[Show abstract][Hide abstract] ABSTRACT: We evaluated the alexia and agraphia of three patients with different lesions using Japanese kanji (morphograms) and kana (phonograms) and made a lesion-to-symptom analysis. Patient 1 (pure alexia for both kanji and kana and minor agraphia for kanji after a fusiform lesion) made more paragraphic errors for kanji, whereas patient 2 (alexia with agraphia for kanji after a posterior inferior temporal lesion) showed severe reading and writing disturbances and more agraphic errors for kanji. Brodmann Area 37 was affected in both patients, but in patient 2 the lesion was located lateral to that in patient 1. Patient 3 showed agraphia without alexia after restricted lesion to the angular gyrus. We believe that pure alexia (patient 1) results from a disconnection between the medial fusiform gyrus and posterior inferior temporal area (the lateral fusiform and inferior temporal gyri), whereas alexia with agraphia for kanji (patient 2), corresponding to lexical agraphia in Western countries, results from damage to the posterior inferior temporal area, in which whole-word images of words are thought to be stored. Furthermore, restricted lesion in the angular gyrus (patient 3) does not produce alexia; the alexic symptom of "angular" alexia with agraphia may be the result of damage to the adjacent lateral occipital gyri.
Journal of the Neurological Sciences 10/2000; 178(1):42-51. DOI:10.1016/S0022-510X(00)00363-4 · 2.47 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: A 40-year-old woman who developed intestinal dysmobility was found, at rectal biopsy, to have marked microvacuolation of mucosal muscle layer cells, which corresponded to increased accumulation of abnormal mitochondria. Skeletal muscle biopsy specimens showed ragged-red fibers, vessels strongly reactive for succinic dehydrogenase, and focal deficiency of cytochrome c oxidase. Autopsy performed at the age of 50 revealed prominent accumulation of abnormal mitochondria in the intestinal smooth muscle cells with a mottled distribution of focal necrosis, multiple small cerebral infarcts with diffuse neuronal loss, and rarefaction of the perivascular white matter. Mitochondrial DNA analysis showed a point mutation at position 3243. This case, showing features of both mitochondrial neurogastrointestinal encephalomyopathy and mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), indicates that routine intestinal biopsy can detect mitochondrial encephalomyopathy with gastrointestinal involvement. The main intestinal changes were extensive accumulation of abnormal mitochondria in the leiomyocytes and scattered focal necrosis.
[Show abstract][Hide abstract] ABSTRACT: It is controversial whether long-term anticholinergic therapy (AC-T) can cause short-term memory disturbance, long-term memory disturbance or intelligence impairment. Ninety patients with Parkinson's disease were examined prospectively to clarify the existence of memory and intelligence impairment induced by long-term AC-T. Neuropsychological tests detected eight patients with at least one of four types of deficits; intelligence impairment in four patients, verbal delayed recall impairment in eight, verbal short-term memory disturbance in five, and verbal long-term memory disturbance in six. Two types of verbal memory disturbance (short-term one and long-term one) appeared with double dissociation, suggesting that there may be an independence between the two types of verbal memory system. Older patients were more prone to suffer from these reversible deficits. Moreover, all of the patients who resumed AC-T, showed recurrence of the deficits acutely. These observations should indicate the tight relation between long-term AC-T and neuropsychological deficits.
Internal Medicine 07/1998; 37(6):514-8. DOI:10.2169/internalmedicine.37.514 · 0.90 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We present a patient with pure alexia following a hemorrhagic infarction in the left fusiform gyrus. The symptom began with alexia preferentially disturbed for kana, but during the course of recovery slight agraphia for kanji became pronounced. In the earlier phase, alexia was more severe than agraphia and he could write kanji that he could not read. Furthermore, kinesthetic reading was effective. These findings are consistent with the symptoms of pure alexia, although the fact that a writing disturbance for kanji persisted resembles the profile of alexia with agraphia for kanji due to a posterior inferior temporal lesion. Based on the fact that the posterior inferior temporal lesion showed more severe agraphic symptom and more frequent nonresponse writing errors of kanji, and that our patient's lesion was mainly located in the fusiform gyrus that is medial to the inferior temporal gyrus, we believe that alexia occurred when the inferior temporal gyrus was disconnected from the fusiform gyrus, as a result, visual information could not reach the inferior temporal gyrus in which the visual images of individual kanji are stored.
