Toe agnosia in Gerstmann syndrome

The following case report presents a patient exhibitingGerstmann syndrome accompanied by toe agnosia. A 72 year old right handed woman had a focal lesion in the angular gyrus of the left hemisphere which was caused by a glioblastoma multiforme. The firstsymptom she had complained of was severe headache. Standardised neuropsychological tests of intelligence, memory, attention, fluency, apraxia, and language functions as well as tests for the assessment ofagraphia, acalculia, right-left disorientation, and digit agnosia wereperformed. The patient displayed all four symptoms of the Gerstmannsyndrome—namely, agraphia, acalculia, right-left disorientation, andfinger agnosia. The patient did not display aphasia, constructional apraxia, or any other neuropsychological impairment. In addition to thefour symptoms of the Gerstmann syndrome an agnosia of the toes wasfound. Further studies should determine whether finger agnosia inGerstmann syndrome is usually accompanied by toe agnosia. Fingeragnosia in the context of this syndrome may be better named digit agnosia.

     

A Rare Clinical Antity; Pure Gerstmann Syndrome

Gerstmann syndrome is defined as a tetrad including agraphia, acalculia, finger agnosia, and right-left disorientation. In the case studies presented in the literature, it has been reported that Gerstmann syndrome usually appears as an incomplete tetrad of symptoms or accompanied by cognitive deficits including aphasia, alexia, apraxia and some perceptual disorders. Here, we present of the patient with left angular and supramarginal gyrus infarction affecting the parietal lobe. In addition to the symptoms mentioned above, the patient had alexia and anomic aphasia as well. We discussed the clinic appearance and reviewed the current literature.     

Gerstmann syndrome: a case report

A case of Gerstmann syndrome following a trauma is presented. After one month the patient showed the four symptoms of the Gerstmann syndrome associated with slight visual memory and constructional praxis deficits. Eight months later, however, he showed only dyscalculia, dysgraphia, right-left disorientation and finger agnosia, in accordance with selective damage of the left angular gyrus revealed by CT scan. The findings seems to support the existence and the localizing value of Gerstmann syndrome.     

Defective spatial imagery with pure Gerstmann's syndrome

Gerstmann's syndrome comprises finger agnosia, peripheral agraphia, anarithmetia, and right-left confusion. We here report a single-case study of an 85-year-old ambidextrous man who exhibited pure Gerstmann's syndrome (i.e., without aphasia) 10 weeks after a stroke involving the angular gyrus in the left parietal lobe. We hypothesize that, in this case, the main cognitive denominator of Gerstmann's tetrad was a severe dysfunction in mental rotation and translation. This report provides further evidence for the spatial nature of Gerstmann's syndrome.     

Multiple sclerosis with higher cerebral dysfunction: a case report

Higher cerebral dysfunctions such as aphasia, apraxia and agnosia have seldom been reported in multiple sclerosis (MS). 12 year-old right-handed boy felt unsteadiness of the body and headache for several days. Two months later, he had the same episode and complained of visual disturbance, and weakness and sensory disturbance on the face and the extremities. Additionally, he showed amnestic aphasia, acalculia, ideomotor apraxia, finger agnosia and right-left disorientation. Cerebrospinal fluid examinations revealed increases IgG, myelin basic protein and neuron specific enolase (11%, 25 ng/ml and 28.8 ng/ml, respectively). X-ray CT scan and MRI-CT examinations revealed sclerotic lesions on the left parietal white matter and the right mid-brain. The diagnosis was made as MS. He was treated with m-PSL (methyl-prednisolone) pulse therapy for three weeks and consecutively treated with PSL for four weeks. He recovered gradually, but visual disturbance and facial palsy remained. After seven months MRI-CT showed a high signal intensity on the left parietal white matter in spite of the disappearance of the lesion on X-ray CT scan. We suggest that these higher cerebral dysfunctions may result from the lesion of the left parietal white matter which produces a disconnection between each cortical area.     

Ictal Gerstmann's syndrome in a patient with symptomatic parietal lobe epilepsy]

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.     

