A case of slowly progressive aphasia accompanied with auditory agnosia]

A 68-year-old right-handed woman was admitted to our hospital because of difficulty to speak and understand conversation over 10 years. She was able to make herself by writing. No change in her personality or behavioral abnormality was observed so that she could live without help. Although her WAIS score and auditory brain stem response were normal, she could not understand the speech or distinguish the sound. She also spoke plenty of words fluently, resulting in undifferentiated jargon. She did not make any effort in speaking. Her speech was, however, unclear and hard to understand. Brain MRI scan disclosed a moderate atrophy of bilateral temporal lobe and enlargement of Sylvius fissure. A three-dimension reconstructed brain surface image showed enlargement of the perisylvian fissure, and atrophy of the gyrus frontalis inferior, operculum, gyrus temporal superior, bilaterally. Reduced cerebral blood flow was demonstrated on 99mTc-ECD SPECT in the left thalamus and bilateral fronto-temporal lobe. A diagnosis of slowly progressive aphasia with auditory agnosia was made. Our case suggests that bilateral disturbance of neuronal network between the primary auditory area and the secondary auditory area is responsible to the consequence of auditory agnosia.     

Frontal lobe hematoma associated with transcortical motor aphasia: case report]

An 81-year-old woman with left medial frontal lobe hematoma, which involved the supplementary motor area, is reported. She was right-handed and was initially mute for several hours after the onset. On admission, she exhibited mild paresis of the right lower extremity. Her spontaneous speech was sparse and not fluent, but her articulation and auditory comprehension were normal and repetition was good. She exhibited excellent performance in the confrontation naming task, but showed difficulty in recalling words from a given category during the word fluency task. On the basis of these clinical features, she was diagnosed as having transcortical motor aphasia. Our case may corroborate previous findings that the left medial frontal lobe is important in word fluency but not in confrontation naming.     

Slowly progressive aphemia--a case report]

A 69-year-old women was admitted to Tokyo Medical and Dental University Hospital because of slowly progressive difficulty in speech production over 8-years. On admission, her spontaneous speech was non-fluent, limited to one-syllable utterance, and severely efforty. But her visual and auditory comprehension was preserved. There was no significant general intellectual deterioration. Severe buccofacial apraxia, but no swallowing was observed. So we considered her difficulty in speech as aphemia. Three-dimensionally reconstructed surface MR image clearly showed severe atrophy in the posterior part of the left inferior frontal gyrus and the lower part of the left pre-central gyrus. The FDG-PET demonstrated a focal hypometabolism in the same region. The lesion in this area was suggested to be a cause of speech production difficulty in this case.     

A case of crossed aphasia with echolalia after the resection of tumor in the right medial frontal lobe]

We report a right-handed woman, who developed a non-fluent aphasia after resection of astrocytoma (grade III) in the right medial frontal lobe. On admission to the rehabilitation department, neurological examination revealed mild left hemiparesis, hyperreflexia on the left side and grasp reflex on the left hand. Neuropsychologically she showed general inattention, non-fluent aphasia, acalculia, constructional disability, and mild buccofacial apraxia. No other apraxia, unilateral spatial neglect or extinction phenomena were observed. An MRI demonstrated resected areas in the right superior frontal gyrus, subcortical region in the right middle frontal gyrus, anterior part of the cingulate gyrus, a part of supplementary motor area. Surrounding area in the right frontal lobe showed diffuse signal change. She demonstrated non-fluent aprosodic speech with word finding difficulty. No phonemic paraphasia, or anarthria was observed. Auditory comprehension was fair with some difficulty in comprehending complex commands. Naming was good, but verbal fluency tests for a category or phonemic cuing was severely impaired. She could repeat words but not sentences. Reading comprehension was disturbed by semantic paralexia and writing words was poor for both Kana (syllabogram) and Kanji(logogram) characters. A significant feature of her speech was mitigated echolalia. In both free conversation and examination setting, she often repeated phrases spoken to her which she used to start her speech. In addition, she repeated words spoken to others which were totally irrelevant to her conversation. She was aware of her echoing, which always embarrassed her. She described her echolalic tendency as a great nuisance. However, once echoing being forbidden, she could not initiate her speech and made incorrect responses after long delay. Thus, her compulsive echolalia helped to start her speech. Only four patients with crossed aphasia demonstrated echolalia in the literature. They showed severe aphasia with markedly decreased speech and severe comprehension deficit. A patient with a similar lesion in the right medial frontal lobe had aspontaneity in general and language function per se could not be examined properly. Echolalia related to the medial frontal lesion in the language dominant hemisphere was described as a compulsive speech response, because some other 'echoing' phenomena or compulsive behavior were also observed in these patients. On the other hand, some patients with a large lesion in the right hemisphere tended to respond to stimuli directed to other patients, so called 'response-to-next-patient-stimulation'. This behavior was explained by disinhibited shift of attention or perseveration of the set. Both compulsive speech responses and 'response-to-next-patient-stimulation' like phenomena may have contributed to the echolalia phenomena of the present case.     

