Gerstmann's Syndrome Research Articles

Surgical treatment of parietal lobe epilepsy

Parietal lobe epilepsy (PLE) accounts for a small percentage of extratemporal epilepsies, and only a few and mostly smaller series have been reported. Preoperative findings, surgical strategies, pathological bases, and postoperative outcomes for PLE remain to be elucidated. Patients with PLE were identified by screening a prospective epilepsy surgery database established in 1989 at the University of Bonn. Charts, preoperative imaging studies, surgical reports, and neuropathological findings were reviewed. Seizure outcome was classified according to Engel class (I-IV). Forty patients (23 females and 17 males) with PLE were identified and had a mean age of 25.0 years and a mean preoperative epilepsy duration of 13.7 years. Nine patients had a significant medical history (for example, trauma, meningitis/encephalitis, or perinatal hypoxia). Preoperative MR imaging abnormalities were identified in 38 (95%) of 40 patients; 26 patients (65%) underwent invasive electroencephalography evaluation. After lesionectomy of the dominant (in 20 patients) or nondominant (in 20 patients) parietal lobe and additional multiple subpial transections (in 11 patients), 2 patients suffered from surgical and 12 from neurological complications, including temporary partial Gerstmann syndrome. There were no deaths. Histopathological analysis revealed 16 low-grade tumors, 11 cortical dysplasias, 9 gliotic scars, 2 cavernous vascular malformations, and 1 granulomatous inflammation. In 1 case, no histopathological diagnosis could be made. After a mean follow-up of 45 months, 27 patients (67.5%) became seizure free or had rare seizures (57.5% Engel Class I; 10% Engel Class II; 27.5% Engel Class III; and 5% Engel Class IV). Parietal lobe epilepsy is an infrequent cause of extratemporal epilepsy. Satisfactory results (Engel Classes I and II) were obtained in 67.5% of patients in our series. A temporary partial hemisensory or Gerstmann syndrome occurs in a significant number of patients.

Dural Arteriovenous Fistula

Images of dural arteriovenous fistula (DAVF) on computed tomography (CT) are usually normal when no hemorrhages have occurred from the DAVF. Therefore, it is difficult to diagnose DAVF by plain CT scans which are usually performed in an initial examination. We present a rare case with DAVF in the transverse-sigmoid sinus that could be diagnosed by reversible speckled high-density spots on a plain CT scan. A 46-year-old man had suffered from depression and had taken some antidepressants for 4 years. He developed a Gerstmann's syndrome and came to our hospital. On a plain CT scan obtained on admission, multiple high-density spots were demonstrated scattered in the left temporal, parietal, and occipital lobes. Subsequently, a digital subtraction angiography was performed. A DAVF in the left transverse-sigmoid sinus was apparent on the angiogram. The DAVF was successfully treated with endovascular surgery. The speckled high density decreased on the postoperative plain CT scan. Multiple high-density abnormalities in the brain shown on a plain CT scan are a rare but important finding for diagnosis of DAVF. Further examinations such as magnetic resonance angiography or digital subtraction angiography should be performed in case with these changes to access the possibility of DAVF.

A study of right unilateral spatial neglect in left hemispheric lesions: the difference between right-handed and non-right-handed post-stroke patients.

We report 20 cases of right unilateral spatial neglect caused by lesions in the left cerebral hemisphere. Differences in neuropsychological symptoms and lesions sites are discussed in connection with handedness. Of the right-handed patients, 6 had severe aphasia, 4 had Gerstmann's syndrome, and 1 had pure agraphia, but unilateral spatial neglect in these cases disappeared after a number of months. Six of the non-right-handed patients had moderate-to-severe aphasia, while the other 3 cases had no aphasia at all. Eight of the 9 cases in this group continued to have right unilateral spatial neglect for more than 6 months. Lesion site as determined by CT differed as to hemisphere, but all fell into the common area previously mentioned in connection with such disorders: i.e., the temporal, parietal and occipital lobes.

Finger recognition and gesture imitation in Gerstmann's syndrome

We report the association between finger agnosia and gesture imitation deficits in a right-handed, right-hemisphere damaged patient with Gerstmann's syndrome (GS), a neuropsychological syndrome characterized by finger and toe agnosia, left–right disorientation and dyscalculia. No language deficits were found. The patient showed a gestural imitation deficit that specifically involved finger movements and postures. The association between finger recognition and imitation deficits suggests that both static and dynamic aspects of finger representations are impaired in GS. We suggest that GS is a disorder of body representation that involves hands and fingers, that is, the non-facial body parts most involved in social interactions.

