Yoko Konagaya

Clinical and magnetic resonance imaging study of extrapyramidal symptoms in multiple system atrophy.

Slit-hyperintensity in the outer margin of the putamen on T2 weighted MRI was found in 17 out of 28 patients with clinically diagnosed multiple system atrophy. Thirteen of these 17 patients showed extrapyramidal signs. Five patients had only unilateral slit-hyperintensity; four of them had contralateral rigidity; and one had bradykinesia. Despite mild rigidity, one case showed no slit-hyperintensity. One of the 14 cases with parkinsonism showed no hyperintensity, and four of the 14 cases without parkinsonism showed hyperintensity. On the other hand, slit-hyperintensity was not seen in any of 25 patients with clinically diagnosed Parkinsons disease. Putaminal slit-hyperintensity is a useful MRI feature in the differential diagnosis between Parkinsons disease and multiple system atrophy predominantly affecting the extrapyramidal system.

Multiple system atrophy with remarkable frontal lobe atrophy

Abstract The autopsy findings of a multiple system atrophy (MSA) patient with remarkable frontal lobe atrophy are described. The patient was a 65-year-old woman with a 13-year history of untreatable parkinsonism, dysautonomia and progressive motor aphasia. The brain weight was 810 g, and there was remarkable atrophy of the cerebrum predominantly in the frontal lobe, striatum, pons and cerebellum. Microscopic examination revealed a preserved cortical structure with laminar gliosis in the sixth layer of the precentral and superior frontal gyri of the frontal lobe, and postcentral gyrus and inferior parietal lobule of the parietal lobe. The second layer of the cortices of these regions were also revealed to be in a spongy state, and mild cell loss was seen in the fifth and six layers. The frontal lobe white matter showed a mild loss of myelinated fibers and axons, and mild gliosis. Glial cytoplasmic inclusions (GCIs) were abundantly observed in the deep layer of the cortex in the regions mentioned above, and were more abundant in the white matter of the frontal and parietal lobes, callosal body, and internal, external and extreme capsules. There was severe degeneration in the olivopontocerebellar and striatonigral systems, and GCIs in widespread regions of the brain. No Pick bodies, Lewy bodies, ballooned neurons, senile plaques, or significant amounts of neurofibrillary tangles were detected. There were no vascular changes. Thus, this was a verified MSA patient with progressive aphasia and remarkable frontal lobe atrophy. We indicate a possible involvement of the cerebral lobes in MSA.

Progressive cerebral atrophy in multiple system atrophy.

Nine patients with multiple system atrophy (MSA) were studied based on MRI findings of cerebral hemispheric involvement. The age at onset was 56.4+/-8.6 (mean+/-S.D.) years, duration of illness at the first MRI study 2.1+/-1.1 years, duration of illness at the last study 9.7+/-2.6 years, and the follow-up duration 7.6+/-2.3 years. Controls were 85 neurologically intact persons (60.2+/-11.1 years age). In the MRI study, measurements of the ratio of each area to the intracranial area were performed for the cerebral hemisphere, frontal, temporal and parietal-occipital lobes. A significant progression of atrophy to under the normal limit was observed in the cerebrum, frontal and temporal lobes. Besides the typical pathological lesions in MSA, five autopsied patients revealed frontal lobe atrophy with mild gliosis, mild demyelination and glial cytoplasmic inclusions (GCIs). One of these patients showed remarkable frontal lobe atrophy with degenerative changes in the cerebral cortex. We observed the involvement of the cerebral hemisphere, especially the frontal lobe.

Upper motor neuron predominant degeneration with frontal and temporal lobe atrophy

Abstract The autopsy findings of a 78-year-old man mimicking primary lateral sclerosis (PLS) are reported. He showed slowly progressive spasticity, pseudobulbar palsy and character change, and died 32 months after the onset of symptoms. Autopsy revealed severe atrophy of the frontal and temporal lobes, remarkable neuronal loss and gliosis in the precentral gyrus, left temporal lobe pole and amygdala, mild degeneration of the Ammon’s horn, degeneration of the corticospinal tract, and very mild involvement of the lower motor neurons. The anterior horn cells only occasionally demonstrated Bunina body by cystatin-C staining, and skein-like inclusions by ubiquitin staining. This is a peculiar case with concomitant involvement in the motor cortex and temporal lobe in motor neuron disease predominantly affecting the upper motor neuron.

