Gerstmann syndrome that was caused by a traumatic origin was very rare. We have reported a case of typical Gerstmann syndrome which was caused by a localized head injury. The case showed finger agnosia, right-left disorientation, acalculia and dysgraphia. And also the case revealed amnesic dysphasia and autotopagnosia. The lesion was an egg-shell typed depressed fracture(4 cm in diameter) of the left inferior parietal bone associated with the intracerebral hematoma in the lower parietal lobe which seemed to be the transitional area of the angular gyrus and the second occipital convolution.
Agnosia ; Agraphia ; Aphasia ; Craniocerebral Trauma ; Dyscalculia ; Gerstmann Syndrome* ; Hematoma ; Parietal Bone ; Parietal Lobe

Gerstmann's syndrome, assigned to a lesion of the dominant parietal lobe, is a neurological disorder characterized by acalculia, agraphia, right-left disorientation and finger agnosia. Some studies report that these symptoms are also shown in other brain lesions. We report two patients who presented with this tetrad of symptoms in initial assessment. Their Brain MRI images both showed lesion of left frontal lobe. Over time, these symptoms became better but some still remained in last assessment. Accordingly, we suggest that a left frontal lesion cause Gerstmann's syndrome.
Agnosia ; Agraphia ; Brain ; Dyscalculia ; Frontal Lobe* ; Gerstmann Syndrome* ; Humans ; Magnetic Resonance Imaging ; Nervous System Diseases ; Parietal Lobe

BACKGROUND: Semantic dementia (SD) is a temporal variant of frontotemporal lobar degeneration (FTLD), which is characterized by naming difficulty, decreased comprehension of words, prosopagnosia and object visual agnosia. We report clinical features, neuropsychological and neuroimaging findings of 4 patients with SD. METHODS: Of 55 patients diagnosed as having FTLD between Jan 1995 and May 2001 at Samsung Medical Center, four patients fulfilled the diagnostic criteria of SD proposed by consensus on FTLD diagnostic criteria. We investigated their clinical features such as presenting symptoms and abnormal behaviors, neuropsychological and neuroimaging findings. Neuropsychological tests included the Seoul Neuropsychological Screening Battery, the Korean-version of Western Aphasia Battery and Hanja reading and writing. All patients underwent brain MRI and FDG-PET. RESULTS: All of the patients showed naming difficulty as a presenting symptom. Language assessments showed severe naming and compre-hension difficulties with preserved fluency and repetition, which were compatible with transcortical sensory aphasia. Whereas Hangul reading aloud and writing were intact, three patients were impaired at Hanja reading and writing. Other neuropsychological tests were remarkable for prosopagnosia, object visual agnosia and memory loss. Brain MRI showed asymmetric temporal atrophies, mainly left antero-inferior temporal cortices. FDG-PET also showed hypome-tabolism in bilateral anterior temporal lobes, more severe on the left. CONCLUSIONS: Our SD patients had characteristic neuropsychological and neuroimaging findings, which can be differentiated from other neurodegenerative diseases. We also found Hanja alexia and agraphia in SD patients, which has not been reported yet.
Agnosia ; Agraphia ; Aphasia ; Aphasia, Wernicke ; Atrophy ; Brain ; Comprehension ; Consensus ; Dyslexia ; Frontotemporal Dementia* ; Frontotemporal Lobar Degeneration ; Humans ; Magnetic Resonance Imaging ; Mass Screening ; Memory Disorders ; Neurodegenerative Diseases ; Neuroimaging* ; Neuropsychological Tests ; Prosopagnosia ; Semantics* ; Seoul ; Temporal Lobe ; Writing

We report a case of 53-year-old woman with personification following right middle cerebral artery territory infarction. Although she knew that her paralyzed left limbs belonged to her, she behaved as if it is a separate person. Verbalization of hatred toward her left arm was frequently observed. She attributed her neurologic deficit to the left arm instead of herself, frequently complaining in a fashion that 'I(a person except for the left arm) am all right but he/she(left arm) is wrong'. This delusional beliefs were mainly confined to her paralysed left limb, and were accompanied by left spatial neglect, transient anosognosia for hemiplegia and right-left disorientation.
Agnosia ; Arm ; Delusions ; Extremities* ; Female ; Hemiplegia ; Humans ; Infarction ; Middle Aged ; Middle Cerebral Artery ; Neurologic Manifestations ; Stroke*

Optic aphasia is a rare syndrome in which patients are unable to name visually presented objects but have no difficulty in naming those objects on tactile or verbal presentation. We report a 79-year-old man who exhibited anomic aphasia after a left posterior cerebral artery territory infarction. His naming ability was intact on tactile and verbal semantic presentation. The results of the systematic assessment of visual processing of objects and letters indicated that he had optic aphasia with mixed features of visual associative agnosia. Interestingly, although he had difficulty reading Hanja (an ideogram), he could point to Hanja letters on verbal description of their meaning, suggesting that the processes of recognizing objects and Hanja share a common mechanism.
Aged ; Agnosia ; Anomia ; Aphasia* ; Dyslexia ; Humans ; Infarction ; Posterior Cerebral Artery ; Semantics