Developmental Gerstmann's syndrome: a distinct clinical entity of learning disabilities

The symptom complex of finger anomia, right-left disorientation, dysgraphia, and dyscalculia constitutes Gerstmann's syndrome. It is mostly described in adults and is caused by acquired lesions of the dominant parietal lobe. It is infrequently described in children with learning disabilities and has been designated developmental Gerstmann's syndrome. Developmental Gerstmann's syndrome goes unnoticed if not specifically sought by clinicians. A detailed evaluation will reveal subtle neurologic deficits, behavioral problems, and neuropsychologic and specific speech and language abnormalities. Ten such patients are reported; six of the children demonstrated improvement with intensive speech training. Early identification and intervention is therefore crucial, and even more important in cultures in which students are required to be biliterate or triliterate, further increasing the constraints on writing. A selective writing, reading, or calculation abnormality in the presence of normal oral communication triggers several interesting possibilities for the brain mechanisms behind normal language processing. Similarly, the association of acalculia with finger anomia and agraphia with right-left disorientation may have specific implications in the neuropsychologic processing of the evolution of calculation and writing. A theoretical possibility of oral and written language processing from the observation of the language behavior of these children is also described.     

Dissociation between personal and extrapersonal neglect in a crossed aphasia study

Abstract Several reports document crossed aphasia following a right cerebral infarct. However, few of them provide a detailed investigation of associated neuropsychological disorders. A personal neglect disorder with no difficulty in orienting attention in the contralesional space has not been frequently reported independently of lesion side for the language deficit. In most cases, the deficit is described in the acute period. We report the case of a patient who showed severe crossed aphasia several months after cerebral damage. In addition to his language deficit, he suffered from finger agnosia, acalculia, and right/left confusion. Although he was able to orient attention in the contralesional space, he had a persistent personal neglect disorder with severe difficulty in attending to his own body. Results suggest that right hemisphere language dominance does not preclude ipsilateral specialisation for other functions. Furthermore, the dissociation between the two spatial functions provides further confirmation that they are subserved by two independent systems.     

Dissociation Between Personal and Extrapersonal Neglect in a Crossed Aphasia Study

Several reports document crossed aphasia following a right cerebral infarct. However, few of them provide a detailed investigation of associated neuropsychological disorders. A personal neglect disorder with no difficulty in orienting attention in the contralesional space has not been frequently reported independently of lesion side for the language deficit. In most cases, the deficit is described in the acute period. We report the case of a patient who showed severe crossed aphasia several months after cerebral damage. In addition to his language deficit, he suffered from finger agnosia, acalculia, and right/left confusion. Although he was able to orient attention in the contralesional space, he had a persistent personal neglect disorder with severe difficulty in attending to his own body. Results suggest that right hemisphere language dominance does not preclude ipsilateral specialisation for other functions. Furthermore, the dissociation between the two spatial functions provides further confirmation that they are subserved by two independent systems.     

Tactile agnosia and tactile aphasia: symptomatological and anatomical differences.

Two patients with tactile naming disorders are reported. Case 1 (right hand tactile agnosia due to bilateral cerebral infarction) differentiated tactile qualities of objects normally, but could neither name nor categorize the objects. Case 2 (bilateral tactile aphasia after operation of an epidural left parietal haematoma) had as severe a tactile naming disturbance as Case 1, but could categorize objects normally, demonstrating that tactile recognition was preserved. Case 1 may be the first case of tactile agnosia clearly differentiated from tactile aphasia. CT scans of Case 1 revealed lesions in the left angular gyrus, and in the right parietal, temporal, and occipital lobes. Case 2 had lesions in the left angular gyrus and of posterior callosal radiations. Our findings suggest that tactile agnosia appears when the somatosensory association cortex is disconnected by a subcortical lesion of the angular gyrus from the semantic memory store located in the inferior temporal lobe, while tactile aphasia represents a tactual-verbal disconnection.     

Reversed laterality of cerebral functions in a non-right-hander: neuropsychological and spect findings in a case of 'atypical' dominance.

