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.     

Broca aphasia with neologisms]

Patients with jargon aphasia generally have fluent speech with poor comprehension. However, outstanding jargons may appear in non-fluent aphasics. We report a 69-year-old left-handed woman with non-fluent jargon aphasia due to lesions in the right frontoparietal area. Features of her speech included non-fluent meaningless sequences of syllables, i. e., phonetic jargon, which was obvious in all the tasks including spontaneous speech, repetition, naming and reading. Her utterance was sparse, but not effortful or anarthric. She understood most of spoken single words, but was confused by complex sentences. Brain CTs revealed acute lesions affecting the inferior and middle frontal gyri, insular cortex, precentral and postcentral gyri, and a part of the angular gyrus in the right hemisphere. Old infarcts were also noted in the right frontal pole and prefrontal area. The unique feature of her language impairment suggested somewhat deviated lateralization and localization of her language function.     

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.     

Jargonagraphia in a left-handed aphasia due to a left hemisphere lesion]

We report a case who developed jargonagraphia, severe aphasia and unilateral spatial neglect due to cerebral infarction in the left hemisphere. The patient was a 57 year-old left-handed woman. She suddenly developed hemiplegia on the right side and aphasia, and was admitted to our hospital. Neuropsychological examination showed non-fluent spontaneous speech. Repetition, reading and auditory comprehension were impaired. Writing was severely impaired and she showed paragraphia without meaning (jargonagraphia). Right unilateral spatial neglect and buccofacial apraxia were noted, but neither ideomotor nor ideational apraxia was observed. The mechanisms of jargonagraphia remain unknown. The localization of language function in this patient is not a mirror image of same function in dextral aphasia. Her condition was considered that free-running on motor engrams of characters stored in the right hemisphere caused jargonagraphia.     

Corticobasal degeneration presenting with progressive conduction aphasia

We report the case of a woman with primary progressive aphasia (PPA) presenting with conduction aphasia. Neurological findings showed bilateral finger tremor and signe de poignet figé in her right hand. Memory, orientation, and activities of daily living were well preserved. Linguistic examination showed severe impairment in repetition, fluent spontaneous speech with phonemic paraphasia, and relatively well preserved comprehension. Limb-kinetic apraxia and parkinsonism were not observed during the course of her illness. T1-weighted magnetic resonance imaging revealed severe atrophy of the left temporal lobe and dilatation of the left Sylvian fissure. Neuropathological findings demonstrated the most severe atrophy in the left superior temporal gyrus and Gallyas–Braak-positive or phosphorylated tau-immunoreactive cytoskeletal structures, which were consistent with corticobasal degeneration (CBD). We speculate that the progressive conduction aphasia of our patient might have been caused by left temporal lobe impairment. We suggest that progressive conduction aphasia may be a feature of CBD presenting with PPA.     

Ambient echolalia in a patient with germinoma around the bilateral ventriculus lateralis: a case report

Ambient echolalia is a rare condition with few reported cases. We report the case of a 20-year-old man with a germinoma around the bilateral ventriculus lateralis who exhibited ambient echolalia. Clinical features included instinctive grasp reaction and compulsive manipulation of tools in his right hand. Speech or mental deterioration has been cited as a cause of ambient echolalia, but neither dementia nor aphasia was present. We propose that ambient echolalia in our case could be interpreted as a disinhibition of pre-existing essentially intact motor subroutines due to damage of the medial frontal lobe.     

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.     

