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。     

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.     

Aphasia, alexia, and oral reading

Alexia is an acquired disturbance in reading. Alexias that occur after left hemisphere damage typically result from linguistic deficits and may occur as isolated symptoms or as part of an aphasia syndrome. This article presents an overview of the classification of the alexias, including both the traditional neuroanatomical perspective and the more recent psycholinguistic approach. Then, assessment procedures are reviewed, followed by a summary of treatment approaches for alexia. Finally, two case studies illustrate how oral reading of connected language (sentences and paragraphs rather than single words) has been used as a technique for treating alexia in patients with aphasia.     

Rehabilitation of pure alexia: A review

Acquired reading problems caused by brain injury (alexia) are common, either as a part of an aphasic syndrome, or as an isolated symptom. In pure alexia, reading is impaired while other language functions, including writing, are spared. Being in many ways a simple syndrome, one would think that pure alexia was an easy target for rehabilitation efforts. We review the literature on rehabilitation of pure alexia from 1990 to the present, and find that patients differ widely on several dimensions, such as alexia severity and associated deficits. Many patients reported to have pure alexia in the reviewed studies, have associated deficits such as agraphia or aphasia and thus do not strictly conform to the diagnosis. Few studies report clear and generalisable effects of training, none report control data, and in many cases the reported findings are not supported by statistics. We can, however, tentatively conclude that Multiple Oral Re-reading techniques may have some effect in mild pure alexia where diminished reading speed is the main problem, while Tacile-Kinesthetic training may improve letter identification in more severe cases of alexia. There is, however, still a great need for well-designed and controlled studies of rehabilitation of pure alexia.     

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.     

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.

     

Contrecoup injury in the misdiagnosis of crossed aphasia

Crossed aphasia is reported to be more frequent in traumatic series than in series of patients with other pathologies. A right-handed young man suffered a closed-head trauma and became aphasic and hemiparetic on the left. CT scan revealed a right frontal-lobe hematoma. Neuropsychological examination revealed a fluent aphasia and a Gerstmann syndrome. These signs were compatible with left supramarginal gyrus syndrome. However, the presence of a right frontal-lobe lesion suggested that this patient could be a crossed aphasic. Subsequent EEG study showed a left occipitotemporal focus and a right frontal one. Aphasic signs could thus be due to the left lesion, which was the result of a contrecoup mechanism. Fluent aphasias have been reported in closed-head trauma with right frontal impact. Attention is called for the possible bias of including cases like this in series of traumatic crossed aphasia.     

Contrecoup injury in the misdiagnosis of crossed aphasis

Abstract

Crossed aphasia is reported to be more frequent in traumatic series than in series of patients with other pathologies. A right-handed young man suffered a closedhead trauma and became aphasic and hemiparetic on the left. CT scan revealed a right frontal-lobe hematoma. Neuropsychological examination revealed a fluent aphasia and a Gerstmann syndrome. These signs were compatible with left supramarginal gyrus syndrome. However, the presence of a right frontal-lobe lesion suggested that this patient could be a crossed aphasic. Subsequent EEG study showed a left occipitotemporal focus and a right frontal one. Aphasic signs could thus be due to the left lesion, which was the result of a contrecoup mechanism. Fluent aphasias have been reported in closed-head trauma with right frontal impact. Attention is called for the possible bias of including cases like this in series of traumatic crossed aphasia.     

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.     

Neurolinguistic analysis of the language abilities of a patient with a "double disconnection syndrome": a case of subangular alexia in the presence of mixed transcortical aphasia.

In contrast to the classic form of alexia without agraphia, subangular alexia results from a single lesion located deep in the white matter of the left parietal lobe. In the present report, a patient with subangular alexia and features of mixed transcortical aphasia is described. Neurolinguistic findings include: alexia without agraphia, paucity of spontaneous speech, moderate auditory comprehension difficulty, excellent repetition, echopraxia, colour agnosia, and naming disorder. Neurolinguistic tests revealed intact phonological organisation and grammatical filter." Our studies revealed a "double disconnection syndrome," the co-existence of two relatively rare neurobehavioural disorders. Furthermore, the studies reported here clearly show dissociations of language functions in both the visual and auditory modality, which demonstrates that the stages of language processing are separable.     

Slowly progressive ischemic stroke as first manifestation of essential thrombocythemia

We report a case of essential thrombocythemia, the only clinical manifestation of which consisted of neurologic symptoms, including anomic aphasia, tactile and painful hypesthesia in the right hand, headache, and Gerstmann syndrome, with a relatively benign and slowly progressive clinical course. Neuroradiologic examination disclosed a large ischemic area in the left temporoparietal region. Cerebral angiography revealed an occlusion of the cortical branches of the left middle cerebral artery with total sparing of the carotid vessel. These findings are discussed in relation to the possible pathogenetic mechanisms of the vascular occlusion due to abnormal platelet function in essential thrombocythemia.     

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.     

Recovery from alexia without agraphia: report of an autopsy.

The patient is a 58-year-old Japanese teacher of German literature who suffered twice from cerebrovascular accidents, showing alexia without agraphia. Pathological examination showed an old infarct in the posterior two-thirds of the fusiform and almost the whole lingual gyrus, involving the posterior border of the parahippocampal gyrus in the left hemisphere. The left cuneus and the calcarine cortex were preserved. There was degeneration of the lower third of the splenium of the corpus callosum, extending to its occipital radiation and tapetum on both sides. Comparing clinico-pathological findings of the 31 known autopsy cases, it was proposed that the lesion of the left spleno-lingual system produces alexia without agraphia but it may ameliorate. In addition, when spleno-cuneate system is also involved alexia becomes persistent and it may accompany object agnosia or optic aphasia.     

