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Objective: To describe the heterogeneity of electrodiagnostic (EDx) studies in Guillain-Barre syndrome (GBS) patients collected as part of the International GBS Outcome Study (IGOS). Methods: Prospectively collected clinical and EDx data were available in 957 IGOS patients from 115 centers. Only the first EDx study was included in the current analysis. Results: Median timing of the EDx study was 7 days (interquartile range 4-11) from symptom onset. Methodology varied between centers, countries and regions. Reference values from the responding 103 centers were derived locally in 49%, from publications in 37% and from a combination of these in the remaining 15%. Amplitude measurement in the EDx studies (baseline-to-peak or peak-to-peak) differed from the way this was done in the reference values, in 22% of motor and 39% of sensory conduction. There was marked variability in both motor and sensory reference values, although only a few outliers accounted for this. Conclusions: Our study showed extensive variation in the clinical practice of EDx in GBS patients among IGOS centers across the regions. Significance: Besides EDx variation in GBS patients participating in IGOS, this diversity is likely to be present in other neuromuscular disorders and centers. This underlines the need for standardization of EDx in future multinational GBS studies.(c) 2022 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).

Summary Background Despite the introduction of plasmapheresis and immunoglobulin therapy, many patients with Guillain-Barre syndrome still have an incomplete recovery. Evidence from pathogenesis studies suggests the involvement of complement-mediated peripheral nerve damage. We aimed to investigate the safety and efficacy of eculizumab, a humanised monoclonal antibody against the complement protein C5, in patients with severe Guillain-Barre syndrome. Methods This study was a 24 week, multicentre, double-blind, placebo-controlled, randomised phase 2 trial done at 13 hospitals in Japan. Eligible patients with Guillain-Barre syndrome were aged 18 years or older and could not walk independently (Guillain-Barre syndrome functional grade 3–5). Patients were randomly assigned (2:1) to receive 4 weeks of intravenous immunoglobulin plus either eculizumab (900 mg) or placebo; randomisation was done via a computer-generated process and web response system with minimisation for functional grade and age. The study had a parallel non-comparative single-arm outcome measure. The primary outcomes were efficacy (the proportion of patients with restored ability to walk independently [functional grade ≤2] at week 4) in the eculizumab group and safety in the full analysis set. For the efficacy endpoint, we predefined a response rate threshold of the lower 90% CI boundary exceeding 50%. This trial is registered with ClinicalTrials.gov, number, NCT02493725. Findings Between Aug 10, 2015, and April 21, 2016, 34 patients were assigned to receive either eculizumab (n=23) or placebo (n=11). At week 4, the proportion of the patients able to walk independently (functional grade ≤2) was 61% (90% CI 42–78; n=14) in the eculizumab group, and 45% (20–73; n=5) in the placebo group. Adverse events occurred in all 34 patients. Three patients had serious adverse events: two in the eculizumab group (anaphylaxis in one patient and intracranial haemorrhage and abscess in another patient) and one in the placebo group (depression). The possibility that anaphylaxis and intracranial abscess were related to eculizumab could not be excluded. No deaths or meningococcal infections occurred. Interpretation The primary outcome measure did not reach the predefined response rate. However, because this is a small study without statistical comparison with the placebo group, the efficacy and safety of eculizumab could be investigated in larger, randomised controlled trials. Funding The Japan Agency for Medical Research and Development, Ministry of Health, Labor and Welfare, and Alexion Pharmaceuticals.

To investigate the diagnostic performance of brain acetylcholinesterase (AChE) activity measurement using N-[(11) C]-methyl-4-piperidyl acetate (MP4A) and PET in patients with dementia with Lewy bodies (DLB) and Alzheimer's disease (AD).Participants were 14 DLB patients, 25 AD patients and 18 age-matched healthy controls (HC). All subjects underwent PET scans and MP4A to measure regional brain AChE activity. We performed anatomical standardization of each brain image, and k3 values, an index of AChE activity, in each voxel were estimated by nonlinear least squares analysis. Volumes of interest (VOIs) were identified on parametric k3 images in frontal, temporal, parietal and occipital cortices, and in anterior and posterior cingulate gyri (ACG and PCG). In each VOI, the differential diagnostic performance between AD and DLB of k3 values was assessed by area under the curve (AUC) of the receiver-operating characteristic. Voxel-based statistical analyses were also performed.Mean cortical AChE activities in AD patients (-8.2% compared with normal mean) and DLB patients (-27.8%) were lower than HCs (p 0.05, p 0.001, respectively). There was a significant difference in mean cortical AChE activities between AD and DLB patients (p 0.001). All regional brain AChE activities of defined VOIs except ACG were able to well discriminate DLB from AD, and notably performance was the most significant in PCG (AUC = 0.989, 95% CI: 0.965-1.000).Brain cholinergic deficit is consistently prominent in DLB compared with AD. PET measurement of brain AChE activity may be useful for the differential diagnosis between DLB and AD.