Arthur Benton and a bunch of Dutch flowers

This paper shows how Arthur Benton's excellent research in the field of neuropsychological topics like hemispheric specialization, Gerstmann syndrome, aphasia, prosopagnosia and visuospatial deficits has influenced the development of the field of neuropsychology in The Netherlands. Also, his outstanding methodological knowledge and clinical experience have had an enormously significant impact on neuropsychological assessment in this country. Benton's knowledge about the different topics in neuropsychology was tremendous, while at the same time he was always critical and aware of findings he was not able to understand.

左中前頭回後部限局性梗塞により不全型Ｇｅｒｓｔｍａｎｎ症候群・超皮質性感覚失語を呈した６５歳男性例

A 65-year-old right-handed man noted a sudden onset of numbness and weakness of the right hand. On the initial visit to our hospital, he showed severe acalculia, and transient agraphia (so called incomplete Gerstmann syndrome) and transcortical sensory aphasia. Brain MRI revealed a fresh infarct in the left middle frontal gyrus. The paragraphia and aphasia improved within 14 days after onset, but the acalculia persisted even at seven months after onset In an 123I-IMP SPECT study, the cerebral blood flow (CBF) was found to be decreased in the infarction lesion and its adjacent wide area, the ipsilateral angular and supramarginal gyri, and contralateral cerebellar hemisphere. We speculate that inactivation in the infarction lesion caused the CBF decrease in the non-infarcted areas due to diaschisis. This case indicates that Gerstmann syndrome can be caused by not only dysfunction of the angular gyrus but also of the left middle frontal gyrus in the dominant hemisphere.

SÍNDROME DE GERSTMANN DEL DESARROLLO Y TRASTORNOS EN LA ADQUISICIÓN DE LAS MATEMÁTICAS

La tétrada caracterizada por una dificultad en el conocimiento práxico de los dedos, indistinción derecha/izquierda, discalculia y trastornos de la escritura (síndrome de Gerstmann) ha sido descrita en el desarrollo, probablemente vinculada a una perturbación madurativa del gyrus angular de manera bilateral o hemisférica derecha. En este trabajo se presentan tres casos con los tres elementos obligados de la tétrada y en un solo caso una perturbación vinculada a los niveles grafémico y ortográfico de la escritura. Dentro de la heterogeneidad de los casos, la perturbación en el dominio de las matemáticas se configura como una anaritmetia, caracterizada en base a un trastorno nuclear en la representación semántica del número. Se realizan las consideraciones teóricas acerca de la relación entre el conocimiento somestésico digital y la capacidad de representarse un espacio regularmente asimétrico, con la adquisición de las facultades matemáticas.

Does finger training increase young children's numerical performance?

Butterworth (1999) suggested that fingers are important in representing numerosities. Furthermore, scores on a finger gnosis test are a better predictor of numerical performance up to 3 years later than intellectual measures (Marinthe et al., 2001; Noël, 2005). We hypothesised that training in finger differentiation would increase finger gnosis and might also improve numerical performance. Accordingly, 47 first-grade children were selected and divided into 3 groups: children with poor finger gnosis who followed the finger-differentiation training programme (G1), a control-intervention who were trained in story comprehension (G2), and a group with high finger gnosis scores who just continued with normal school lessons (G3). The finger training consisted of 2 weekly sessions of half an hour each, for 8 weeks. Before the training period, children in G3 performed better in finger gnosis and enumeration than children in the two other groups. After the training period this pattern remained for the children in G2 and G3, but the children in G1 were significantly better than those in G2 at finger gnosis, representation of numerosities with fingers, and quantification tasks; they also tended to be better at the processing of Arabic digits. These results indicate that improving finger gnosis in young children is possible and that it can provide a useful support to learning mathematics. Such an approach could be particularly appropriate for children with a developmental Gerstmann syndrome. Theoretically, these results are important because they suggest a functional link between finger gnosis and number skills.

発作時にＧｅｒｓｔｍａｎｎ症候群を呈した症候性頭頂葉てんかんの１例

A 34-year-old man with astrocytoma in the left parietal lobe had symptomatic partial epilepsy, and he presented transient episodes of acalculia, agraphia and finger agnosia. Occasionally he had difficulty in finding appropriate letters when making an e-mail, and difficulty in writing and calculation. Neurological examinations revealed ictal symptoms of Gerstmann's syndrome without right to left disorientation. No other higher cortical dysfunction or neurological deficits were noted. Scalp EEGs showed frequent, regional ictal discharges in the left parietal area lasting for 60-240 seconds. These clinico-electrographical observations strongly support that epileptic seizures produced a loss of cortical higher function manifesting Gerstmann's syndrome.