A 54-year-old non-right-handed man with positive familial sinistrality showed a pure right hemisphere syndrome following a left hemisphere stroke. Severe right side hemineglect, transcortical motor dysprosodia, spatial dysgraphia and visuo-constructive impairments were observed. At no time were the expected left hemisphere abnormalities such as aphasia, alexia, right-left disorientation or finger agnosia noted. A left fronto-temporal subcortical lesion was documented on CT scan. A Tc-99m HM-PAO SPECT study revealed no cerebral blood flow changes in the right hemisphere while in the left hemisphere a fronto-temporo-parietal cerebral blood flow reduction was evident. This case of a complete reversed laterality of cognitive functions argues for a distinction to be made between 'anomalous' cerebral dominance and 'atypical' cerebral dominance.     

Pure Gerstmann's syndrome from a focal lesion

It is controversial whether a focal lesion can specifically induce Gerstmann's syndrome (dyscalculia, left-right disorientation, finger agnosia, and agraphia). Also, Gerstmann's tetrad has been attributed to other cerebral symptoms, particularly aphasia. We examined a patient who had all four symptoms of Gerstmann's syndrome, without other symptoms or signs, and who had a discrete left parietal lesion.     

Developmental Gerstmann syndrome associated with cerebellar neoplasm. Report of a case and review of the literature]

A Developmental Gerstmann syndrome in a 7 years-old-boy with hyperactivity, short attention span, acalculia, agraphia, right-left confusion, finger agnosia and constructional apraxia is reported. An initial trial with methylphenidate was done with good improvement regarding hyperactivity, attention span and constructional apraxia. In the clinical course developed a cerebellar syndrome and intracranial hypertension. Surgical exploration of the cerebellum discovered a medulloblastoma. The author believes that this is the first described association of Developmental Gerstmann syndrome and cerebellar neoplasia. The clinical findings, the etiology and the topography of the Gerstmann syndrome are discussed.     

Impairment of Parietal Cortical Functions Associated with Episodic Prolonged Spike-and-Wave Discharges

A case of a 16-year-old girl who showed selective impairment of higher cortical functions and very slight lowering of initiative activity without overt disturbance of consciousness, during prolonged generalized spike-and-wave discharges with a parietal predominance, is reported. Neuropsychological investigations during the episode revealed a disturbance of parietal cortical functions, such as constructional apraxia, ideational or ideomotor apraxia, finger agnosia, acalculia, right-left disorientation, disturbance of visual-spatial orientation, and agraphia. The transient impairment of parietal cortical functions associated with the parietal-dominant spike-and-wave discharges was suggested as a principal pathophysiology of the clinical picture. Emphasis is placed on the importance of neurophysiological assessment in such a case.     

Number processing in posterior cortical atrophy--a neuropsycholgical case study

Posterior cortical atrophy (PCA) is an uncommon syndrome of dementia with early onset, characterised by disorders of higher visual function, variable symptoms of Balint's syndrome, visual agnosia, alexia, agraphia, finger agnosia, right-left disorientation and dyscalculia [Benson D. F., Davis R. J., & Snyder B. D. (1988). Posterior cortical atrophy. Archives of Neurology, 45, 789-793]. In a single case study specific numerical deficits were observed which may be predicted by parietal neurodegeneration (more pronounced on the right side; verified by SPECT). Besides impairments in all tasks involving visuo-spatial abilities (e.g., dot counting, analog number scale task), deficits appeared in tasks requiring access to an internal representation of numbers such as mental number bisection, approximation, estimation and semantic facts. In number comparison an increased distance effect was found. In simple arithmetic, a striking dissociation between operations was found-multiplication and addition facts being preserved at a superficial level, subtraction and division being severely impaired. The study confirms the close relation between spatial and numerical processing and highlights the modular organisation of the semantic system (number semantics impaired). Moreover, the study adds evidence about the clinical manifestation of the particular degenerative syndrome.     

Gerstmann tetrad in Leopard syndrome

We report a 12-year-old boy with multiple lentigines (Leopard) syndrome who was evaluated for learning difficulties and Gerstmann tetrad syndrome (i.e., dyscalculia, left-right disorientation, finger agnosia, and dysgraphia). Cranial computed tomography revealed left ventriculomegaly, more pronounced in the occipital horn suggesting mild atrophy of the left parietal lobe. This is the first report of an association between the Leopard and Gerstmann syndromes and one of the few to demonstrate a computed tomographic abnormality in the latter.     

