[A patient of ADEM with central and peripheral conduction block improved with ultra-high-dose methylprednisolone].

A patient with exanthem and fever showed progressive disturbance of consciousness and flaccid quadriplegia predominantly in the lower extremities. Antibiotics, aciclovir, high-dose methylprednisolone (1 g/day for 3 consecutive days) and IVIG (400 mg/kg/day for 5 consecutive days) were not effective. Nerve conduction study and SEP in the lower extremities showed peripheral and central conduction block. EEG showed irregular sharp and slow waves predominantly in the left hemisphere. ABR and SEP in the upper extremities were normal. Consecutive studies of cranial and spinal MRIs showed no abnormalities. A diagnosis of acute disseminated encephalomyelitis (ADEM) was made. We started administration of ultra-high-dose methylprednisolone (5.4 mg/kg/h for 47 hours), the dose for acute spinal cord injury based on the randomized controlled trial of The Third National Acute Spinal Cord Injury Study in the USA. After this, she regained consciousness and the quadriplegia improved. The abnormalities in the electrophysiological studies also normalized. It is thought that the neuroprotective mechanism of ultra-high-dose methylprednisolone could be attributed to its inhibition of lipid peroxidation, secondary, ischemia, energy failure and so on. If the usual treatment is not effective for severe encephalomyelitis cases, we can consider the administration of ultra-high-dose methylprednisolone as one of the new treatment options.

[A case of retrobulbar optic neuritis associated with Sjögren syndrome which remitted spontaneously].

A 21-year-old woman came to the department of ophthalmology because of decreased visual acuity detected at the driving license center. MRI revealed T 1 iso-, T 2 hyper-intense, and Gd-enhanced lesion in the left optic nerve adjacent to the optic chiasma. No autoantibodies indicating systemic lupus erythematosus, Sjögren syndrome, or anti-phospholipid syndrome were elevated except for antinuclear antibody. There were no cerebral and spinal lesions on MRI. Neither oligoclonal IgG band nor myelin basic protein was found in the cerebrospinal fluid. Although she did not complain of dry mouth, secretion of the saliva was decreased. Biopsy of the minor salivary gland showed sialadenitis with infiltration of mononuclear cells, suggesting subclinical Sjögren syndrome. Visual acuity and MRI findings were remitted spontaneously. This case is distinguished from multiple sclerosis and vasculitis or ischemic mechanism(s) associated with autoantibodies might play a role in the development of the disease.

Median neuropathy at the wrist as an early manifestation of diabetic neuropathy.

AIMS/INTRODUCTION: To elucidate the clinical significance of median neuropathy at the wrist (MN) in patients with diabetes. MATERIALS AND METHODS: In total, 340 patients with diabetes who were hospitalized for glycemic control were enrolled in the present study. The diagnoses of MN and diabetic polyneuropathy (DPN) were based on electrophysiological criteria. A total of 187 patients were divided into four subgroups: patients without MN or DPN; patients with MN without DPN; patients with MN and DPN; and patients with DPN without MN. Intergroup comparisons of clinical characteristics and results of nerve conduction studies were carried out. RESULTS: A total of 71 patients had neither MN nor DPN; 25 had MN, but no DPN; 55 had MN and DPN; and 36 had DPN, but no MN. In comparison with the MN and DPN group, the MN without DPN group included more patients in the early phase of diabetes (diagnosed within the past 5 years) and fewer patients with diabetic microangiopathy. Comparative median nerve conduction studies showed significantly lower motor and sensory nerve conduction velocities, longer F-wave latencies, and smaller sensory nerve action potentials in patients with MN and DPN than in those without DPN. CONCLUSIONS: MN in patients with diabetes could be attributed to an impairment in axonal function at common entrapment sites, and could be used to identify an early manifestation of diabetic neuropathy.

Intraepidermal nerve fiber density and nerve conduction study parameters correlate with clinical staging of diabetic polyneuropathy.

AIM AND METHODS: To assess the usefulness of the diagnostic and staging criteria for diabetic polyneuropathy (DP) by the Diabetic Neuropathy Study Group in Japan (DNSGJ) we examined clinical features, intraepidermal nerve fiber densities (IENFD) and nerve conduction studies (NCS) and coefficient of variation of the R-R intervals (CVR-R) in 44 patients with diabetes. RESULTS: The patients were classified into stage I (n=20), II (n=6), III+IV (n=12), and V (n=6) according to the staging criteria by DNSGJ. IENFD decreased as stages progressed (13.8±7.1 fiber/mm in stage I to 0.8±1.3 fiber/mm in stage V). Compound motor and sensory action potential and motor and sensory nerve conduction velocity decreased with progressing stage. F-wave latency prolonged as stages progressed. CVR-R decreased with progressing stage (4.41%±2.65% in stage I to 1.33%±0.57% in stage V). IENFD correlated with the various parameters of NCS (r=0.378-0.636, p<0.05) and CVR-R (r=0.399, p=0.007). CONCLUSIONS: Clinical staging for DP by DNSGJ reflects the results of small and large fiber neuropathy.

Serum VEGF--as a prognostic factor of atherosclerosis.

Vascular endothelial growth factor (VEGF) has been noted in the pathogenesis of atherosclerosis. To examine the usefulness of the serum concentration of VEGF as an index of atherosclerosis, we analyzed the serum VEGF concentrations in 443 adults who underwent a medical checkup. The mean serum VEGF concentration of men (229+/-147 pg/ml) was significantly higher than that of women (182+/-112 pg/ml). The platelet count showed a slight correlation with the serum VEGF concentration in both genders (men R=0.287, women R=0.296), corresponding with the results of experiments that platelets are the major source of VEGF in circulating peripheral blood. In men, the serum VEGF concentrations correlated with platelet counts, body fat percentages, leukocyte counts, and HDL-cholesterol concentrations (negative correlation). In the multiple regression analysis performed for men's serum VEGF concentrations, the decision coefficient (R2) was maximized (R2=0.173) when the leukocyte count, the body fat percentage, and the HDL-cholesterol concentration were taken into account besides the platelet count. Male smokers' serum VEGF concentrations were higher than non-smokers'. Smoking in men significantly affected the sex difference in the serum VEGF concentration, leukocyte count, and HDL-cholesterol concentration. We concluded that the serum VEGF concentration might be closely related to atherosclerosis accelerating factor, especially in men.