Clinical-anatomical correlation in a selective phonemic speech production impairment

Although phonemic paraphasias are common in aphasic disorders, including Broca's aphasia, conduction aphasia and transcortical motor aphasia, selective phonemic speech production impairment, or phonemic disintegration, is unusual. A patient with a selective phonemic speech production disorder underwent clinical, neuropsychological and structural neuroradiological assessment over a period of 6 years. The disorder was characterised by phonemic paraphasias (phonemic disintegration) with preserved comprehension and naming. Imaging showed a focal lesion in the white matter of the left precentral gyrus and, to a lesser extent, the posterior part of the left middle frontal gyrus, with overlying cortical atrophy. Biopsy of the lesion, after several years of observation, showed a calcified haemangioma. Clinical-anatomical correlation in this case suggests the importance of primary motor cortex of the inferior precentral (pre-Rolandic) gyrus and subjacent white matter in phoneme production, with sparing of the posterior inferior frontal gyrus (Broca's area).     

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.     

Transcortical aphasia and echolalia; problems of speech initiative]

Transcortical aphasia accompanied by echolalia occurs with malacias involving the postero-median part of the frontal lobe which includes the supplementary motor field of Penfield and is nourished by the anterior cerebral artery. The syndrome manifests itself in such cases even in fine detials in the same form as does in Pick's atrophy. The same also holds true for cases in which a tumour involves the region mentioned. Sentences or fragments of sentences are echolalised; tendency to perseveration is very marked. It is hardly, if at all, possible to evaluate the verbal understanding of these patients. Analysis of their behaviour supports the assumption that they have not lost the adaptation to some situations. Echolalia is often associated with forced grasping and other compulsory phenomena. Therefore, it may be interpreted as a sign of disinhibition of the acusticomotor reflex present during the development of the speech. Competition between the intentionality and the appearance of compulsory phenomena greatly depends on the general condition of the patient, particularly on the clarity of consciousness. The integrity of the postero-median part of the frontal lobe is indespensable for a normal reaction by speech to stimuli received from the sensory areas. The influence of the supplementary motor field on speech intention seems to be linked to the dominant hemisphere. In case lesions of the territory of the anterior cerebral artery and the cortico-bulbar neuron system are coexisting in the dominant hemisphere, the speech disturbance shifts to complete motor aphasia. In such cases the pathomechanism is analogous to that of the syndrome of Liepmann, i.e., right-sided hemiparesis with left-sided apraxia. So-called transcortical motor aphasia without echolalia can be caused by loss of stimuli from the sensory fields.     

Case of Moyamoya disease with agraphia caused by a frontal lesion

A 45-year-old woman showed agraphia and decreasing spontaneity. She was diagnosed as Moyamoya disease by cerebral angiography. CT scans revealed the abnormal low density area in the left frontal lobe, and the regional cerebral blood flow study showed low perfusion in the bilateral frontal lobe and the left high convexity area. She could copy specimens of the author's writing, but her spontaneous writing and dictation were impaired with either hand. The form of each letter she wrote was not so distorted, but wrong letters were substituted for the correct ones. Errors in Kana-writing were more common than in Kanji. Although her spontaneous speech was diminished, auditory comprehension, reading and constructional abilities were unaffected. She exhibited no agnosia and apraxia. Agraphia in this case was caused by a disconnection between the visual image and auditory image and/or between the visual image and kinesthetic image. Inner speech (Luria, AR et al, 1968) in this case might be disturbed, and decreasing spontaneity and delay of response resulted from the frontal lesion. The writing process may be constituted of many factors; images of visual, auditory and kinesthetic as well as primary functions of sensory and motor. Furthermore, it may be necessary for normal writing that one's inner speech, activities and intention of writing behavior are intact.     

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.     

Atypical clinical features associated with mixed pathology in a case of non-fluent variant primary progressive aphasia

A 66-year-old woman presented with agrammatism and apraxia of speech, meeting criteria for non-fluent/agrammatic variant primary progressive aphasia (nfvPPA). However, three years later, she developed frontal/executive, short-term phonological memory, visuospatial, and visual memory deficits suggesting involvement of multiple brain networks. Multimodal neuroimaging showed damage of both fronto-striatal and posterior brain regions. She was found to have multiple pathological processes: corticobasal degeneration (CBD), Alzheimer’s disease (AD), and TAR DNA-binding protein (TDP)-43 type A. We hypothesize that cognitive and neuroimaging findings consistent with damage to multiple brain networks, each associated with vulnerability to certain molecular disease subtypes, could indicate mixed pathology.     

