Teruhiko Kachi

Videofluorographic Study of Swallowing in Parkinson's Disease

Abstract. We studied 16 patients with Parkinsons disease (PD) with dysphagia and 8 young and 7 elderly normal controls videofluorographically to evaluate the nature of swallowing disorders in PD patients. In 13 patients, abnormal findings in the oral phase were residue on the tongue or residue in the anterior and lateral sulci, repeated pumping tongue motion, uncontrolled bolus or premature loss of liquid, and piecemeal deglutition. Thirteen patients showed abnormal findings in the pharyngeal phase, including vallecular residue after swallow, residue in pyriform sinuses, and delayed onset of laryngeal elevation. Ten of these patients also showed abnormal findings in both the oral and pharyngeal phases. Aspiration was seen in 9 patients. The oral transit duration was significantly longer in the patients with and without aspiration than in the control subjects. The stage transition duration, pharyngeal transit duration, duration of the upper esophageal sphincter (UES) opening, and total swallow duration were significantly longer in the patients with and without aspiration than in the young controls, but were not longer than in the elderly controls. These durational changes in the pharyngeal phase of swallowing were similar to those in the elderly controls. The findings suggest that the disturbed motility in the oral phase of swallowing may be due to bradykinesia. Although PD patients with dysphagia evince a variety of swallowing abnormalities, the duration of pharyngeal swallowing may remain within the age-related range until the symptoms worsen.

Occipital hypoperfusion in Parkinson’s disease without dementia: correlation to impaired cortical visual processing

Objective: The purpose of this study was to analyse changes in regional cerebral blood flow (rCBF) in Parkinson’s disease (PD) without dementia. Methods: Twenty eight non-demented patients with PD and 17 age matched normal subjects underwent single photon emission computed tomography with N-isopropyl-p-[123I]iodoamphetamine to measure rCBF. The statistical parametric mapping 96 programme was used for statistical analysis. Results: The PD patients showed significantly reduced rCBF in the bilateral occipital and posterior parietal cortices (p<0.01, corrected for multiple comparison p<0.05), when compared with the control subjects. There was a strong positive correlation between the score of Raven’s coloured progressive matrices (RCPM) and the rCBF in the right visual association area (p<0.01, corrected for multiple comparison p<0.05) among the PD patients. Conclusions: This study showed occipital and posterior parietal hypoperfusion in PD patients without dementia. Furthermore, it was demonstrated that occipital hypoperfusion is likely to underlie impairment of visual cognition according to the RCPM test, which is not related to motor impairment.

Visual hallucination in Parkinson's disease with FDG PET

To determine the characteristics of cerebral glucose metabolism in Parkinsons disease patients with visual hallucinations, group comparison studies using [18F]fluorodeoxyglucose positron emission tomography were performed. Nondemented Parkinsons disease patients in advanced stages were classified into two groups: (1) patients without visual hallucinations; (2) patients with visual hallucinations. Compared to patients without hallucinations, the relative regional cerebral glucose metabolic rate was greater in the frontal areas in patients with visual hallucinations, and the increase reached a significant level in the left superior frontal gyrus. Relative frontal hypermetabolism may be a feature of Parkinsons disease patients with visual hallucinations.

Subclinical phenotypic expressions in heterozygous females of X-linked recessive bulbospinal neuronopathy

Four of 8 definite heterozygous female carriers determined by PCR amplification of tandem CAG repeat of the AR gene, from 4 families of X-linked recessive bulbospinal neuronopathy (X-BSNP) showed extensive high amplitude motor unit potentials in examined muscles although all subjects were neurologically normal. Plasma creatine kinase, myoglobin, myosin light chain, lactate and pyruvate were all normal even in the carriers who showed EMG abnormalities. Muscle biopsy showed a type 2 fiber preponderance and possible very mild type 2 fiber grouping in a carrier with an EMG abnormality. These results suggest that a mutant AR gene may express subclinical phenotypic manifestations in a subpopulation of the heterozygous females of X-BSNP.

Chronic progressive sensory ataxic neuropathy : clinicopathological features of idiopathic and Sjogren's syndrome-associated cases

Eleven patients with chronic progressive sensory ataxic neuropathy were examined clinicopathologically. Three cases were associated with primary Sjögrens syndrome (SS-SAN) and the others were considered to be idiopathic (ISAN). The major clinical symptom in both was loss of proprioceptive and kinesthetic sensation with some impairment of superficial sensation, with multifocal and asymmetrical distribution and progression. The truncal and trigeminal nerves were frequently involved. The motor system was substantially preserved. These somatic sensory and motor symptoms did not differ between ISAN and SS-SAN, but autonomic nervous system signs were more frequent in SS-SAN. Polyclonal elevations of serum IgG and/or IgA were seen in 8 patients. One autopsied case with ISAN combined with previous reports suggested that systemic T-and B-cell infiltration into the nervous tissues, as well as a wide variety of the visceral organs, may be a common finding in ISAN and SS-SAN, and could participate in the cause of this neuropathy and polyclonal hypergammaglobulinaemia.

