann syndrome. acalculia corresponds to anarithmetia. The remaining types It could be tentatively concluded that the most probable of acalculia are considered secondary. Therefore, acalculia localization of the Gerstmann syndrome involves the left pos- can result from not only primary disturbances in numerical terior parietal lobe (angular gyrus) with a subcortical abilities but also to executive function impairments, spatial extension. disturbances, and oral and written language defects. The acalculia found in the Gerstmann syndrome corresponds to a Gerstmann Syndrome and Semantic Aphasia primary acalculia. Since the initial description of primary acalculia [40], it has The potential association of the Gerstmann syndrome with so- been related to left posterior parietal pathology. Dehaene and called semantic aphasia has not been sufficiently analyzed colleagues [41] proposed a tripartite organization of numerical [21]. Indeed, for decades, semantic aphasia has been a knowledge. The horizontal segment of the intraparietal sulcus neglected type of aphasia [22, 23]. During the early twentieth is systematically activated whenever numbers are manipulat- century, Head [24] described a language alteration whereby he ed. A left angular gyrus area, in connection with other left- defined an inability to recognize simultaneously the elements hemispheric perisylvian areas, supports the manipulation of within a sentence, and he called it semantic aphasia. Initially, numbers in verbal form. Finally, a bilateral posterior superior this syndrome was well accepted and integrated to the aphasia parietal system supports attentional orientation on the mental literature (e.g., [25–28]). However, when the Wernicke- number line, just like on any other spatial dimension. Lichtheim model of aphasia [29] was adopted in the western The following is a case of a patient presenting both seman- neuropsychology and (e.g., [30]), the tic aphasia and Gerstmann syndrome. Ardila, Concha, and interest in semantic aphasia virtually disappeared. Since then, Rosselli [2] reported a 58-year-old right-handed male, with only some few references have appeared in the an ischemic lesion to the left angular gyrus, who showed sub- literature [21–23, 31–34]. Luria [27, 35] considered that lan- stantial difficulties in understanding logic-grammatical rela- guage deficiencies of semantic aphasia can be found in the tionships, comparison adverbs (e.g., bigger-smaller, youn- following situations: (a) sentences with a complex system of ger-older, etc.), place adverbs (e.g. over-below, on-beneath, Curr Neurol Neurosci Rep (2020) 20:48 Page 3 of 5 48 etc.), and time adverbs (e.g., before-after), compatible with a provides the only reasonable explanation for pure semantic aphasia. difficulties were minimal. In addi- Gerstmann syndrome. The critical pathology in consequence tion, he had important impairments in finger gnosis, right-left should be subcortical. This particular type of lesion location discrimination, understanding numbers, using numerical con- would impair not only a single fiber tract but crossing different cepts, and performing arithmetical operations. The authors fiber tracts and consequently would disconnect separate corti- proposed that in the Gerstmann syndrome agraphia should cal networks. Rusconi et al. [49••] further suppose that it is be replace by semantic aphasia or semantic aphasia should very unlikely that damage to the very same population of be considered as the fifth clinical sign of the Gerstmann cortical could explain the four clinical signs. They syndrome. emphasize that a pure form of Gerstmann syndrome could only arise from a disconnection due to a lesion in the subcor- Recent Research tical parietal white matter. Other authors have presented an alternative explanation, During the last 10 years, only some few papers describing assuming that patients with Gerstmann syndrome present a Gerstmann syndrome have been published. Most of them defective ability to mentally manipulate information; conse- have been single-case reports [42–44]. Two new reports of quently, an impaired transformation of images would be the paroxysmal Gerstmann syndrome as a manifestation of epi- common denominator underlying the four clinical signs. This lepsy have also been published [45, 46]. interpretation was initially proposed by Gold et al. [40]and From the conceptual perspective, two theoretical explana- during the following years has been supported by different tion of the Gerstmann syndrome have been proposed: (1) as a authors [2, 15–17]. Gold et al. [50]studiedapatientafocal impairing different fiber tracts infarct of the left angular gyrus; the patient’s performance was connecting subcortical and cortical areas [10••, 47–48] and always poor when mental rotations to a commands were re- (2) as a disturbance in the ability to verbally mediate some quired. The authors hypothesized that a defect in horizontal spatial knowledge [55]. The first one is assuming that there is translation, i.e., mental rotation, accounted for the right-left a kind of anatomical subcortical hub that can control and result disorientation in their patient. Moreover, acalculia and other in four different types of cortical manifestations. The second signs associated with the Gerstmann syndrome could also be one supposes that the angular gyrus is indeed not specialized understood as a defect in the performance of these mental in finger gnosis, right-left discrimination, writing, or numeri- rotations. This impairment in mental rotations could potential- cal ability, but in a particular way of information processing ly be observed in the impaired understanding of comparisons, underlying these four abilities. time, and place adverbs, observed in semantic aphasia. Rusconi et al. [49••] suggested that Gerstmann syndrome Consequently, it could be hypothesized that a single underly- have been so enigmatic and polemic because the four clinical ing deficit—defective mental rotations—could potentially ac- signs that are included lack a functional logic relationship that count for right-left disorientation, finger agnosia, acalculia, would withstand more rigorous examination. Simply speak- and semantic aphasia; their simultaneous manifestations in a ing: the crucial question is: “Is it possible to find a common single clinical syndrome is not coincidental. cognitive factor responsible for the four clinical signs of the Ardila [49••] emphasized that Gerstmann syndrome is the Gerstmann syndrome?”. If it is not, we should assume that its result of a disturbance in the ability to verbally mediate some association is due to a brain pathology capable to simulta- spatial knowledge (kind of verbal mediation of spatial opera- neously impair these four cognitive abilities. These authors tions) that is manifested both at the linguistic and at the nu- suggest that the Gerstmann syndrome does not (or if so only merical level. Gerstmann syndrome could be restated to in- indirectly) reflect the functional architecture of human cogni- clude acalculia, finger agnosia, right-left disorientation, and tion but rather the functional architecture of the brain and semantic aphasia. Agraphia would still remain unexplained should be interpreted as a disconnection between some sub- by this unifying underlying mechanism. However, when the cortical and cortical areas. Rusconi [10••, 47–48]considers pathology extends toward the superior parietal gyrus, agraphia that a local lesion involving the left parietal white matter can be found. As illustrated in Fig. 1, a single underlying

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