[Show abstract][Hide abstract] ABSTRACT: To evaluate the role of the sub-cortical white matter and cortical areas of the supramarginal gyrus in short-term memory impairment (shortened digit or letter span) and repetition difficulty, four patients with conduction aphasia and impaired short-term memory and two patients with only short-term memory impairment were given digit span, letter span, speech audiometry and dichotic listening tests. The results showed that in most of the patients letter span was inferior to digit span and that bilateral ear suppression in the dichotic listening test was observed in two patients with a lesion in the inferior part of the supramarginal gyrus, suggesting that what was affected was phonological information and that the supramarginal gyrus was the storage site. The overlapped lesion of conduction aphasia patients with short-term memory impairment was the periventricular white matter at the upper to middle part of the trigone, while patients with only short-term memory impairment had a lesion in the inferior supramarginal gyrus in common. Thus, damage to the periventricular white matter at the trigone may yield the phonemic paraphasia characteristic of conduction aphasia, while damage to the inferior part of the supramarginal gyrus may result in the impairment of short-term memory. We believe that as a part of the mechanisms of short-term memory and repetition, phonological information is processed in the primary auditory cortex and goes through the periventricular white matter to the inferior part of the supramarginal gyrus and is temporarily stored there.
Journal of the Neurological Sciences 03/1998; 154(2):182-93. DOI:10.1016/S0022-510X(97)00227-X · 2.47 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We report a patient with primary progressive aphasia who first presented with amnesic aphasia that developed over the course of 3 years into nonfluent aphasia with buccofacial apraxia, followed in the next year by cognitive impairment and parkinsonism. Pathological findings were typical for corticobasal degeneration except for the distribution of cortical atrophy. This case suggests that corticobasal degeneration should be included in the differential diagnosis of primary progressive aphasia, especially in association with parkinsonism.
European Neurology 02/1996; 36(3):134-7. DOI:10.1159/000117229 · 1.36 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Long-term oral anticholinergic (AC) therapy can occasionally produce intellectual impairment. We investigated a patient with Parkinson's disease accompanied by intellectual impairment induced by long-term AC therapy. The intellectual impairment of the patient disappeared after cessation of AC therapy. Positron emission tomography (PET), during and after long-term oral AC therapy, revealed that it causes bilateral diffuse decrease of glucose metabolism in the cortex, basal ganglia, thalamus, hippocampus and cerebellum. Cessation of the therapy resulted in diffuse increase of glucose metabolism in all of the above regions. Cranial CT and magnetic resonance imaging (MRI) showed no abnormalities. Our results suggest that long-term AC therapy causes reversible bilateral diffuse glucose hypometabolism.
Journal of the Neurological Sciences 10/1995; 132(1):89-92. DOI:10.1016/0022-510X(95)00131-K · 2.47 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Five patients suffering from painful alcoholic neuropathy showed severe painful sensory disturbance in their extremities. Although their pain was not ameliorated by the typical usual analgesic agents, oral mexiletine (MX) therapy was remarkably effective for the pain (especially tingling and aching sensation) without major side effects. This study indicated that the minimum effective dose was 300 mg per day and the effective concentration of MX in plasma was 0.66 +/- 0.15 micrograms/ml in these patients. Thus, oral MX therapy can be a reliable treatment for pain in alcoholic neuropathy.
Internal Medicine 07/1995; 34(6):577-9. DOI:10.2169/internalmedicine.34.577 · 0.90 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: In patients with POEMS syndrome, which often accompanies plasma cell dyscrasia, the roles of interleukin-6 (IL-6) and other acute phase cytokines are unknown. Serum IL-6, interleukin-1 beta (IL-1 beta) and tumor necrosis factor-alpha (TNF-alpha) were measured by the enzyme-linked immunosorbent assay technique in 16 patients with POEMS syndrome and in patients with other neurological diseases (OND) as control. Serum IL-6 was more frequently detected and higher in active POEMS than in stable POEMS or in OND patients. No sample was positive for IL-1 beta. TNF-alpha was positive in 3 out of 7 active POEMS patients, but its significance was not definite. Serum IL-6 levels in POEMS patients reflected the disease activity but not the severity of accompanying plasma cell dyscrasia.
Internal Medicine 11/1994; 33(10):583-7. · 0.90 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: In patients with POEMS syndrome, which often accompanies plasma cell dyscrasia, the roles of interleukin-6 (IL-6) and other acute phase cytokines are unknown. Serum IL-6, interleukin-1beta (IL-1beta) and tumor necrosis factor-alpha (TNF-alpha) were measured by the enzyme-linked immunosorbent assay technique in 16 patients with POEMS syndrome and in patients with other neurological diseases (OND) as control. Serum IL-6 was more frequently detected and higher in active POEMS than in stable POEMS or in OND patients. No sample was positive for IL-1beta. TNF-alpha was positive in 3 out of 7 active POEMS patients, but its significance was not definite. Serum IL-6 levels in POEMS patients reflected the disease activity but not the severity of accompanying plasma cell dyscrasia.
