Characteristics of Early-Onset Dementia in Chiba Prefecture, Japan: A Multicenter Survey.

INTRODUCTION: Early-onset dementia (EOD), defined as dementia onset before the age of 65 years, is relatively rare, but its social impacts are significant. This study aimed to characterize the diagnosis and clinical and social status of EOD subjects in the 11 dementia centers in Chiba Prefecture, Japan. METHODS: A retrospective 1-year survey was conducted. Collected data included clinical diagnosis, age at onset, age at survey, neuropsychological test, family history, employment, and living status. RESULTS: We identified 208 EOD subjects, including 123 (59.4%), 24 (11.6%), 21 (10.1%), 17 (8.2%), and 10 (4.8%) with Alzheimer's disease (AD), vascular dementia, frontotemporal lobar degeneration (FTLD), dementia with Lewy bodies/Parkinson's disease dementia, and alcohol-related dementia, respectively. The Mini-Mental State Examination (MMSE) score <24 was observed in 50-75% of patients and was not correlated with disease duration. Twenty-four (16.4%) subjects had positive family history of EOD. EOD subjects were at risk of early retirement, and 133 subjects lived with their family, in whom 64 (30.8%) lived with their child. CONCLUSION: In dementia centers, AD, FTLD, and Lewy body dementia had relatively large proportion. Employment, economy, and social supports are urgently needed for EOD subjects and their family.

Botulinum toxin treatment for difficult-to-treat finger pressure ulcers caused by severe hand flexion: case report.

We report the successful treatment of two cases of difficult-to-treat pressure ulcers with botulinum toxin type A (BoNT-A). A 71-year-old male patient with Parkinson's disease presented with severe hand grip deformities of the fingers and a pressure ulcer (PU) on the right hand. He received 240U of BoNT-A into the upper limb muscles, which improved finger mobility during passive extension and resulted in resolution of the palm PU. No recurrence was noted. A 69-year-old female patient with Lewy body dementia presented with a PU on the palm side of the middle finger apex of the right hand, with exposure of the phalanx bone and dark red oedematous granulation of the tip of the finger. Severe muscle tone was noted. She received 240U of BoNT-A injected into the muscles of the upper extremities. This resulted in the disappearance of the contracture between the middle finger cusp and palm, and prompt healing of the PU. A protective finger orthosis was also used to improve hand finger grip and prevent further PUs. Although BoNT-A injection resulted in only slight improvement in the range of motion, it produced relief of pressure with consequent healing of the PU. Injection of BoNT-A into the affected muscles of the patients in this case report was effective in reducing flexor muscle tone, relief of pressure on the palm skin and healing of hand PUs.

Effects of Flexor Digitorum Longus Muscle Anatomical Structure on the Response to Botulinum Toxin Treatment in Patients with Post-Stroke Claw Foot Deformity.

(1) Background: The purpose of this retrospective case-control study was to determine the relationship between the control of toe movements by flexor hallucis longus (FHL) and flexor digitorum longus (FDL) muscles and the response to treatment with botulinum toxin (BoNT) in post-stroke patients with claw toe. (2) Methods: Subjects with stroke-related leg paralysis/spasticity and claw toes received multiple injections of BoNT (onabotulinumtoxin A) into the FHL or FDL muscles. We investigated the relationship between the mode of transmission of FHL and FDL muscle tension to each toe (MCT) and treatment outcome using the data of 53 patients who received 124 injections with clinically recorded treatment outcome. We also dissected the potential variables that could determine the treatment outcome. (3) Results: The effectiveness of BoNT treatment was significantly altered by FDL-MCT (OR = 0.400, 95% CI = 0.162-0.987, p = 0.047). Analysis of the response to the first BoNT injection showed an odds ratio of FDL-MCT of approximately 6.0 times (OR = 0.168, 95% CI = 0.033-0.857, p = 0.032). The more tibial the influence of the FDL muscle on each toe, the better the treatment outcome on the claw toe. (4) Conclusions: The anatomic relation between FDL muscle and each toe seems to affect the response to treatment with BoNT in post-stroke patients with claw toes.

Spontaneous platelet aggregation in normal subject assessed by a laser light scattering method: an attempt at standardization.