Partial Seizure with Aphasic Speech Arrest Caused by Watching a Popular Animated TV Program

On the evening of December 16, 1997, about 700 children across Japan were hospitalized because of convulsive seizures or vomiting experienced while watching a popular animated TV program that included blue and red stimuli that alternated at 12 flashes per second. In one case, an 11-year-old girl developed a hallucination in the right visual field and a subsequent cramp on the right side of her face, with aphasic speech arrest. She had no history of seizures. Her electroencephalogram (EEG) showed normal background activity and no epileptiform discharges. Intermittent photic stimulation provoked a photoparoxysmal response. Her main clinical manifestation was a TV-induced left occipital lobe seizure spreading toward the left inferior frontal lobe. This suggested a functional link from the occipital lobe to the frontal operculum.     

Symptomatology and Neuropathology of patients presenting with focal cortical signs

Here, we describe two patients who presented with focal cortical signs and underwent neuropathological examination. Case 1 was a 73-year-old woman with progressive speech disorder and abnormal behavior. She showed agraphia of the frontal lobe type, featured by the omission of kana letters when writing, other than pyramidal tract signs, pseudobulbar palsy, and frontal lobe dementia. Neuropathological examination, including TAR DNA-binding protein 43 (TDP-43) immunohistochemistry, revealed bilateral frontal and anterior temporal lobe lesions accentuated in the precentral gyrus and posterior part of the middle frontal gyrus. Both upper and lower motor neurons showed pathological changes compatible with amyotrophic lateral sclerosis. Case 2 was a 62-year-old man with progressive speech disorder and hand clumsiness. He had a motor speech disorder, compatible with apraxia of speech, and limb apraxia of the limb-kinetic and ideomotor type. Neuropathological examination revealed degeneration in the left frontal lobe, including the precentral gyrus, anterior temporal, and parietal lobe cortices. Moreover, numerous argyrophilic neuronal intracytoplasmic inclusions (Pick body) and ballooned neurons were observed in these lesions and the limbic system. The pathological diagnosis was Pick disease involving the peri-Rolandic area and parietal lobe. In these two cases, the distribution of neuropathological changes in the cerebral cortices correlated with the clinical symptoms observed.     

Kinesthetic alexia due to left parietal lobe lesions

To investigate the neuropsychological mechanisms of kinesthetic alexia, we asked 7 patients who showed kinesthetic alexia with preserved visual reading after damage to the left parietal region to perform tasks consisting of kinesthetic written reproduction (writing down the same letter as the kinesthetic stimulus), kinesthetic reading aloud, visual written reproduction (copying letters), and visual reading aloud of hiragana (Japanese phonograms). We compared the performance in these tasks and the lesion sites in each patient. The results suggested that deficits in any one of the following functions might cause kinesthetic alexia: (1) the retrieval of kinesthetic images (motor engrams) of characters from kinesthetic stimuli, (2) kinesthetic images themselves, (3) access to cross-modal association from kinesthetic images, and (4) cross-modal association itself (retrieval of auditory and visual images from kinesthetic images of characters). Each of these factors seemed to be related to different lesion sites in the left parietal lobe.     