Aberrant androgen action and increased size of tandem CAG repeat in androgen receptor gene in X-linked recessive bulbospinal neuronopathy

Plasma levels of testosterone, luteinizing hormone (LH) and follicle-stimulating hormone (FSH) after 3 or 6 days of administration of the synthetic androgenic hormone fluoxymesterone (10 mg/day) were measured in 26 patients with X-linked recessive bulbospinal neuronopathy (X-BSNP) and 22 age-matched male controls. The testosterone, LH and FSH levels in the controls were markedly suppressed after administration, but in the patients with X-BSNP, they were suppressed significantly less. The level of suppression varied considerably with the patients, and those of plasma testosterone and FSH were significantly correlated with the number of CAG repeats in the androgen receptor gene. These findings suggest that the androgen action was aberrantly transduced in the target organs in the patients with X-BSNP and which is related to the elongated CAG repeat in the androgen receptor gene.

Central motor and sensory conduction in X-linked recessive bulbospinal neuronopathy.

Central conduction was studied in 12 patients with X-linked recessive bulbospinal neuronopathy (XBSN) using percutaneous electrical cortical, cervical and lumbar stimulation and somatosensory evoked potentials (SEPs). The central motor conduction time from the motor cortex to the cervical and lumbar segments of the spinal cord was normal in XBSN. SEPs, however, were abnormal or central sensory conduction time was prolonged in patients with XBSN. These results are consistent with the clinicopathological findings of XBSN in which the primary sensory neurons are involved as well as the lower motor neurons in the CNS, whereas the upper motor neurons are well preserved.

Pain-Related and Electrically Stimulated Somatosensory Evoked Potentials in Patients With Stroke

BACKGROUND AND PURPOSE Pain-related somatosensory evoked potentials (SEPs) were applied to study abnormality of pain sensation in stroke, together with electrically stimulated SEPs for deep sensation. METHODS We recorded pain-related SEPs after CO2 laser stimulation to the dorsum of the hand and electrically stimulated SEPs after median nerve stimulation at the wrist in 12 patients with stroke. We analyzed P340 in pain-related and parietal N20 in electrically stimulated SEPs. RESULTS In 5 patients with a putaminal lesion, P340 was absent or its latency was delayed, and N20 was absent or reduced in amplitude. In 3 patients with a thalamic lesion, P340 and N20 showed various patterns according to the involved sites. In 4 patients with a lesion in the corona radiata, P340 and N20 were normal. CONCLUSIONS Abnormalities of P340 and N20 in the stroke location were related to impairment of the pain and vibration senses, respectively. Thus, pain-related and electrically stimulated SEPs were useful to investigate the sensory function of each structure in the central nervous system in patients with stroke.

Pain-related somatosensory evoked potentials in dementia

Pain-related somatosensory evoked potentials (pain SEPs) were examined in 25 demented and non-demented patients to investigate the cognitive function for pain in the progression of dementia. Pain SEPs by CO2 laser stimulation were recorded together with auditory event-related potentials (auditory ERPs). P340 in pain SEPs and P300 in auditory ERPs were analysed. The latency of P300 evoked in mildly demented patients was inversely correlated with the Mini-Mental state examination score, and the latency of P340 was also inversely correlated to that score but to a lesser extent. Pain SEPs were not recorded in 4 of 7 severely demented patients. These results indicate that the P340 component of pain SEPs is apparently different from the P300 component of auditory ERPs and suggest that the pain perception in severely demented patients may be abnormal.

Age-related changes in brain neuromagnetic responses to face perception in humans

In order to investigate the effects of ageing on face perception, we studied the magnetic responses to face images in 15 young (19-38 years) and 10 elderly (51-81 years) subjects. Face-specific responses (160mF), which originate in the inferior occipitotemporal cortices, and face non-specific responses (100m), which originate in the primary visual cortices, were evoked in all subjects. Averaged peak latency of the 160mF in the elderly group (174.0+/-9.1 ms) was significantly longer (P<0.0005) than that in the young group (161.5+/-5.1 ms), while no inter-group difference was found in the 100m latency. There was a significant correlation between age and 160mF latency (+0.35 ms/year, R=0.747) suggesting age-related decline of face perception.