Human PTH(1–34) infusion test in differential diagnosis of various types of hypoparathyroidism: an attempt to establish a standard clinical test

To introduce a simple procedure and reliable diagnostic criteria for parathyroid hormone (PTH) infusion test, 128 patients with either pseudo- (PsH) or idiopathic hypoparathyroidism (IdH) and 25 normocalcemic controls were studied. Incremental responses of urinary cyclic AMP and phosphate to 20 micrograms (67 U) or 30 micrograms (100 U) of human PTH(1-34) were assessed by using simple parameters of urinary excretion rates of the two substances. The results are summarized as follows. (1) PTH dose-cyclic AMP response relation suggests that 100 U of PTH is more appropriate than 67 U as a standard test dose for adults. (2) By presenting the magnitude of cyclic AMP response as either net increase or fold increase during 1 h after 100 U of PTH infusion, we can differentiate PsH type I from others without overlap. (3) Differentiation between PsH and IdH or normocalcemic subjects by phosphaturic response is less clearcut than that made by cyclic AMP response whatever indices and criteria are used. Thus it seems difficult to diagnose PsH type II merely based on the discrepancy between cyclic AMP and phosphaturic responses to exogenous PTH. (4) The test results are essentially similar in the examinations performed before and during vitamin D therapy. However, when the magnitude of phosphaturic response is expressed as net increase during 2 h after PTH, it tends to be enhanced after vitamin D therapy in patients with PsH compared to the response before therapy.

The fluctuation of serum myoglobin levels in Duchenne muscular dystrophy and the carrier

The diurnal changes of serum myoglobin level and CK activity were investigated in 20 cases of Duchenne dystrophy and 6 normal males under ordinary circumstances in their daily lives. Changes of myoglobin levels were also checked in 25 cases of Duchenne carrier and 11 control females with muscular exercise by ergometer of 70 W for 3 min. In Duchenne dystrophy, the myoglobin level was rather low before waking up, then it abruptly increased thereafter and remained high until retiring. The fluctuation range of myoglobin was greater than that of CK. The myoglobin level of control males was extremely low compared with that in Duchenne dystrophy. In Duchenne carriers, although CK activity did not significantly change after the exercise, 5 of 11 known carriers showed an increased myoglobin level.

A clinical and pathological study of a Japanese case of Amyotrophic Lateral Sclerosis/Parkinsonism-Dementia Complex with family history.

Abstract. This report concerns a Japanese family with neuropathological findings consistent with amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) in the Island of Guam. The proband was a 68-year-old woman with an 8-year history of parkinsonism which was followed by psychiatric symptoms and neurogenic amyotrophy 5 years after the onset. She had a family history of parkinsonism associated with dementia in all of her three siblings. They grew up in the Hobara village, a focus of amyotrophic lateral sclerosis in the Kii Peninsula of Japan in their childhood. Their parents were not consanguineous nor natives of the Kii Peninsula. The brain weight was 1040 g and there were mild frontal lobe atrophy, moderate atrophy of pes hippocampi, decoloration of the substantia nigra and locus coeruleus, and atrophy of the anterior root of the spinal cord. The microscopic examinations revealed degeneration of CA1 portion of the hippocampus to the parahippocampus gyrus, substantia nigra, locus coeruleus and spinal anterior horn with Bunina bodies. The spinal pyramidal tracts also mildly degenerated. Neurofibrillary tangles (NFT) were observed in the cerebral cortex, especially in the cortices from hippocampus to lateral occipitotemporal gyri, basal nucleus of Mynert, basal ganglia, thalamus, substantia nigra and widespread regions of the central nervous system through the brainstem to spinal cord including the nucleus of Onufrowitcz. In spite of a small amount of the senile plaques in the cerebral cortex and Lewy bodies in the substantia nigra and locus coeruleus, abundant NFT were distributed mainly in the third layer of the cerebral cortex, which is the characteristic feature of ALS/PDC. Thus, this was likely to be an ALS/PDC case outside the Guam Island. A tau mutation was not found on DNA analysis.

Study of daily driving characteristics of individuals with dementia using video-recording driving recorders.