Reading speech from still and moving faces: the neural substrates of visible speech

Speech is perceived both by ear and by eye. Unlike heard speech, some seen speech gestures can be captured in stilled image sequences. Previous studies have shown that in hearing people, natural time-varying silent seen speech can access the auditory cortex (left superior temporal regions). Using functional magnetic resonance imaging (fMRI), the present study explored the extent to which this circuitry was activated when seen speech was deprived of its time-varying characteristics. In the scanner, hearing participants were instructed to look for a prespecified visible speech target sequence ("voo" or "ahv") among other monosyllables. In one condition, the image sequence comprised a series of stilled key frames showing apical gestures (e.g., separate frames for "v" and "oo" [from the target] or "ee" and "m" [i.e., from nontarget syllables]). In the other condition, natural speech movement of the same overall segment duration was seen. In contrast to a baseline condition in which the letter "V" was superimposed on a resting face, stilled speech face images generated activation in posterior cortical regions associated with the perception of biological movement, despite the lack of apparent movement in the speech image sequence. Activation was also detected in traditional speech-processing regions including the left inferior frontal (Broca's) area, left superior temporal sulcus (STS), and left supramarginal gyrus (the dorsal aspect of Wernicke's area). Stilled speech sequences also generated activation in the ventral premotor cortex and anterior inferior parietal sulcus bilaterally. Moving faces generated significantly greater cortical activation than stilled face sequences, and in similar regions. However, a number of differences between stilled and moving speech were also observed. In the visual cortex, stilled faces generated relatively more activation in primary visual regions (V1/V2), while visual movement areas (V5/MT+) were activated to a greater extent by moving faces. Cortical regions activated more by naturally moving speaking faces included the auditory cortex (Brodmann's Areas 41/42; lateral parts of Heschl's gyrus) and the left STS and inferior frontal gyrus. Seen speech with normal time-varying characteristics appears to have preferential access to "purely" auditory processing regions specialized for language, possibly via acquired dynamic audiovisual integration mechanisms in STS. When seen speech lacks natural time-varying characteristics, access to speech-processing systems in the left temporal lobe may be achieved predominantly via action-based speech representations, realized in the ventral premotor cortex.     

An autopsy case of transcortical motor aphasia

An autopsy case of transcortical motor aphasia is presented with a pathology located anterior and superior to the pars opercularis of the left inferior frontal gyrus. Case H. Y. A 60-year-old right-handed man. On Nov. 14, 1978, the patient had surgery to remove cerebral hematoma in the left frontal lobe. In the neuropsychological examination before the operation, he had shown the clinical features of transcortical motor aphasia characterized by good comprehension of language, preserved repetition, and spontaneous speech disorder. In this stage, it was supposed that the underlying disturbance of spontaneous speech was due to the disabilities of contextual constructions of sentences rather than the lack of speech initiation. Following the operation, however, spontaneous speech disappeared completely for several days. At the same time, the patient showed problems in comprehension, reading, writing and confrontation naming as well as symptoms of disorientation, pathological inertia and 'loss of initiation' in the psychomotor domain. During the following three months, however, the patient did show slight improvement, except for contextual sentence constructions and pathological inertia when taking the complex animal drawing test. In his terminal stages, the clinical symptoms could be summarized as transcortical motor aphasia and mild frontal lobe syndrome. On March 1, 1979, the patient died of Hamman-Rich syndrome. Postmortem examination: The brain weighed 1294 gm. The external observation of the brain disclosed the linear tissue defect, about 15 mm in length and 10 mm in width, along the radial sulcus of the pars triangularis of the left inferior frontal gyrus.(ABSTRACT TRUNCATED AT 250 WORDS)     

Familial antithrombin III abnormality accompanied with progressing ischemic stroke

We report a case of familial antithrombin III (AT-III) abnormality accompanied with progressing ischemic stroke. The patient was a 31-year-old female who developed consciousness disturbance and left hemiparesis on December 1 in 1987. She had a history of two transient ischemic attacks and three episodes of thrombophlebitis of the extremities. Cerebral CT scan showed a low density area on the right temporal lobe that had extended to the right parietal and on the left frontal lobe as clinical symptoms worsened. Cerebral angiogram revealed branch occlusions of the right middle cerebral artery and showed no cerebral venous and sinus obstruction. When her symptoms had been progressing to show semi-comatose state, left hemiplegia and transient Cheyne-Stokes respiration, we found her decreased biological activity and normal immunological level of AT-III. The diagnosis of familial AT-III abnormality had been made by familial investigation. As the treatment of AT-III concentrates transfusion was started from the third day, her symptoms gradually recovered and the low density area stopped extending. Further examinations revealed that she was a homozygote of AT-III abnormality presenting no affinity for heparin and that her parents were heterozygotes. It was suggested that the homozygous AT-III abnormality was the main cause of her progressing ischemic stroke.     