The syndrome of progressive posterior cortical dysfunction: A multiple case study and review.

Dementia presenting with prominent higher order visual symptoms may be observed in a range of neurodegenerative conditions and is often challenging to diagnose.ObjectivesTo describe cases of progressive dementia presenting with prominent visual cortical symptoms.MethodsWe conducted a retrospective search of cases of progressive dementia with predominant visual symptoms, seen at our dementia unit from 1996 to 2006.ResultsTwelve patients (5 men, 7 women) were identified, with ages ranging from 49 to 67 years. At the first examination, the duration of the symptoms ranged from one to ten years and the Mini-Mental State Examination scores from 7 to 27. Eleven patients presented with predominant visuospatial symptoms (partial or complete Balint syndrome) and one with visuoperceptive impairment. Other reported manifestations were: constructional apraxia in 11 patients, partial or complete Gerstmann syndrome in ten, ideomotor apraxia in nine, hemineglect or extinction in four patients, alien hand phenomenon in three, and prosopagnosia in one patient. Memory loss was reported by ten patients, but was not the main complaint in any of these cases. Insight was relatively preserved in five patients even after a long period following the onset of symptoms. Six patients developed parkinsonism during evolution. Clinical diagnoses were possible or probable AD in seven patients, cortico-basal degeneration in four, and dementia with Lewy body in one.ConclusionsClinicians should consider this condition especially in presenile patients with slowly progressive higher-order visual symptoms. Although described in association with different conditions, it may also occur in Alzheimer disease.

Specular Right-left Disorientation, Finger-agnosia, and Asomatognosia in Right Hemisphere Stroke

To search for specular disorders of body representations in right hemisphere stroke. Mirror self-misidentification, asomatognosia, and personal confabulation are similar to body illusions or changes in sensorial or sensorimotor perceptions generated by mirror in right brain damage patients with body image disorders. Prospective study. Ten consecutive right-handed patients (1) performed body part naming and localization tasks and (2) were examined for asomatognosia at the acute phase of stroke, then at least 3 months after stroke, under 3 test conditions: without a mirror, with a conventional mirror, and with an inverted mirror. Video recordings of the tests were analyzed to assess performance. Analysis of variance of the data confirmed that the interaction of mirror's conditions (specifically without a mirror vs. an inverted mirror) with subtest type was significant. The errors are symmetrically distributed. Asomatognosia was "reactivated" in 10 patients who experienced asomatognosia during the acute phase. No particular pattern characterized the clinical manifestations of asomatognosia. A causal conflict of sensorial input is proposed. The specific symptoms observed would suggest the existence of an incomplete specular Gerstmann syndrome and/or Anton Babinski syndrome. These results emphasize the role of specular input in the generation of body representations and self-awareness.

Spatial Neglect, Balint-Homes' and Gerstmann's Syndrome, and Other Spatial Disorders

Brain-damaged patients with lesion or dysfunction involving the parietal cortex may show a variety of neuropsychological impairments involving spatial cognition. The more frequent and disabling deficit is the syndrome of unilateral spatial neglect that, in a nutshell, consists in a bias of spatial representation and attention ipsilateral to of extrapersonal, personal (ie, the body) space, or both, toward the side of the hemispheric lesion. The deficit is more frequent and severe after damage to the right hemisphere, involving particularly the posterior-inferior parietal cortex at the temporo-parietal junction. Damage to these posterior parietal regions may also impair visuospatial short-term memory, which may be associated with and worsen spatial neglect. The neural network supporting spatial representation, attention and short-term memory is, however, more extensive, including the right premotor cortex. Also disorders of drawing and building objects (traditionally termed constructional apraxia) are a frequent indicator of posterior parietal damage in the left and in the right hemispheres. Other less frequent deficits, which, however, have a relevant localizing value, include optic ataxia (namely, the defective reaching of visual objects, in the absence of elementary visuo-motor impairments), which is typically brought about by damage to the superior parietal lobule. Optic ataxia, together with deficits of visual attention, of estimating distances and depth, and with apraxia of gaze, constitutes the severely disabling Balint-Holmes' syndrome, which is typically associated with bilateral posterior parietal and occipital damage. Finally, lesions of the posterior parietal lobule (angular gyrus) in the left hemisphere may bring about a tetrad of symptoms (left-right disorientation, acalculia, finger agnosia, and agraphia) termed Gerstmann's syndrome, that also exists in a developmental form.