Internal Medicine 10/1994; 33(10):583-587. DOI:10.2169/internalmedicine.33.583 · 0.90 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The case is described of a patient with alexia and agraphia for kanji, and severe anomia after a subcortical haemorrhage in the left posterior inferior temporal area. Magnetic resonance imaging at four months after onset showed a lesion in the inferior temporal and fusiform gyri, extending from the temporo-occipital junction toward the anterior third of the temporal lobe. Comparison with other reported cases of alexia with agraphia and anomia made it clear that when accompanied by severe anomia, the lesions extended either forward to the anterior part of the middle temporal gyrus or medially to the parahippocampal gyrus. It is suggested that the disconnection of association fibres between the parahippocampal, fusiform, middle, and inferior temporal gyri, especially between the parahippocampal gyrus and the other temporal gyri, or the cortical damage to the posterior part of these gyri is essential for the production of anomia.
[Show abstract][Hide abstract] ABSTRACT: Since an oral regimen of levodopa has been instituted for treatment of Parkinson's disease, its absorption and metabolism has been well demonstrated. However, its chemical characteristics of high solubility in acid solution and low solubility in water have not been well known. We paid attention to this characteristic and studied the relationship between its absorption and gastric acid secretion in 38 patients with Parkinson's disease who became refractory to therapy of levodopa. We measured the pH and amount of collected fasting gastric juice. Gastric acid secretion was decreased in 22 patients (58%). In ten of these 22 patients, 30 ml of lemon juice was prescribed in every administration of levodopa as a supplement to gastric acid for two weeks. Increases of L-dopa concentration after 60 min. and 180 min. were observed after lemon juice supplement therapy. Among the Parkinson symptoms, rigidity, akinesia, and small step gait were improved in every case except one patient who showed decrease of L-dopa concentration at 180 minutes. However, improvement of tremor was less remarkable. We consider this supplement therapy to gastric acid is one of the effective and useful methods in the management of Parkinson's disease.
[Show abstract][Hide abstract] ABSTRACT: We describe a patient with axial myoclonus. Myoclonus first occurred in the upper abdominal muscle and spread up to the neck muscle and down to the lower abdominal muscle. Physiological studies of the jerks revealed that the myoclonus arose in the thoracic spinal cord and spread slowly up and down the spinal cord at about 3 m/s. We conclude that the myoclonus is mediated by the propriospinal tract.
European Neurology 02/1994; 34(1):48-50. DOI:10.1159/000117008 · 1.36 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We report a 44-year-old Japanese man with chronic meningitis due to coccidoiodomycosis. He was admitted to our hospital because of pneumonia after the business trip to Phoenix, Arizona. Coccidioid immitis was cultured from periathric abscess on the sternoclavicular joint. He became asymptomatic by 5-FC administration. One year later, however, he complained of headache and fever. Coccidiodial meningitis was diagnosed by cerebrospinal fluid (CSF) examination. Both systemic and intrathecal administration of miconazole and oral itraconazole were ineffective. While meningitis was not cured for three years, normal pressure hydrocephalus (NPH) developed. CSF cell count fell into normal range after administration of fluconazole (800 mg/day) for thirteen months, but NPH continued. This is the first report of coccidiodimycosis with CNS involvement in Japan.
[Show abstract][Hide abstract] ABSTRACT: We investigated somatosensory evoked magnetic fields (SEFs) and somatosensory evoked potentials (SEPs) in patients with myoclonus epilepsy. The median nerve was stimulated at the wrist, and responses were recorded over the contralateral hemisphere. The source of the enlarged cortical component of the giant SEF was localized on the post-central sensory cortex. The P1 component of the giant cortical response was composed mainly of a tangentially oriented dipole at area 3b. This is the first magnetoencephalographic analysis of an abnormally enlarged somatosensory cortical response.