Frontal Pick's Disease

The authors reported a case of frontal Pick's disease clinicopathologically. (1) Clinical course: In a 77-year-old female patient at the time of death, the disease began with inattentiveness, indifference and forgetfulness at the age of 71. She was euphoric and showed uninhibited manner towards her surroundings. Meaningless hyperactivity was not noted. The disease was progressive and she became demented and filthy. In the terminal stage, she was confined to bed and totally out of contact. “Stehende Redensarten” was not noted throughout her stay in the hospital, but a slight echoing of phrases was noted. (2) Histopathological findings: The brain weighed 980 g. In the frontal lobe, the right convexity and the left FI and bilateral orbital gyri were atrophic. In the temporal lobe, the right hippocampal gyrus was slightly atrophic with a slight fibrillary gliosis in the right T2. The right temporal pole, T2, T3 and T4 showed nerve cell loss and astrocytic proliferation. Fibrillary gliosis was noted in the hippocampal gyrus bilaterally. Pick's cells were found. (3) Histopathological changes in the cerebral cortex were widespread than was indicated grossly, being present to varying extent in non atrophic temporal areas. From the survey of the literature and from our case, it is concluded that in frontal Pick's disease “stehende Redensarten” has been reported to be less frequent than in temporal or temporo-frontal types.     

A case of conduction aphasia due to small infarction in the left parietal lobe]

This is a report on the patient with conduction aphAsia due to small infarction in the left parietal lobe. The patient is a right-handed man aged 74, who developed a speech disorder and mild paralysis of the right hand on November 13. 1996. A CT scan showed a small low-density in the supramarginal gyrus of the left parietal lobe. Standard Language Test of aphasia (SLTA) conducted at five days after admission to the hospital showed preserved auditory comprehension and phonemic para-aphasia symptoms with respect to volitional speech, naming, reading aloud and repetition. Frequent self-correction was also observed while repetition was not remarkably impaired. A test at three months after the onset revealed generally fluent speech, while there still remained occasional phonemic para-aphasia and self-correction for the speech disruption. Three years and four months later, most of the aphasic syndromes disappeared, although the patient claimed he still had difficulty in speaking. This case suggests that conduction aphasia can be caused by a lesion, though small, located in arcuate fibers of the cerebrum. The characteristics are phonemic para-aphasia with respect to general speech functions as well as self-correction toward target words. The indicated that lesions in the pathway connecting Broca's area and Wernicke's area causes difficulties in selecting accurate phonemes due to a malfunctioning interface between the two areas.     

Progressive supranuclear palsy with widespread cerebral lesions.

A 51-year-old woman with no history of any familial neurological diseases initially presented with numbness in her extremities, slowing of movements, comprehension deficit, memory disturbance, dyscalculia, muscle rigidity, hyperreflexia, Parkinsonian gait, increasing disorientation, left-right disturbance, finger agnosia, alexia, acalculia, apraxia, aspontaneity, euphoria, gait disturbance, aphasia, echolalia, and in the terminal stage, mutism, contracture of lower extremities and cachexia. She died of bronchopneumonia at the age of 55. The brain showed widespread cerebral lesions, consisting of nerve cell loss and neurofibrillary tangles in the frontal, parietal and occipital cortex, demyelination and gliosis in the frontal, parietal and occipital subcortical white matter in addition to the typical pathological findings of progressive supranuclear palsy (PSP): severe neuronal loss with gliosis and neurofibrillary tangles (NFTs) in the subthalamic nucleus, globus pallidus and substantia nigra. In conclusion, we present a case of PSP with unusual clinical features (extrapyramidal signs, frontal and parietal lobe syndromes without ophthalmoplegia) and neuropathologically widespread cerebral lesions in addition to the typical pathological findings of PSP. The differential diagnosis of PSP and Alzheimer's disease and other degenerative disorders is discussed.     