Posterior cortical atrophy: A brief review

Posterior cortical atrophy is a striking clinical syndrome in which a dementing illness begins with visual symptoms. Initially, the problem may seem to be loss of elementary vision, but over time the patient develops features of visual agnosia, topographical difficulty, optic ataxia, simultanagnosia, ocular apraxia (Balint's syndrome), alexia, acalculia, right-left confusion, and agraphia (Gerstmann's syndrome), and later a more generalized dementia. Occasional patients have visual hallucinations and signs of Parkinson's disease or Lewy body dementia. A number of different neuropathologic disorders are associated with posterior cortical atrophy.

Acquired Alexia With Agraphia Syndrome in Childhood

The acquired alexia with agraphia syndrome is a conspicuous disorder of reading and writing in the absence of significant other language impairments that has mainly been recorded in adults. Pure cases are rare, with most patients displaying mild aphasic deficits. In children, acquired reading and writing disorders are generally reported as part of more encompassing aphasic syndromes affecting oral and written language equally, for example, Broca or Wernicke aphasia. Documented instances of predominant acquired reading and writing disorders in childhood are exceptional. We report an 11-year-old, right-handed boy who sustained a left temporoparieto-occipital hematoma following rupture of an arteriovenous malformation and who consecutively presented with the acquired alexia with agraphia syndrome associated with word-finding difficulties. Neuropsychologic and neurolinguistic data showed that there was no concomitant Gerstmann and/or angular gyrus syndrome. Th e recoveryfrom the anomia was quite favorable, but recovery of written language was more protracted and acted on the patient's further scholastic achievement. This case is reminiscent of a historical childhood case reported in 1939 and is consonant with adult cases in terms of lesion location and semiologic picture.

What are we seeing?

Posterior cortical atrophy (PCA) is a dementing syndrome that presents with signs and symptoms of cortical visual dysfunction.1 The clinical features of PCA reflect dysfunction of both the ventral/occipito-temporal visual processing pathway causing apperceptive visual agnosia, alexia, and prosopagnosia and dorsal/occipito-parietal pathway causing Balint syndrome (simultanagnosia, optic ataxia and ocular apraxia), transcortical sensory aphasia, apraxia, and some or all of the elements of Gerstmann syndrome (agraphia, acalculia, finger agnosia, right-left disorientation).2,3 Frontal lobe functions and memory are relatively preserved until later in the course of the disease. In this issue of Neurology , McMonagle et al.4 describe the cognitive profile of 19 patients with PCA and compare this to the profile of 11 patients with typical amnestic Alzheimer disease …

Resection of parietal lobe gliomas: incidence and evolution of neurological deficits in 28 consecutive patients correlated to the location and morphological characteristics of the tumor

The goal of this study is to report the incidence and clinical evolution of neurological deficits in patients who underwent resection of gliomas confined to the parietal lobe. Patient demographics, findings of serial neurological examinations, tumor location and neuroimaging characteristics, extent of resection, and surgical outcomes were tabulated by reviewing inpatient and office records, as well as all pre- and postoperative magnetic resonance (MR) images obtained in 28 consecutive patients who underwent resection of a glial neoplasm found on imaging studies to be confined to the parietal lobe. Neurological deficits were correlated with hemispheric dominance, location of the lesion within the superior or inferior parietal lobules, subcortical extension, and involvement of the postcentral gyrus. The tumors were located in the dominant hemisphere in 18 patients (64%); had a mean diameter of 39 mm (range 14-69 mm); were isolated to the superior parietal lobule in six patients (21%) and to the inferior parietal lobule in eight patients (29%); and involved both lobules in 14 patients (50%). Gross-total resection, documented by MR imaging, was achieved in 24 patients (86%). Postoperatively, nine patients (32%) experienced new neurological deficits, whereas seven (25%) had an improvement in their preoperative deficit. A correlation was noted between larger tumors and the presence of neurological deficits both before and after resection. Postoperatively higher-level (association) parietal deficits were noted only in patients with tumors involving both the superior and inferior parietal lobules in the dominant hemisphere. At the 3-month follow-up examination, five of nine new postoperative deficits had resolved. Neurological deterioration and improvement occur after resection of parietal lobe gliomas. Parietal lobe association deficits, specifically the components of Gerstmann syndrome, are mostly associated with large tumors that involve both the superior and inferior parietal lobules of the dominant hemisphere. New hemineglect or sensory extinction was not noted in any patient following resection of lesions located in the nondominant hemisphere. Nevertheless, primary parietal lobe deficits (for example, a visual field loss or cortical sensory syndrome) occurred in patients regardless of hemispheric dominance.