Conflict of Interest: The editor in chief has reviewed the conflict of interest checklist provided by the authors and has determined that the authors have no financial or any other kind of personal conflicts with this paper. Funding for this study was provided by research grants from the Ministry of Health, Labor and Welfare of Japan; the Chiyoda Mutual Life Foundation; the Hori Sciences and Arts Foundation; the Conference for Expressway-related Social Contribution Activities; the Japan Health Foundation; the Suzuken Memorial Foundation; the ZENKYOREN; and the General Insurance Association of Japan. Author Contributions: Naoko Kawano: Concept and design, acquisition of participants and data, analysis and interpretation of data, and preparation of manuscript. Kunihiro Iwamoto, Tetsuya Iidaka, and Norio Ozaki: Acquisition and interpretation of data. Kazutoshi Ebe: Acquisition and analysis of data. Kunihiro Iwamoto and Yusuke Suzuki: Acquisition of participants and preparation of manuscript. Jun Hasegawa, Katsuyuki Ukai, and Hiroyuki Umegaki: Acquisition of participants. Sponsor’s Role: None.

Urinary Adenosine 3′,5′-Monophosphate, Circulating Amino-Terminal PTH (1–34) and Carboxyl-Terminal PTH in Hypoparathyroidism

In 38 patients with hypoparathyroidism, electrolytes, amino-terminal PTH(1--34) and carboxyl-terminal PTH in serum and urinary cyclic AMP were measured. Serum PTH(1--34) levels were low and C-PTH levels measured simultaneously in the same sera were low except one having a high level. In pseudohypoparathyroidism, serum C-PTH was elevated and in 1 of 2 patients serum PTH(1--34) was elevated. Urinary cyclic AMP was decreased in hypoparathyroidism and there was a positive correlation between urinary cyclic AMP and serum PTH in normal subjects and parathyroid dysfunctions. Responses of urinary cyclic AMP to PTH were better in hypoparathyroidism and less in primary hyperparathyroidism than normal subjects. These data suggest that measurements of serum PTH(1-34), C-PTH and urinary cyclic AMP are important in the diagnosis and pathophysiological classification of hypoparathyroidism.

Clinical analysis of longstanding subacute myelo-optico-neuropathy (SMON), a clioquinol into/INS;xi/INS;cation

WCN 2013 No: 1155 Topic: 36 — Other topic 9.4T MRS characterization of human umbilical cord mesenchymal stem cells underwent death metabolism Y. Xiao, H. Dai, R. Wu. Department of Medical Imaging, The Second Affiliated Hospital of Shantou University Medical College, Shantou, China; Department of Medical Imaging, HuiZhou Municipal Certral Hospital, HuiZhou, China Objective: To explore the metabolite profile of mesenchymal stem cells (MSCs) underwent death using 9.4T high resolutionNMR Spectroscopy. Methods:Human umbilical cordmesenchymal stem cells were cultured and collected. MSCs were treated for 0, 6, 12 and 24 h in a stimulated condition which include hypoxia, serum deprivation and changes of microenvironment. Cell death and the mortality rate was detected by light microscopy, Hoechst staining and flow cytometry analyses. Cell metabolite extraction was prepared by methanol–chloroform (M/C) method and analyzed on a 9.4T NMR device. H-NMR Spectroscopy was obtained and the metabolite concentraction of each time point was calculated. Results: Necrosis is the major form of cell death in the built model. In the early stage of cell death (6 and 12 h), the fatty acid metabolite concentraction increased with statistical significance (p b 0.05), while in the last stage of cell death, the fatty acid peak decreased (p b 0.05). Conclusions: There are some specific characteristics on MRS of MSCs that underwent death, and the fatty acid peak may serve as a biomarker for cell death. doi:10.1016/j.jns.2013.07.2155 Abstract — WCN 2013 No: 1157 Topic: 36 — Other topic Clinical analysis of longstanding subacute myelo-optico-neuropathy (SMON), a clioquinol intoxication WCN 2013 No: 1157 Topic: 36 — Other topic Clinical analysis of longstanding subacute myelo-optico-neuropathy (SMON), a clioquinol intoxication M. Konagaya, S. Kuru, M. Sakai, Y. Saito, Y. Konagaya, SMON Research Committee. Neurology, National Organization Suzuka Hospital, Suzuka, Japan; Neurology, Higashi Nagoya National Hospital, Japan; Research Division, Ohbu Dementia Care Research and Training Center,