Promomycin, a polyether promoting antibiotic production in Streptomyces spp.

Widespread interspecific stimulation of antibiotic production occurs in strains of Streptomyces owing to the activity of diffusible substances, as previously determined in our investigations of the cross-feeding effect. In this study, we newly isolated a substance produced by a Streptomyces strain closely related to Streptomyces scabrisporus, based on the observation that this substance induced the production of an unknown antibiotic in another strain related to Streptomyces griseorubiginosus. This substance, named promomycin, is a polyether structurally related to lonomycin. Promomycin itself had an antibiotic activity, but it stimulated antibiotic production in multiple Streptomyces strains at sub-inhibitory concentrations. Evidence implies that this stimulation effect is widespread within this group of bacteria.

Hypoxia-inducible factor 1alpha may be a marker for vasculitic neuropathy.

Neuromuscular biopsy is still an essential method for diagnosing vasculitic neuropathy, although its diagnostic sensitivity is at most 60%. Our objective was to examine the expression of hypoxia-inducible factor 1alpha (HIF-1alpha) in peripheral nerves and to evaluate its usefulness in diagnosing vasculitic neuropathy, especially for discrimination from other axonal neuropathies. Forty-one patients with vasculitic neuropathy consisting of 20 definite, 14 probable and seven possible diagnoses, 15 patients with metabolic neuropathy, five with motor neuron disease and six with chronic inflammatory demyelinating polyneuropathy were included. Nerve biopsy specimens were immunohistochemically examined for HIF-1alpha and various cell markers. Distinct immunoreactivity (IR) was observed in nuclei of endoneurial cells in 54% (22/41) of vasculitic patients, while specimens from metabolic neuropathies showed less nuclear IR and the difference of mean density of HIF-1alpha-positive nuclei was significant. Two patients with possible vasculitis who showed HIF-1alpha-positive nuclei in endoneurium, were later confirmed to have vasculitis by skin biopsies. Most of the cells expressing HIF were demonstrated to be Schwann cells. There was a trend in the vasculitic patients with early phase nerve damage to display higher endoneurial HIF-1alpha-IR. HIF-1alpha may be an immunohistochemical marker for vasculitic neuropathy, especially when the observed section contains no vasculitic lesions.

Gluten sensitivity in Japanese patients with adult-onset cerebellar ataxia.

OBJECTIVE: Gluten sensitivity is associated with multiple neurological abnormalities including gluten ataxia, motor neuron disease-like neuropathy, small fiber type neuropathy, cognitive impairment, and even parkinsonism. We investigated whether or not gluten sensitivity is involved in Japanese patients with idiopathic cerebellar ataxia with extracerebellar presentation. PATIENTS OR MATERIALS: Fourteen patients with idiopathic cerebellar ataxia with extracerebellar presentation (autonomic instability, parkinsonism, or pyramidal dysfunction in varying combinations) were screened for anti-gliadin antibodies (AGA) to analyze for the presence or absence of gluten sensitivity. Patients with typical MR findings of multiple system atrophy of the cerebellar type were excluded. As disease controls without cerebellar ataxia, 9 patients with Parkinson's disease and 18 patients with amyotrophic lateral sclerosis were screened for AGA. Forty-seven normal controls were also screened for AGA. RESULTS: We found a high prevalence of AGA in 5 (36%) of 14 cerebellar ataxia patients, but in only 1 (4%) of 27 disease controls without cerebellar ataxia (odds ratio, 14.4; 95% CI, 1.41147; p<0.05) and in only 1 (2%) of 47 normal controls (odds ratio, 25.6; 95% CI, 2.66246; p<0.001). Among the cerebellar ataxia patients, atypical features such as sensorimotor neuropathy and/or mild cognitive impairment were more prevalent in the AGA-positive group (60%) than in the AGA-negative group (0%). In one of the ataxic patients with AGA, a gluten-free diet had positive effects on neurological symptoms and nutritional status. CONCLUSION: Gluten sensitivity is involved in at least some of the unexplained neurological symptoms of Japanese patients with adult-onset, sporadic cerebellar ataxia.

Hyperostosis frontalis interna associated with hypogonadism in an elderly man.

Hyperostosis frontalis interna (HFI), symmetric thickening of the inner table of the frontal bone, is relatively common in women but very rare in men. We report the case of an elderly male patient with HFI. This patient was accompanied by primary hypogonadism, which may be related to the underlying pathogenesis of HFI.

[Falls in peripheral neuropathy].

Falling accidents are predisposing factors in worsening the quality of life. Causes of falls included gait and balance problems, visual disorder, cognitive impairment and drugs. However, falls in neuropathic patients are not often investigated. We studied the actual condition and causes of falls associated with neuropathy. One hundred and ninety-three patients (122 males and 71 females, ages 57 +/- 15 years) with neuropathy were selected for evaluation. In all patients, more than half a year had passed since the onset of symptoms and the present condition was considered comparatively stable. We divided the patients into 2 groups: falling or non-falling. Patients experiencing accident accompanied by external injury once or more in the past year, or patients who answered "often fell" (1 time or more per month) on flat ground or in their residence comprised the fall group. The severity of disease was evaluated by modified Rankin Scale (0 to 5). The relationship between falls and neuropathic symptoms (proximal muscle weakness, distal muscle weakness, aching or numbness, and the position and vibration sensory loss) was statistically examined. The distribution of patients in the fall group according to modified Rankin Scale of neuropathy was scale 5: 0/0 (0%), scale 4: 5/36 (14%), scale 3: 24/72 (33%), scale 2: 7/56 (13%), and scale 1: 1/19 (5%). There were six fractures among all patients. Regarding the etiology, the fall group showed a high level of axonal neuropathies (44%). On analysis according to condition and symptoms in the patients with a score of 2, 3, or 4 who demonstrated a high rate of falls, there was a strong correlation between deep sensory loss and fall (p < 0.05). However, as independent factors, there were no correlations between falling and proximal muscle weakness, distal muscle weakness or aching numbness, respectively. We thought it necessary to add ataxic gait resulting from deep sensory loss to one of the fall risks.

Amyotrophic lateral sclerosis with IgM antibody against gangliosides GM2 and GD2.

We report a case of amyotrophic lateral sclerosis (ALS) with IgM antibody against gangliosides GM2 and GD2. A 57-year-old woman presented with slowly progressive muscular weakness of the upper extremities and dysarthria. She fulfilled the clinical and electrophysiological criteria of ALS, and died from sudden suffocation about 3 years after the onset of illness. The patient's serum IgM antibody was shown to recognize the structure shared by GM2 and GD2. Since anti-GM2 antibodies have been implicated in motor neuropathy or motor neuron syndrome, this rare case might contribute to the understanding of the immunological aspects of ALS.