De l'autotopoagnosie à un modèle de représentations des connaissances du corps

Body knowledge can break down in a variety of ways after brain damage. In this article some of these disruptions traditionally associated with left parietal lesion are reviewed. At the beginning of the 20th century, Pick first described autotopoagnosia as an inability to indicate parts of the own body whereas the ability to denominate is preserved. Some years later, Gerstmann introduced the notion of finger agnosia which is the main element of Gerstmann's syndrome (finger agnosia, agraphia, acalculia and left-right confusion). More recently, heterotopoagnosia has been defined as a selective inability to point to another person's body parts while the capacity to point to one's own is intact. Clinical manifestations, cognitive performances and neuroanatomical correlations are exposed and linked.These perturbations are very rare, at least in the pure form (without anomia, apraxia, visuo-spatial or global cognitive dysfunction).They are constant, affecting bilateral parts of the body, without any impact on the daily living. Based on the cases in the literature, the specificity of autotopoagnosia is discussed across different hypotheses. Autotopoagnosia has either been attributed to pure fiction or to a language impairment or to the inability to analyse a whole into its parts. During the last 20 years, further single case studies with circumscribed lesion without aphasia have reported that autotopoagnosia cannot be reduced to a non-specific cognitive disorder. Several accounts propose that autotopoagnosia reflects a human body-specific knowledge impairment. Moreover, implications from the dissociation of disturbed designation and preserved lexico-semantic representations in autotopoagnosia are integrated in a model of the organisation of the body knowledge with multiple levels of representations. The first contains semantic and lexical information about body parts, such as names and functional relationships, strongly linked to the verbal system. The second contains the category-specific visuospatial representations of bodies, such as the structural description of the body, the position and the proximity of the body parts, more strongly linked to the non-verbal visual and somatic sensory systems. The third is a body-reference system that provides a dynamic and actual body image, emerging from various sources of sensory afferences. Finally, motor representations participate to the construction and maintenance of the somatosensory representations. It is proposed that autotopoagnosia is an impairment which predominantly affects body-specific visuospatial representations. Finally, neuroimaging data are suggestive of dysfunction originating in the left dominant parietal regions.

Dexterity with numbers: rTMS over left angular gyrus disrupts finger gnosis and number processing

Since the original description of Gerstmann's syndrome with its four cardinal symptoms, among which are finger agnosia and acalculia, the neuro-cognitive relationship between fingers and calculation has been debated. We asked our participants to perform four different tasks, two of which involved fingers and the other two involving numbers, during repetitive transcranial magnetic stimulation (rTMS) over the posterior parietal lobe of either hemisphere. In the finger tasks, they were required to transform a tactile stimulus randomly delivered on one of their fingers into a speeded key-press response either with the same or with the homologous finger on the opposite hand. In the numerical tasks, they were asked to perform a magnitude or a parity matching on pairs of single digits, in the context of arithmetically related or unrelated numerical primes. In accordance with the original anatomical hypothesis put forward by Gerstmann [Gerstmann, J. (1924). Fingeragnosie: eine umschriebene Stoerung der Orienterung am eigenen Koerper. Wiener clinische Wochenschrift, 37, 1010–12], we found that rTMS over the left angular gyrus disrupted tasks requiring access to the finger schema and number magnitude processing in the same group of participants. In addition to the numerous studies which have employed special populations such as neurological patients and children, our data confirm the presence of a relationship between numbers and body knowledge in skilled adults who no longer use their fingers for solving simple arithmetical tasks.

Unusual Neurological Complication Of Typhoid Fever

A 36-year-old male with typhoid fever presented with conduction aphasia and parietal lobe dysfunction (Gerstmann Syndrome) due to an infarct in the left inferior parietal/superior temporal cortex documented by CT Scan. This case highlights an unusual neurological complication of typhoid fever hitherto not reported in the literature.

A Great Case

“It's a really great case,” the neurology resident said. “Gerstmann's syndrome.” I was a third-year medical student, and neurology was my first clinical rotation. The resident listed the four findings associated with the disorder: agraphia, right–left disorientation, finger agnosia, and acalculia. “Due to a tumor in the parietal lobe,” he explained.We entered the patient's room. A disheveled man in a hospital gown looked at us uncertainly. The resident had the man attempt a series of tasks and maneuvers demonstrating all the elements of the syndrome's tetrad.“What a great case,” I said as we left. The resident smiled.Internal . . .