Electroencephalography and Clinical Neurophysiology 12/1993; 87(5):300-5. DOI:10.1016/0013-4694(93)90183-V
[Show abstract][Hide abstract] ABSTRACT: A 67-year-old patient was admitted to our hospital owing to coma and tetraplegia. MRI showed T2 weighed high intensity areas in the pontine tegmentum, lower aspect of the right cerebellar hemisphere, left half of medulla oblongata, and bilateral paramedian thalamus. He showed marked recovery by urokinase injection becoming able to walk in a few months, but severe amnesia and hypersomnia persisted even 5 months after onset. EEG showed diffuse alpha activity with occasional delta waves in frontal leads. Intelligence was considered normal (WAIS score; verbal IQ 97, performance IQ 102, total IQ 99), through performance on Wechsler memory Scale-R (Revised Japanese edition, WMS-R) and Benton Visual Retention Test indicated impairment of both verbal and visual memory. Verbal memory was impaired to a greater degree than visual memory (Scores of WMS-R: verbal memory index 60, visual memory index 98, index of general memory 72, attention index 95, index of delayed memory 71). He was able to finish only 0 and 1 categories on two trials of the Wisconsin Card Sorting Test. FDG (18F-fluorodeoxyglucose)-PET showed diffuse areas of decreased metabolism in bilateral thalami, frontal lobes, cingulate gyri and medial temporal lobes. The bilateral thalamic lesion seemed to affect the following structures, as judged from MRI: 1) The anterior thalamic peduncle including most of the reciprocal connections between dorsomedial nucleus of thalamus (MD nucleus) and the frontal lobe, 2) The inferior thalamic peduncle which serves as the reciprocal pathway between MD nucleus and medial temporal lobe, especially the amygdala (component of Yakovlev circuit), and 3) The inferior part of mammillothalamic tract (component of Papez circuit).(ABSTRACT TRUNCATED AT 250 WORDS)
[Show abstract][Hide abstract] ABSTRACT: We describe a 53-year-old woman with portal-systemic encephalopathy and altered thyroid function. Endocrinological studies revealed low levels of free thyroid hormone with an inappropriately low level of thyroid-stimulating hormone that responded to bolus injection of thyrotropin-releasing hormone with a normal but somewhat delayed pattern. On the diagnosis of hypothalamic hypothyroidism, she was treated with levothyroxine sodium. Thyroid hormone replacement improved not only the symptoms of hypothyroidism but the hyperammonemia and consciousness disturbance, which suggested a hitherto undescribed possibility that hypothyroidism may be an exacerbation factor of hyperammonemia and portal-systemic encephalopathy.
Internal Medicine 09/1993; 32(8):655-8. DOI:10.2169/internalmedicine.32.655 · 0.90 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Propriospinal myoclonus is one type of myoclonus that is proposed to originate in the spinal cord and spread up and down via propriospinal tract. There are a few reports describing the detailed electrophysiological findings of this myoclonus. In this communication, we present the results of electrophysiological analysis of a patient with propriospinal myoclonus. A 23-year-old woman suddenly noticed abdominal pain, which spontaneously faded in a few hours. Irregular involuntary flexion jerks of the trunk appeared spontaneously without pain or hiccups after this episode. It involved the bilateral axial muscles; sternocleidomastoid muscles (SCM), paravertebral muscles (PVM), abdominal muscles (ABD), and intercostal muscles (ICM), but not the limb muscles. It was worsened by the mental stress, but not by her posture or position. While she slept, the jerks were not observed. Routine laboratory examinations were all normal. Magnetic resonance imaging of the spinal cord revealed no abnormalities. The electrophysiological studies done on this patient are polymyography, back-averaging of the EEG activity preceding spontaneous jerks (jerk-locked averaging (JLA), and movement related cerebral potential (MRCP) preceding the involuntary jerks and voluntary abdominal movements. No EEG activities preceding the myoclonus were demonstrated by JLA or MRCP. No MRCP recorded preceding the myoclonus suggests that the jerk is not a self-initiated or externally triggered voluntary movement. Polymyography revealed that the jerks involved the bilateral axial muscles including SCM, PVM, ICM, and ABD, but not the limb muscles. Homologous muscles were activated synchronously. The duration of EMG bursts was variable ranging 50 to 250 ms in these muscles.(ABSTRACT TRUNCATED AT 250 WORDS)
[Show abstract][Hide abstract] ABSTRACT: Reports about chronic-onset dementia due to ACA have been rare. Here we described five patients with chronic dementia induced by ACA. We examined their clinical aspect, neuropsychological aspect, and neuroradiological aspect in detail. Our study suggests the possibility of ACA-induced chronic-onset dementia in PD patients.
Advances in neurology 02/1993; 60:479-83. · 1.05 Impact Factor