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.     

Slowly progressive dressing and constructional apraxia: symptomatological study, especially for dressing apraxia]

In 1982, Mesulam drew attention to a clinical picture characterized by slowly progressive aphasia without dementia, and since then, there have been many such reports. Recently, there have been 30 reports of slowly progressive apraxia. However, the nature of this apraxia is not uniform. We now report a patient with slowly progressive dressing and constructional apraxia. The patient is a 60-year-old right-handed woman with a 2-year history of a slowly progressive praxic disturbance. On admission, she was alert and aware of this difficulty. A neurological examination disclosed mild rigidity and myoclonus in her left hand. A neuropsychological assessment disclosed severe dressing apraxia, which was unlikely to be caused by dementia and moderate constructional apraxia. Her dressing apraxia was manifested in upper limbs, neck, trunk and lower limbs. However, she could express verbally the action of dressing. She also showed mild limb-kinetic apraxia, but neither ideational apraxia nor ideomotor apraxia was present. Aphasia and agnosia were also absent. On an MRI, the bilateral cerebral hemispheres were atrophic (right > left). A 99m-Tc ECD SPECT revealed decreased uptake in the right cerebral hemisphere and left frontal lobe, and an EEG showed slow waves over the right cerebral hemisphere. There have been 30 reports of slowly progressive apraxia. Most of these cases presented with slowly progressive clumsiness in one or both hands as an initial symptom, followed by constructional, ideomotor or dressing apraxia. Our patient differed from these cases in that dressing and constructional apraxia progressed slowly without any other apraxia except only mild limb-kinetic apraxia. There was a similarity between dressing apraxia of our patient and that of Marie's and Brain's original cases.     

An epilepsy case presenting temporal aphonia and prosodic deficit with left hemiparesis]

A 53-year-old right-handed woman was admitted to a hospital with a seizure, exhibiting loss of consciousness and paroxysmal myoclonic movement. MRI showed no abnormalities of her brain. Electroencephalography revealed sporadic sharp and slow wave complexes, starting from the right central region. Cerebral perfusion SPECT revealed increased blood supply in the right hemisphere. She received diagnosis of epilepsy and was treated with phenytoin. After she regained her consciousness, she had no voice and presented with left hemiparesis which was interpreted to be Todd's palsy. She gradually recovered in using her voice, to full normalization of affective prosody in 11 days. The left hemiparesis recovered first in the upper and later in the lower extremities. Her symptoms could be interpreted to be vocal and prosodic disturbance, but not aphasia, because she could understand speach and communicate with writing. The lesions responsible for prosodic deficit are still controversial. The symptoms observed and the findings obtained may indicate that the deficiency of the affective prosody may be caused by the dysfunction of the medial surface of the non-dominant frontal lobe in this case.     

Primary progressive aphasia presenting as conduction aphasia.

We report a case of a woman with primary progressive aphasia (PPA) who presented with conduction aphasia. A 60-year-old, right-handed, Japanese female suffering from progressive aphasia had difficulty in repeating words and phrases. She displayed phonemic paraphasias but had preserved comprehension and had no cognitive or behavior disorder for more than 6 years after the onset of the condition. She was able to continue to work successfully and to perform all her normal daily activities. T1-weighted magnetic resonance imaging revealed minute dilatation of the left inferior horn and sulci in the left hemisphere, and positron emission tomography revealed mild hypometabolism in the left supramarginal gyrus and its surrounding areas. Therefore, she was diagnosed as suffering from PPA presenting as conduction aphasia. We believe that the progressive conduction aphasia of the patient belongs to one of the fluent forms of PPA, and the ability to continue normal work along with the clinical portrayal of preserved memory and cognition skills may be features of a form of PPA presenting as conduction aphasia.