OBJECTIVES: Clinical studies have shown cognitive dysfunctions and soft neurological signs in schizophrenic patients and these findings have been suggested as evidence of organic bases in the pathophysiology of schizophrenia. This study was intended to investigate the characteristics of cognitive deficits and soft neurological signs in schizophrenia and, to determine whether any abnormality in these functions can be regarded as a trait marker of the illness which is independent of antipsychotic treatment and clinical improvement. We also investigated the correlation between cognitive deficits and soft neurological signs reflecting cognitive dysmetria, respectively. METHODS: Twenty schizophrenic patients were assessed for the soft neurological signs and cognitive functions before and after neuroleptic treatment. The patients had been medicated for at least 3 weeks with one of the atypical neuroleptics. Cognitive functions were evaluated by Trail making A, B, Stroop test and Word fluency test. Soft neurological signs were assessed by Cambridge Neurological Inventory(Part 2). Positive and Negative Syndrome Scale(PANSS) and Clinical Global Impression(CGI) were used to assess the clinical severity and Extrapyramidal Symtoms Rating Scale was used to estimate the extrapyramidal symptoms. Cognitive functions and soft neurological signs of twenty normal controls were assessed with the same scale. RESULTS: Before treatment, schizophrenic patients showed significant impairments on cognitive function tests(Trail Making A, B, Stroop Test) and soft neurological sign tests(8 items:Grasp reflex, Go/no-go, Finger thumb opposition, Rhythm tapping, Finger agnosia, Fist-edge-palm, Left-right orientation, Extinction). Although significant clinical improvements were observed after the treatment, there were no significant changes in cognitive functions and soft neurological signs(except for Go/no-go test and Finger agnosia). Among the soft neurological signs of the patients, abnormality in Rhythm tapping was significantly correlated with cognitive deficits. CONCLUSION: Schizophrenic patients showed characteristic cognitive deficits and soft neurological signs which were independent of medication and clinical symptoms. And these two characteristics were partly correlated with each other.
Agnosia ; Antipsychotic Agents ; Cerebellar Ataxia ; Fingers ; Humans ; Reflex ; Schizophrenia ; Stroop Test ; Thumb

Landau-Kleffner syndrome, or acquired epileptiform aphasia, is an epilepsy syndrome with variable disruption of acquired language and epileptiform discharges on electroencephalograph (EEG). Auditory agnosia can deteriorate into total unresponsiveness and impaired expressive communication. In spite of the presence of this condition, the pure tone audiometry, otoacoustic emission and brainstem evoked auditory potential could be normal. Therefore, in the above hearing tests which are the mainstream methods for pediatric hearing evaluation, otolaryngologists should have suspicion for the presence of this syndrome, although it is rare.
Agnosia ; Audiometry ; Brain Stem ; Epilepsy ; Hearing ; Hearing Tests ; Landau-Kleffner Syndrome*

Frontotemporal lobar degeneration (FTLD) is a progressive dementia with prominent neuropsychiatric features, aphasia or both. FTLD predominantly affects the frontal and anterior part of temporal cortex. FTLD is classified into frontotemporal dementia (FTD), progressive nonfluent aphasia (PA), and semantic dementia (SD). FTLD is estimated to account for 20% of cases of degenerative dementia with presenile onset. This disease typically has onset in the mid- or early fifties. FTD is characterized by behavioral change and executive dysfunction, PA features a progressive nonfluent aphasia. SD is characterized by a progressive semantic aphasia and associative agnosia. Structural imaging shows atrophy of the frontal lobe and the anterior portion of the temporal lobe, bilaterally symmetric or asymmetric. Pathologically, FTLD can be classified into tau-positive pathology, tau-negative, ubiquitin positive pathology, dementia lacking distinctive histology. At present, there are no specific pharmacological therapies approved for use in any of the FTLD syndrome.
Agnosia ; Aphasia ; Atrophy ; Dementia ; Frontal Lobe ; Frontotemporal Dementia ; Frontotemporal Lobar Degeneration* ; Pathology ; Primary Progressive Nonfluent Aphasia ; Temporal Lobe ; Ubiquitin

The mechanisms and functional anatomy underlying the early stages of speech perception are still not well understood. Auditory agnosia is a deficit of auditory object processing defined as a disability to recognize spoken languages and/or nonverbal environmental sounds and music despite adequate hearing while spontaneous speech, reading and writing are preserved. Usually, either the bilateral or unilateral temporal lobe, especially the transverse gyral lesions, are responsible for auditory agnosia. Subcortical lesions without cortical damage rarely causes auditory agnosia. We present a 73-year-old right-handed male with generalized auditory agnosia caused by a unilateral subcortical lesion. He was not able to repeat or dictate but to perform fluent and comprehensible speech. He could understand and read written words and phrases. His auditory brainstem evoked potential and audiometry were intact. This case suggested that the subcortical lesion involving unilateral acoustic radiation could cause generalized auditory agnosia.
Acoustics ; Agnosia ; Audiometry ; Brain ; Brain Stem ; Evoked Potentials ; Hearing ; Humans ; Male ; Music ; Speech Perception ; Temporal Lobe ; Writing

Auditory agnoia is defined as a disability to recognize spoken languages and/or nonverbal environmental sounds and music despite adequate hearing while spontaneous speech, reading and writing are preserved. Usually, either bilateral or unilateral temporal lobe especially transverse gyral lesions are responsible for auditory agnosia. Subcortical lesions without cortical damage else rarely causes auditory agnosia. We pesent a 65-year-old right-handed male with generalized auditory agnosia caused by bilateral subcorcal lesions. After the two attacks of stroke, he could not understand and recognize spoken words and meet of non verbal sounds. Repetition and dictation were impossible but spontaneous speech was fluent and comprehensible even though mild paraphasic error was noted. Naming was normal. He could understand and read aloud the written words and phrases. Clinical examination of the ears was normal as were audiometry and brainstem auditory evoked potential. Brain magnetic resonance images showed a large cavitary lesion due to an old intracerebral hematoma in right subcortical temporoparietal lobe with preserved superior temporal gyrus and a slit like subcortical lesion in the left insula.
Aged ; Agnosia* ; Audiometry ; Brain ; Ear ; Evoked Potentials, Auditory, Brain Stem ; Hearing ; Hematoma ; Humans ; Male ; Music ; Stroke ; Temporal Lobe ; Writing