Hallucinations and Reversed Cerebral Dominance in Mesial Temporal Sclerosis

The localizing value of experiential phenomena in temporal and occipital lobe epilepsy has become increasingly elucidated. We describe complex visual and auditory hallucinations in a right-handed adolescent and review the localization value of ictal visual and auditory auras in partial epilepsy. A 15-year-old right-handed girl with 2 previous secondarily generalized seizures manifested a new semiology of complex visual and auditory hallucinations, characterized by seeing a school bus full of children and then hearing a male voice tell her to “feed the children.” Feeling compelled, she “fed” the children, and they reboarded the bus and the bus drove away. Video electroencephalogram monitoring demonstrated fluent language during her seizures. Magnetic resonance imaging was compatible with left mesial temporal sclerosis. Fluorodeoxyglucose positron emission tomography demonstrated left temporal hypometabolism. An ictal single-photon emission computed tomography study demonstrated left anterior temporal hyperperfusion; Wada testing revealed reversed cerebral dominance. The patient underwent left anterior temporal lobectomy without complication and remains seizure-free. Complex auditory and visual hallucinations can occur in occipitotemporal and anteromedial temporal epilepsy. Reversed cerebral dominance is more common in children than adults and should be considered in any dextral person with fluent ictal speech with a left-sided epileptogenic lesion.     

A case of Gogi aphasia with predominantly left temporal and parietal lobe atrophy]

We report a 68-year-old right-handed Japanese woman who had a history of progressive difficulty in understanding speech and naming. Neuropsychological examination presented Gogi (word meaning) aphasia and impairment of semantic memory for some common objects. She also presented acalculia and mild constructional impairment. There was no evidence of impairment in elementary perception and motor skills. Her memory performance of visual task was within normal range. She had neither personality change nor behavioral disorder. Magnetic resonance (MR) imaging showed atrophy in the left temporal lobe and the left parietal lobe. Single photon emission computed tomography (SPECT) scans demonstrated a decrease of regional cerebral blood flow in the atrophic sites and the left frontal lobe. We pointed out that the atrophy of the parietal lobe was atypical in the early stage of cortical degenerative disease presenting Gogi aphasia, in addition to in the light of classification of Fronto-Temporal Lobar Degeneration (FTLD).     

Cerebral embolism following trivial trauma in children--report of three cases]

Three cases of cerebral embolism secondary to trivial trauma are reported. Case 1: A 12-year-old male suffered a severe headache followed by a generalized convulsion after he turned his head when he was flying a kite. A neurological examination on admission demonstrated right hemiparesis and aphasia. A CT revealed a low density in the left putamen, temporal lobe and frontal lobe. Left carotid angiography (CAG) showed irregular narrowing of the internal carotid with an embolic occlusion and narrowing of the middle cerebral artery with the intraluminal presence of emboli both in the anterior and middle cerebral arteries. He is now doing well but has right hemiparesis. Case 2: This 6-year-old female could not grasp chopsticks and had neck pain 10 minutes after being pulled up by the right arm by her father. Neurological examination demonstrated a right hemiparesis and aphasia. A CT scan and magnetic resonance imaging (MRI) of the head showed an infarcted area in the left caudate head, anterior limb of the internal capsule and putamen. Left CAG revealed an obstruction of the trunk of the middle cerebral artery. She has slight weakness in her right extremities. Case 3: This 11-year-old female noted a weakness in her left lower limb soon after her hair was pulled backward. On admission, a neurological examination failed to demonstrate any abnormality. CT showed an ill defined low density lesion in the right putamen. MRI revealed a high intensity lesion in a T2 weighted image. Right CAG showed an irregularity of the arterial wall in the cavernous portion of the right internal carotid artery.(ABSTRACT TRUNCATED AT 250 WORDS)     

Repetitive transcranial magnetic stimulation of the dominant hemisphere can disrupt visual naming in temporal lobe epilepsy patients.

