[Cerebral infarction accompanied by dysgeusia--a clinical study on the gustatory pathway in the CNS].

Dysgeusia was investigated in 11 patients with thalamic infarction and 13 patients with corona radiata infarction to locate the gustatory pathways based on the sites of the lesions. Dysgeusia was present in 4 out of 11 patients with thalamic infarction and 3 out of 13 patients with corona radiata infarction. The dysgeusia was contralateral to the lesion in all these patients. Cheiro-oral syndrome was observed as a complication in 2 patients each from both groups. The responsible lesion was located on the medial side of the ventral posterolateral (VPL) nucleus and the ventral posteromedial (VPM) nucleus in the patients with thalamic infarction who developed dysgeusia, and was located posteriorly to the corona radiata in the other group. In the patients without gustatory disturbance, on the other hand, the lesions showed no such spread. These findings suggest that the gustatory pathway ascends contralaterally in the cerebral hemisphere and that the pathway from the thalamus projects to the cerebral cortex via the posterior part of the corona radiata. It is also suggested that the pathways in the thalamus and corona radiata are very close to the sensory fibers from the mouth and hands projecting to the sensory area.

[Unilateral gustatory disturbance by pontine infarction].

We reported a case of pontine infarction presenting as diminished taste on the contralateral side. A 67-year-old man was hospitalized with a sudden onset of right hypogeusia. No neurological abnormalities were found except diminished taste in the areas innervated by the chorda tympani, greater petrosal and glossopharyngeal nerves on the right side. Brain MRI demonstrated a lesion with low-intensity on T1-weighted images and high-intensity on T2-weighted images in the left suprapontine tegmentum. Cases of pontine disease presenting as contralateral dysgeusia have rarely been reported. In the present case, we considered that infarction occurred superior to the pontine taste are (PTA). It is suggested that the gustatory pathway superior to PTA takes a chiasmal tract ascending in the brainstem.

[Objective measurement of motor activity in Parkinson's disease by actigraphy. Clinical assessment of akinesia].

Actigraphy measures physiological activity by using an acceleration sensor and RAM equipped with a data logger. Since actigraphy can continuously and easily record data over a long period of time without disturbing the normal activities of subjects, it is possible to analyze a large number of subjects. Actigraphy was performed in patients with Parkinson's disease who did not exhibit trembling. Results showed that the daily motor activity of patients was lower than that of the healthy individual. Daily motor activity was also found to be correlated with Hoehn-Yahr's classification. Furthermore, side-effects due to L-dopa, such as abnormal involuntary movement and on-off phenomenon, could be objective assessed. The results of long-term actigraphic examination, conducted after anti-Parkinsonian treatment, showed that akinesia improved with time. From these findings, it is concluded that actigraphy could quantitatively assess the degree of akinesia in Parkinsonian patients. Furthermore, actigraphy may be applied to the clinical assessment of drugs.

[Tactile extinction following the cerebral sinus thrombosis. A study using event-related potentials].

We reported a 46-year-old woman in whom cerebral sinus thrombosis manifested itself in headache and weakness while she was on an oral estrogen preparation. The neurological examinations revealed monoparesis of the right upper extremity, hyperactive right deep tendon reflex and disturbance of the joint sense, stereognostic sense, two-point discrimination and skin writing sense in the right upper extremity, in addition to tactile extinction. Brain CT showed empty triangle sign. MRI revealed infarction near the left postcentral gyrus and thrombosis of right sigmoid sinus. In the MRA study, there was low signal intensity in the posterior portion of the superior sagittal sinus and right transverse sinus. Short latency SEP in upper extremity showed that the central conduction time was normal, while the amplitude of wave components after N20 was reduced on the right side. Both palms were given bilateral or unilateral pressure stimuli. When event-related potentials (ERP) were evoked, using bilateral stimuli as a rare target, and left unilateral stimuli as a non-target, the P 300 latency was prolonged to 450 msec but was restored to normal with the improvement of tactile extinction. These results seem to suggest that the tactile extinction is ascribable to a disturbance of recognition at the higher brain function level and that ERP can be a useful objective yardstick in the evaluation of extinction phenomenon.

Electric field distribution of event-related potentials in stroke patients.

Electrical field distributions of event-related potentials (ERP's) were recorded during an auditory "oddball paradigm" and were analyzed in terms of time and space. Fourteen normal subjects and 14 chronic patients were cerebral thrombosis were studied. For the components N1 and P3 of the ERP's to target stimuli, reference-independent measures (latency, global field power, location of maximal or minimal potential, and location of centroids) were determined. Stroke patients displayed P3 abnormalities in latency, amplitude, and electrical field on the scalp. In addition, N1 electrical fields were also abnormal. These ERP abnormalities correlated significantly with the extent of mental function impairment in the stroke patients, and they improved after administration of a cerebral metabolic enhancer (Nefiracetam: DM-9384). The ERP's seemed to be sensitive in indicating the effects of the drug. These data suggest that time-course analysis of the spatial distribution of the ERP electrical field might be useful for evaluation of the extent of mental function impairment and the efficacy of drugs.

[Klinefelter syndrome accompanied by hypoplasia of the bilateral internal carotid arteries--a case report].

The patient was a 31-year-old male who was admitted to our hospital due to slowly progressive weakness of the right upper limb and both lower limbs. Physical examination showed a tall stature, gynecomastia, feminine pubic hair, small penis and small testis. His intellectual function on the Kohs-cubic test scored 85, on the WAIS scored performance 80, verbal 74 and full scale 78. He showed BE mixed type in YG personality test and neurotic in CMI. No abnormalities were observed except for severe weakness of the right upper limb, moderate weakness of the bilateral lower limbs and hypalgesia in the limbs. Clinical laboratory tests revealed low blood and urinary testosterone levels, and a high blood gonadotropin level. Cytogenetic studies showed a 47, XXY karyotype. On neuroradiological examinations, there were small bilateral carotid canals in CT scan and multiple long T2 lesions in MRI. Angiography showed hypoplasia of the bilateral internal carotid arteries and dilatation of the bilateral vertebral arteries. The results of activity measurements of affected limbs using actigraph indicated that a conversion type hysteria had resulted in weakness of the limbs. There is no report of Klinefelter syndrome accompanied by the hypoplasia of bilateral internal carotid arteries, and the condition is considered to be extremely rare.

[Central diabetes insipidus complicated with upbeat nystagmus and cerebellar ataxia].

A 34-year-old male presented with central diabetes insipidus accompanied by upbeat nystagmus and cerebellar ataxia. The patient without family history of above began to walk at the age of 2 years. Polyposia and polyuria were noted at the age of about 4 years. Dysbasia developed at the age of about 7 years. From the age of 30, lalopathy appeared together with various clinical symptoms including cataracts, disturbed intelligence, upbeat nystagmus, abnormal ocular movements, ataxic speech, cerebellar ataxia and reduction of the muscle tone of the limbs, and hypotonic polyuria. Abnormal laboratory findings included square wave jerks, upbeat nystagmus, and "bow tie" nystagmus by EOG, atrophy of the cerebellum and the brainstem by CT, a slight prolongation of the P300 latency, and a central diabetes insipidus pattern by water deprivation test and Carter-Robbins tests. There have been occasional reports of diabetes insipidus complicated by cerebellar ataxia, but in no earlier reports has diabetes insipidus been concurrent with abnormal ocular movements such as upbeat nystagmus. A degenerative disease primarily of the posterior lobe of the hypophysis, hypothalamus, cerebellum, and brainstem was suspected.