Difficulty differentiating a case of posterior cortical atrophy from a psychogenic disturbance of vision

Differentiating posterior cortical atrophy (PCA) from other diseases can be difficult and time‐consuming, and there is a particularly high possibility of misdiagnosis when psychiatrists diagnose complaints related to visual perception. Here, a case of PCA involving prominent visual perceptual disorders is reported; PCA was difficult to distinguish from psychogenic disturbance of vision in this case. For a year, a 59‐year‐old woman had had visual perceptual disorders, including a distorted view and prosopagnosia. She underwent examinations at multiple clinical departments at several medical institutions before receiving a definitive diagnosis of PCA. This PCA diagnosis was based on clinical symptoms, including Gerstmann syndrome, Bálint's syndrome, and transcortical sensory aphasia, and hypoperfusion in the occipital lobe observed on single‐photon emission computed tomography. This case was initially misdiagnosed as a psychogenic disease partly because characteristic clinical manifestations of PCA include visual agnosia with a disjunctive component. This patient displayed a disordered perception of stationary objects but an intact perception of moving objects. For example, she had to grope her way through a room at home, but she could visit a familiar hair salon on foot without hindrance. Behaviours like claiming to be blind while inexplicably moving without colliding with surrounding objects may lead to the misdiagnosis of PCA as a psychogenic or dissociative disorder involving histrionic or neurologically irrational symptoms with an expectation of sympathy or personal gain. It is critical to make every effort to exclude organic diseases, even in cases provisionally diagnosed as psychogenic disease. Despite its low prevalence, PCA should be considered a syndrome caused by Alzheimer's disease, dementia with Lewy bodies, or other dementias.     

Crossed aphasia: New perspectives

Abstract

Crossed aphasia reflects an unusual latcralization pattern characterized by a dissociation between manual and language hemispheric dominance in a right-handed individual. In order to circumscribe the syndrome of crossed aphasia, authors have developed stringent exclusion criteria to define that syndrome. We believe that such an approach artificially created a homogeneous syndrome. As an alternative to the rigorous exclusion criteria approach, we propose to broaden the definition of crossed aphasia to accommodate the various demonstrated aphasic symptoms. The interest in crossed aphasia can then shift from the search for a universal symptom complex to different subgroups of patients. Indeed, there now appears to be overwhelming evidence (including Wada experiments) to indicate that crossed aphasia can cither be the mirror image of uncrossed aphasia or indicate a bilateral language representation pattern. Two patients are presented and their symptoms are discussed in the light of the literature. One of our patients is the only one to our knowledge to evidence affective prosody impairments. We argue that this latter symptom be added to the list of associated signs to be assessed as part of a crossed aphasia battery.     

Developmental Gerstmann's syndrome

The tetrad of finger agnosia, dysgraphia, dyscalculia, and right-left disorientation make up Gerstmann's syndrome. The tetrad has been infrequently described in children with learning disability and has been called developmental Gerstmann's syndrome (DGS). Developmental Gerstmann's syndrome may occur in brain-damaged and apparently normal children. Five children in whom DGS occurred in association with brain abnormalities underwent long-term observation, which indicated persistence of the deficits. The identification of these cases suggests that DGS may not be as rare as previously thought and may often be unrecognized. Testing for the Gerstmann elements in learning-disabled children may identify otherwise undiagnosed cases of DGS and should be routinely employed in the neurologic examination. Until appropriate teaching methods for DGS are found, "bypassing" the deficits and utilizing the child's strengths, plus counseling, seem to offer an effective treatment approach.     

Is There a Syndrome of Tuberothalamic Artery Infarction? A Case Report and Critical Review

Short-term post-acute neuropsychological, neurological, and neuroradiological test results and a 16-month follow-up of a 65-year-old patient with a right hemisphere ischemic lesion in the tuberothalamic area of vascular supply are reported. During a 6-week period of examinations the originally left- but trained right-handed patient exhibited fluctuating neuropsychological disorders including aphasia, visuo-perceptive and visuoconstructive disorders, and memory and attention deficits. In the follow-up examination the patient exhibited no aphasia and significant improvements in most neuropsychological tasks. Based on three-dimensional reconstruction of MRI, lesion topography and involvement of thalamic nuclei were established. We discuss the neuropsychological and neurological symptoms of the present case against the background of the ‘syndrome of unilateral tuberothalamic artery territory infarction’ proposed by Bogousslavsky and coworkers (1986) and the neuropsychological literature on unilateral ischemic anterior/anterolateral thalamic infarction.     

DOES ALEXIA WITHOUT AGRAPHIA ALWAYS INCLUDE HEMIANOPSIA?

A patient with alexia without agraphia (pure alexia) is reported. Bedside examination of the visual fields disclosed no abnormalities, but perimetric examination demonstrated an incomplete right homonymous hemianopsia with some sparing of the peripheral part of the visual field. The literature on pure alexia without hemianopsia is reviewed and it is concluded that this syndrome has hardly been described with certainty.     

Developmental Gerstmann syndrome without aphasia in Fragile X syndrome

Considerable controversy surrounds Gerstmann's syndrome, with some authors questioning its localizing significance, and others doubting its existence as a distinct entity. This literature is reviewed, and a case is presented of a young boy with Fragile X syndrome who showed all five symptoms of the developmental Gerstmann syndrome. He was one of a group of individuals found to be cytogenetically positive for Fragile X syndrome, who were observed to possess three or more of Gerstmann's symptons in the complete absence of aphasic disorder.