We used repetitive transcranial magnetic stimulation (rTMS) to study visual naming in 14 patients with temporal lobe epilepsy. Ten had left hemisphere language by Wada testing and all experienced speech arrest with rTMS of the motor speech area in the left frontal lobe. One left-hander had speech arrest with stimulation of sites on both sides. Subjects were asked to name pictures or read words presented on a computer monitor. rTMS was delivered on half of the trials. Stimulation sites were the motor speech area in the left frontal lobe, the mirror site on the right, and the left and right mid superior and posterior temporal lobes. rTMS at left hemisphere sites caused more naming errors than did right hemisphere rTMS. All individual subjects, except two who had temporal lobe resections and the one with bilateral speech arrest, produced more naming errors with rTMS of left hemisphere sites. There was no significant effect on word reading. rTMS at the left hemisphere and right frontal sites produced reductions in reaction time for picture naming, but not for word reading. This was observed for both correct and incorrect responses. This study shows that left hemisphere rTMS can disrupt visual naming selectively.     

A Chance SPECT Study of Ictal Aphasia During Simple Partial Seizures

Summary: We report obtaining an ictal single photon emission computed tomographic (SPECT) scan in a right-handed 51-year-old man who had an astrocytoma in the left cerebral hemisphere and simple partial seizures characterized by aphasia. An epileptic seizure producing loss of speech and right-sided facial twitching occurred by chance during a SPECT scan. During the attack, he was unable to speak, but auditory comprehension and writing were intact. Ictal SPECT showed an area of increased perfusion in the left frontal cortex, with the area of highest perfusion involving the left frontal operculum to the inferior part of the left precentral gyrus. Interictal SPECT showed hypoperfusion in the same area. These SPECT findings suggest that the frontal operculum of the dominant hemisphere is one of the regions that can give rise to epileptic aphasia.     

原发性进行性失语的临床、影像及语言特征

目的 探讨原发性进行性失语（PPA）的临床、影像及语言特征.方法 PPA患者7例,其中语义性痴呆（SD）6例,进行性非流利性失语（PNFA）1例,收集患者的人口学资料、病史,进行头MRI检查,采用汉语失语成套测验进行语言评估.结果 患者平均发病年龄56岁,均缓慢起病,以语言表达、命名障碍为首发症状.MRI示左侧颞极萎缩为主,病程长的患者左侧额叶和顶叶、右侧颞叶也明显萎缩.语言评估发现所有患者的自发语言、复述、命名、听理解、阅读和书写均不同程度损害.SD患者言语流利,复述、朗读能力下降相对较轻,命名、复杂语句的理解能力损害突出.PNFA言语顿挫吃力,患者列名能力明显下降,但命名相对保存完好.结论 PPA多为老年前期发病,语言障碍为最早、最突出的症状.MRI特征性的改变为额叶和颞极萎缩,左侧为著.其中SD表现为命名性失语和经皮质感觉性失语,PNFA表现为经皮质运动性失语的特征.     

A case of pure agraphia with a deficit of drawing]

We examined a drawing ability in a case with pure agraphia. The patient, having lesions in the left middle frontal gyrus and inferior parietal lobule, could not write in both dictation and spontaneous writing tasks, with the ability to copy letters being well spared. Correspondingly, he showed a deficit of spontaneous drawing, with the ability to copy complex figures being well spared. These findings suggest that he could have an impairment of retrieving visual image, not restricted to letter image. Although some previous cases with pure agraphia were attributed to an impairment of retrieving visual letter image, drawing ability which seemed to be related to retrieving visual image had not been investigated. In the present case, disability of both writing and drawing could be attributed to a common mechanism, that was an impairment of retrieving visual image. In addition, the patient did not show a significant difference between the performance on Kana writing and on Kanji writing. But it has been said that an impairment of retrieving visual letter image could be related to a deficit of Kanji writing more than of Kana writing. Then, it could be assumed that the mechanism of Kana writing involved not only the pathway depending on graphical motor pattern but one depending on visual image.     

