Neurosyphilis with mesiotemporal magnetic resonance imaging abnormalities.

We describe a patient with mesial temporal T2-weighted image hyperintensity on magnetic resonance imaging that mimicked paraneoplastic limbic encephalitis. The patient showed pupillary abnormalities suggestive of a diagnosis of neurosyphilis, and the diagnosis was supported by the results of a serum Treponema pallidum hemagglutination assay (TPHA) and cerebrospinal fluid examination. Making a diagnosis of neurosyphilis is occasionally difficult because of the variety of clinical and imaging findings. Appropriate diagnosis and commencing adequate treatment are needed for a good prognosis; thus, neurosyphilis should be included in the differential diagnosis of mesiotemporal magnetic resonance imaging abnormalities.

Survival rate of patients with amyotrophic lateral sclerosis in Wakayama Prefecture, Japan, 1966 to 2005.

To investigate longitudinal changes in the survival rate of patients with amyotrophic lateral sclerosis in Wakayama Prefecture, Japan, we made a retrospective hospital-based study of 454 patients diagnosed with motor neuron disease (MND) at Wakayama Medical University (WMU) Hospital between 1966 and 2005. Of the 454 patients, 240 who were born and who lived in Wakayama Prefecture were diagnosed with definite or probable ALS during this period, according to the El Escorial criteria. The clinical data of the 240 patients, including sex, birth date, birthplace, address, age at onset, initial symptoms, date when respiratory support was applied (tracheostomy, noninvasive positive pressure ventilation, or mandatory artificial ventilation), and date of death were reviewed retrospectively. The age at onset of patients who developed initial symptoms before 1990 was 53.4+/-10.6 (mean+/-S.D.) and that in 1990 or thereafter was 64.8+/-10.3, respectively, showing a significant difference (p<0.0001). Clinical duration was determined from onset to either date of death or initiation of respiratory support in this study. Survival rate was compared using the Kaplan-Meier method according to age at onset, sex, initial symptoms and year of onset. Mean age at onset shifted towards older age according to a later year of onset, due to the overwhelming senility rate in Wakayama Prefecture. Older onset patients had a significantly poorer survival rate than younger onset patients when it was compared based on 10-year age groups (log rank, p<0.0001). Male patients had a poorer survival rate than female patients (p<0.0001). ALS patients with bulbar palsy onset showed shorter clinical durations than those with lower leg onset (p<0.0071, Breslow-Gehan-Wilcoxon test). Patients over 70 years old more frequently showed bulbar palsy onset compared to those younger than 69 (p=0.003). In a comparison of year of onset before and after 1990, ALS patients after 1990 had characteristics of older age onset and shorter clinical duration, and more frequently showed bulbar palsy onset compared with those before 1990. These findings indicated that younger onset patients with ALS decreased after 1990 in Wakayama Prefecture and this might partly explain the recent decline of ALS incidence in Wakayama Prefecture. The shift of the mean age at onset to older age might be due to exogenous factors, including changes in lifestyle, food, and drinking water in this area. Bulbar palsy onset and age at onset were expected as predictors of the survival rate.

Effects of zonisamide on experimental tremors in rats.

OBJECTS: To study the effect of zonisamide on experimental tremors in rats. METHODS: Effect of zonisamide on harmaline- or oxotreorine-induced tremors, and tacrine-induced tremulous jaw movements (TJMs) was studied. RESULTS: Zonisamide significantly suppressed both harmaline- and oxotremorine-induced tremors dose-dependently. Zonisamide also significantly suppressed tacrine-induced TJMs, and this effect was not lost under conditions of monoamine-depletion or dopaminergic blockade. CONCLUSION: The anti-tremor effects of zonisamide may be achieved by a non-dopaminergic mechanism. Since it effectively suppressed tremors that are based on different kinds of tremors, we propose a novel perspective of clinical potential of zonisamide as a non-specific, anti-tremor drug.

Transcranial sonography of the substantia nigra in Japanese patients with Parkinson's disease or atypical parkinsonism: clinical potential and limitations.

OBJECTIVE: There is growing interest in the use of transcranial sonography (TCS) of the substantia nigra (SN) in patients with Parkinson's disease (PD), as it has been reported that SN hyperechogenicity may be present in about 90% of PD patients. However, TCS of the SN has not been applied in Japanese patients, and its clinical potential has not been determined. PATIENTS AND METHODS: TCS of the SN was performed in patients with PD, progressive supranuclear palsy (PSP), multiple system atrophy (MSA), and essential tremor (ET), and age-matched controls. Ultrasound images of the SN were assessed using semi-qualitative estimation criteria by two investigators unaware of clinical diagnosis. RESULTS: SN hyperechogenicity was observed in approximately 83% of accessible SNs in Japanese PD patients. In comparison, SN hyperechogenicity was less frequently observed in healthy subjects or in patients with PSP, MSA, and ET. However, the rate of successful recording of the SN by TCS decreased prominently with advancing age, particularly in females. CONCLUSION: The present study confirmed that TCS of the SN is potentially useful in the investigation of Japanese patients, and it provides a better differential diagnosis between PD and atypical parkinsonism. The recording failure of TCS in aged, particularly female subjects, may limit the clinical potential of TCS of the SN in Japanese patients.

[Amyotrophic lateral sclerosis associated with extrapyramidal symptoms or signs].

This investigation was conducted to clarify the frequency and characteristics of ALS associated with extrapyramidal symptoms or signs in Wakayama prefecture. The questionnaires to survey ALS cases were mailed to all medical centers in Wakayama prefecture. A total of 252 cases were found to have motor neuron diseases. Among them, 204 cases fulfilled probable or definite according to El Escorial Criteria. In 10 of them, extrapyramidal signs were identified as follows: rigidity 50%, tremor 40% and akinesia 10%. Family history of ALS in these cases (20%) is higher than expected in usual ALS, and all of them are negative for SOD-1 mutation. Dementia and autonomic nervous symptoms were observed in several cases. Incidence of extrapyramidal signs in ALS resulted in 4.8%. The incidence of extrapyramidal signs is more frequent than expected by chance, suggesting that the degeneration of basal ganglia and/or substantia nigra may not be so rare in ALS.

[Inclusion body myositis associated with chronic thyroiditis, Sjögren's syndrome and autoimmune cholangitis].

A 67-year-old woman was admitted to our hospital, because of wasting of the thigh muscles. Muscular atrophy was confined to the thigh muscles, suggesting that she had quadriceps myopathy. Muscle biopsy from quadriceps muscle revealed characteristic findings, such as invasion of the endomysium and muscle fibers by inflammatory cells, the presence of rimmed vacuoles and ragged red fibers; thereby, she was diagnosed as having inclusion body myositis (IBM). Based on elevated titers of autoantibody as well as biopsy findings from salivary gland and liver, she was also diagnosed as having chronic thyroiditis, Sjögren's syndrome and autoimmune cholangitis. Currently, the pathogenic mechanism underlying IBM is not clarified, however, various factors have been suggested to contribute to it, such as viral infection or mitochondrial insufficiency. Although the pathogenic backgrounds underlying IBM are likely heterogeneous, an autoimmune-mediated mechanism may be related to the pathogenesis of IBM in the present patient.