Platelet aggregometry by the laser light scattering (LS) method is sufficiently sensitive to detect small platelet aggregates that form spontaneously in vitro in the absence of agonists. Platelet aggregation without agonists is named spontaneous platelet aggregation (SPA). Since SPA has been suggested to be associated with various thrombotic diseases, it is essential to measure SPA and to establish a standard range of SPA values. In this study, we measured SPA in 167 healthy subjects by the LS method and attempted to clarify various factors influencing SPA, including the blood collection procedure. We also attempted to establish a tentative standard range of SPA values. SPA was quantitatively measured in terms of the maximum total LS intensity, which reflects small aggregates formed over 10 minutes (SMAX) and the area under the total LS intensity curve of small aggregates (SAUC). Since both the values of SMAX and SAUC were skewed and the log SMAX and log AUC values showed a normal distribution, the statistical analyses were performed using log SMAX and log SAUC. The log SMAX and log SAUC were significantly higher in the samples collected using a tourniquet and/or a 21 G needle, than in those collected without a tourniquet and/or with an 18 G needle. The log SAUC values were significantly lower in samples obtained with a syringe and/or 3.8% sodium citrate than in those obtained in vacuum sampling tubes and/or 3.13% or 3.14% sodium citrate. The Ht and plasma glucose concentration influenced the log SMAX values. We propose that to standardize SPA measurements, the measurements should be completed within two hours of blood sample collection and collected using the regular concentration of citrate. The standard range of SMAX values measured in samples obtained using a tourniquet and a 21 G needle was 2.0-23.99 (*10(3) mV*count). The standard range of SAUC values measured under same conditions was 0.58-9.12 (*10(6) mV*count*min). The standard range of SMAX values measured in samples obtained using a tourniquet, 21 G needle and a vacuum tube was 1.7-29.51 (*10(3) mV*count). The standard range of SAUC values measured under same conditions was 0.59-9.33 (*10(6) mV*count*min).

[Analysis of brain images in patients with spinocerebellar degeneration; using statistical parametric mapping (SPM) and easy Z score imaging system (eZIS)].

In order to investigate the cerebral blood flow objectively, the easy Z score imaging system (eZIS), was developed, and has been applied in clinical practice. SPECT with 99mTc-ethyl cysteinate dimer (99mTc-ECD) was performed, and the images were analyzed using the SPM97 and the eZIS Ver. 2 to investigate cerebral blood flow in patients with two types of spino-cerebellar degeneration. We compared the distribution of cerebral blood flow between 13 patients with cortical cerebellar atrophy (CCA) and 26 patients with olivopontocerebellar atrophy (OPCA). In the both groups, cerebellar blood flow was decreased generally. In our evaluation using the eZIS Z score, the scores for the brain stem and cerebellar nucleus in the OPCA group were lower than those in the CCA group. This method facilitates the objective evaluation of cerebral blood flow in patients with spinocerebellar degeneration, and may be useful for analyzing the condition of these disease.

[A case of ventriculitis with bacterial meningitis occurred during the treatment of liver abscess].

A 47-case-year old male was admitted to our hospital because of high fever and general fatigue. He had no immune deficiency, and had no other disease in his past history. On admission, the white blood cell count and C-reacted protein were severely elevated (18,700/microliter, 27.7 mg/dl, respectively) and abdominal CT revealed multiple low density, From these results, he was diagnosed as liver abscess. Intravenous MINO and SBT/CPZ injection were started. On the fifth hospital day, he suffered from headache and nuchal rigidity. The clinical data revealed the cerebro-spinal fluid (CSF) counting 8,336 cells/mm3 (mononuclear 8,000,) protein at 119 mg/dl, and sugar 42 mg/dl. CSF cultures were negative, but Klebsiella was recognized in the blood culture and drainage fluid in liver abscess. This condition was diagnosed as bacterial meningitis and antibiotics were changed to intravenous CTRX and MEPM. Furthermore we administered oral PSL and intravenous steroid-pulse therapy. After these combination therapies his condition improved gradually. After 40 hospital day, however, he suddenly had double vision, Axial FLAIR (SE6,000/120) image revealed with high signal intensity at 4th ventricle. Intravenous MEPM was administered again. On the 60th hospital day, double vision was gradually improved and abnormal intensity at 4th ventricle was almost disappeared. This case may provide us a considerable suggestion on the treatment of bacterial meningitis.

[A case of a middle-age man with equinovarus movement at the end of the swing phase of walking].

We present a case with walking-induced equinovarus caused by dystonic contraction of the left ankle. This abnormal motor behavior occurred only when the patient walked, more specifically when the left leg was at the end of the swing phase. Surface electromyography confirmed continuous abnormal discharges in the left gastrocnemius muscles during the swing phase of walking. Brain and spinal magnetic resonance imagings (MRIs) were normal. The patient was unresponsive to drug therapy. However, the symptom reduced by the attachment of the short leg brace. From the effectiveness of the brace in normalizing, the patient was diagnosed with task-specific focal dystonia of the left leg.