Gerstmann综合征临床与神经心理学研究

目的研究Gerstmann综合征(GS)的临床与神经心理学特点。方法采用汉语失语症成套测评(ABC)、书写及视空间检查,对259例经CT或MRI检查确诊为脑卒中或脑损伤的患者进行评价。结果 (1)259例患者中有19例患有Gerstmann综合征(GS),其中9例有GS四主征、10例有GS的核心症状、命名性失语(AA)5例、经皮质运动性失语(TCM)2例、经皮质感觉性失语(TCS)4例、Wernicke失语3例、构音障碍2例、无失语5例;(2)病变部位:左顶枕、左侧颞顶、左侧颞顶枕、左侧额顶枕和左侧基底节共17例,右侧基底节、顶叶损害2例。结论 (1)Gerstmann综合征不是一个独立的综合征;(2)其病变部位不仅仅限于优势半球顶叶。     

Arithmetic Facts without Meaning

The paper presents a single-case study of patient J.G. showing severe calculation problems (and also agraphia, finger agnosia, right-left disorientation and apraxia) after the surgery of a left parietal tumor. Although the patient completely lost conceptual knowledge of arithmetic, she preserved part of memorised fact knowledge (multiplications and some additions and subtractions). The study indicates that arithmetic facts can be represented at a superficial level without understanding of the operation performed. The study completes a valid double dissociation between two types of knowledge involved in calculation (memorised facts and conceptual knowledge).     

Right parietal lobe syndrome as a clinical variant of Creutzfeldt-Jakob disease

We report a 57‐year‐old patient with Creutzfeldt‐Jakob disease that initially presented with right parietal lobe syndrome. He featured early dystonic and hemiparetic posture of the left upper limb, finger agnosia, geographic disorientation and acalculia and developed myoclonus from the left upper extremity and right‐sided periodic synchronized discharges. He died 2 years and 3 months later. Brain autopsy revealed a marked reduction in brain weight, extensive cortical spongiform changes and white matter degeneration. Immunolabeled prion proteins were deposited diffusely in the neuropil. Accentuation of the cortical change was unclear but white matter degeneration was more severe in the right parietal lobe than in the left parietal lobe. Right parietal lobe syndrome is rarely reported as an initial symptom of Creutzfeldt‐Jakob disease and can be a clinical variant of the disease.     

Multi-infarct dementia clinically simulating dementia of Alzheimer type. A comparison with angular gyrus syndrome

A 74-year-old right-handed man with multiple cerebral infarction who presented with dementia simulating dementia of Alzheimer type (DAT) is reported. He had been well until April 20, 1987 when he developed transient right hand palsy lasting overnight. Eleven days later, he became confused, disorientated, and amnestic. He was admitted to this hospital on June 8. Physical examination revealed hypertension (170/90mmHg). On neurological examination, his consciousness was clear but he was demented. He showed disorientation, amnesia, and urinary incontinence. His most prominent symptom was disturbance of speech, including fluent aphasia and alexia with agraphia. Additionally, he showed ideomotor apraxia, construction apraxia, right-left agnosia, finger agnosia, and acalculia. On July 9, he had a transient attack of right hemiplegia with confusion. The brain CT scan performed on admission was unremarkable except for cavum septi pellucidum and a small low density area in the right basal ganglia. However, single photon emission computed tomography (SPECT) by 123I-labeled N-isopropyl-p-iodoamphetamine disclosed hypoperfusion of the cerebral blood flow in the border zones of the temporoparietal and frontal lobes on the left. A follow-up brain CT scan taken one month later demonstrated low density in the new areas corresponding to hypoperfusion shown by SPECT. Although the clinical features of the present case resembled those of DAT, dementia in this case was regarded as the result of multiple cerebral infarction since it occurred acutely with mild motor deficits, and brain CT scans and SPECT showed lesions indicating focal cerebral ischemia.(ABSTRACT TRUNCATED AT 250 WORDS)