A case of crossed aphasia in a dextral patient with polycythemia]

We report a 72-year-old right-handed man who was diagnosed as having crossed aphasia. He had polycythemia, hypertension and an old cerebral infarction in the right occipital lobe. He was admitted to our hospital because of muscle weakness in the left extremities at the age of 71. In the laboratory data, red blood cells(689 x 10(4)/microliter) and platelets(87.6 x 10(4)/microliter) were increased in number. Brain CT detected a right putaminal hematoma and an old infarct in the occipital lobe on the right. After admission, he developed non-fluent speech, and impairments of auditory comprehension, writing and naming due to the infarction in his right cerebral hemisphere including the middle cerebral artery distribution. The standard language test of aphasia(SLTA) revealed marked impairments in the language function, except for reading kana and kanji words. This writing was severely impaired compared with other language dysfunctions. Auditory comprehension, repetition and reading were impaired at the sentence level rather than at the kana word level. Furthermore, he suffered from left hemiparesis and left unilateral spatial neglect. We diagnosed his impairments of language function as crossed aphasia based on his right-handedness, CT findings and the results of SLTA. His language center was considered to be located in both cerebral hemispheres. Compared with typical findings in reported cases of crossed aphasia, the presence of both non-fluent speech and mutism were consistent with previous observations. However, the marked impairments of auditory comprehension, repetition and naming were different. Polycythemia and hypertension were considered to be the risk factors of cerebral infarction in our patient.     

Hyperlexia in an adult patient with lesions in the left medial frontal lobe]

A 69-year-old right-handed woman developed a transcortical motor aphasia with hyperlexia following resection of a glioma in the left medial frontal lobe. Neurological examination revealed grasp reflex in the right hand and underutilization of the right upper extremity. An MRI demonstrated lesions in the left medial frontal lobe including the supplementary motor area and the anterior part of the cingulate gyrus, which extended to the anterior part of the body of corpus callosum. Neuropsychologically she was alert and cooperative. She demonstrated transcortical motor aphasia. Her verbal output began with echolalia. Furthermore hyperlexia was observed in daily activities and during examinations. During conversation she suddenly read words written on objects around her which were totally irrelevant to the talk. When she was walking in the ward with an examiner she read words written on a trash bag that passed by and signboards which indicated a name of a room. Her conversation while walking was intermingled with reading words, which was irrelevant to the conversation. She also read time on analog clocks, which were hung on a wall in a watch store. In a naming task, she read words written on objects first and named them upon repeated question about their names. When an examiner opened a newspaper in front of her without any instructions she began reading until the examiner prohibited it. Then she began reading again when an examiner turned the page, although she remembered that she should not read it aloud. She showed mild ideomotor apraxia of a left hand. Utilization behavior, imitation behavior, hypergraphia, or compulsive use of objects was not observed throughout the course. Hyperlexic tendency is a prominent feature of this patient's language output. Hyperlexia was often reported in children with pervasive developmental disorders including autism. There are only a few reports about hyperlexia in adults and some of them were related to diffuse brain dysfunction. Hyperlexia of our patient was associated with echolalia but not with the other "echo" phenomena, which may be because the lesion was unilateral on the left side. Dysfunction of the left supplementary motor area could lead to disinhibition of regulatory mechanism of verbal output in response to auditory and visual stimuli.     

Slowly progressive dysarthria and impaired language function--a case report]

A 68-year-old right-handed woman was admitted to Tokyo Metropolitan Geriatric Hospital because of slowly progressive dysarthria and writing disability over 2-year period. On admission, severe dysarthria was observed, but no dysphagia. The dysarthria mostly resembled a type of pseudobulbar palsy, although it was associated with effortful speech production. An oro-facial apraxia was also found. She could name objects, and could understand spoken words correctly. Examination using the Western Aphasia Battery showed diminution of word fluency, impaired repetition and perseveration and writing errors. On the Wechsler Adult Intelligence Scale-R verbal IQ was 100 and performance IQ was 87. These scores did not suggest any significant degree of general intellectual deterioration. Wisconsin card sorting test disclosed mild frontal dysfunction. Magnetic resonance imaging showed cortical atrophy in the bilateral frontal and temporal lobes. Measurements of regional cerebral metabolic rate by 18F-FDG-PET demonstrated decreased uptake in the latero-dorso-inferior area of the bilateral frontal lobes, especially on the left side. The present case showed slowly progressive dysarthria and progressive aphasia without generalized dementia, and without typical aphasia. These symptoms are speculated to be related to the atrophy in the bilateral frontal and temporal lobes shown by MRI and the decreased metabolic rate in the left dominant bilateral frontal lobes on PET study. The pathologic process responsible for these lesions remains obscure.