It is usually difficult to distinguish between idiopathic Parkinson's disease (PD) and parkinsonian-type multiple system atrophy (MSA-P) in the early stage. However, it is important to make a careful early-stage diagnosis. Therefore, we determined whether an examination of pelvic organ dysfunction would be helpful to distinguish between PD and MSA-P.We recruited 61 patients with PD and 54 patients with MSA-P who were examined at our neurology clinic. The mean ages of the patients with PD and MSA-P were 67 and 64 years, respectively. The mean disease duration of both groups was 3.2 years. We administered a questionnaire on pelvic organ dysfunction to the PD and MSA-P groups. The questionnaire had sections focusing on bladder, bowel, and sexual function. Dysfunction, as described in the responses, was evaluated as normal, mild (once a month), moderate (once a week), or severe (once a day). The Mann-Whitney U-test was used for statistical analysis.Compared with the PD group, the prevalence and severity of pelvic dysfunction in the MSA-P group was significantly higher for urinary urgency (MSA-P 76%, PD 58%, P0.05), retardation in initiating urination (79%, 48%, P0.05), prolongation in urination (79%, 72%, P0.05), and constipation (58%, 31%, P0.05). The quality-of-life index among pelvic organ dysfunctions indicated that urinary and bowel function was significantly more impaired in the MSA-P group than in the PD group.Urinary urgency, retardation in initiating urination, prolongation in urination, and constipation are more prevalent and severe in MSA-P compared to PD.

Although the benefit of treatment for relapsing-remitting multiple sclerosis (MS) is firmly established, whether interferon beta-1b (IFNB-1b) therapy is efficacious for neuromyelitis optica (NMO) has been debated.We reviewed the responses to IFNB-1b treatment in 18 patients with relapsing NMO and compared the results with those from 38 patients with relapsing-remitting MS. We compared clinical characteristics, the annualized relapse rate (ARR) and the probability of being relapse free before and after IFNB-1b treatment in patients with NMO and MS.The proportion of patients with more than 50% increase in the ARR after IFNB-1b treatment was much higher in NMO than in MS (P = 0.046). ARR was significantly lower in patients with MS after IFNB-1b administration than before (P = 0.015), but not in NMO. Kaplan-Meier and log-rank statistical analyses revealed that relapse-free rates were lower in NMO than MS after IFNB-1b treatment (P = 0.032). The analyses also showed lower relapse-free rates during the pre-IFNB-1b treatment period than the post-IFNB-1b treatment period in MS (P0.001), but not in NMO.IFNB-1b treatment does not appear to be effective for preventing relapse in NMO likely because of differences between the immune-pathogenesis of NMO and MS.

We read with interest the recent article by Kuwabara et al.1 describing the clinical and electrophysiologic findings in 9 patients with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome and treated with autologous peripheral blood stem cell transplantation (auto-PBSCT). From May 2003 until September 2006, we treated 4 patients with POEMS syndrome with chemotherapy and auto-PBSCT.2 There were 3 men and 1 woman with a median age of 53 years (range 44–62). Three patients had severe and rapidly progressive sensory–motor peripheral neuropathy with predominant involvement of the lower limbs leading to inability to walk, reduced muscle strength of the upper limbs, absent deep tendon reflexes, and distal sensory impairment. One patient presented with a mild clinical picture of distal paresthesias and hypoesthesia in the lower limbs and normal muscle strength. The median time from first symptoms to diagnosis was 7 months (range 4–48 months). Before auto-PBSCT, 3 out of 4 patients received standard treatment for inflammatory neuropathy with no improvement. Time from the diagnosis to auto-PBSCT was 3 months. After a median follow-up …

The presence of a projection from the primary motor cortex to the ipsilateral muscles has been established in human, but whether this pathway contributes to functional recovery after stroke is unclear. We investigated whether the ipsilateral tract is activated in hemiparetic stroke.Motor-evoked potentials (MEPs) were simultaneously recorded from the bilateral trapezius or abductor digiti minimi (ADM) muscles after magnetic stimulation to the motor cortex in 40 acute stroke patients.At rest, ipsilateral trapezius MEPs were recordable in none of the 24 normal controls, and in 38% of the patients after stimulation to the non-affected hemisphere (P0.001). With voluntary contraction, ipsilateral trapezius MEPs were elicited in 21% of the normal controls and 73% of the patients (P0.001). Ipsilateral ADM MEPs were rarely recordable in both controls (0%) and patients (3%). The presence of ipsilateral trapezius MEPs was associated with less severe paresis in the trapezius (P = 0.04) and deltoid (P = 0.07), but not in the more distal muscles.The ipsilateral cortico-spinal tract is acutely facilitated after stroke in the trunk or proximal muscles, but not in the hand muscles. Activation of such pathway appears to partly compensate motor dysfunction of the trunk/proximal muscles.

Beneficial treatment has not been established for chronic sensory ataxic neuropathy associated with Sjögren's syndrome (CSAN-SS). We describe two patients with CSAN-SS who clinically improved in response to D-penicillamine treatment. Their neuropathic symptoms were lessened after D-penicillamine, and the results of electrophysiologic studies support the clinical improvement. D-Penicillamine can be considered a potentially beneficial agent in the treatment of CSAN-SS.

A 72-year-old male underwent coronary artery bypass grafting for 3 vessels disease. After the operation, the peak C-reactive protein (CRP) level was 0.85 mg/dl and CRP levels stayed very low. Forty days after the operation the patient developed a progressive median chest wound dehiscence but the bacterial cultures were negative. In spite of conventional therapies such as debridement of necrotic tissue or irrigation, the wound granulation was underdeveloped. After 6 months an epidermis had developed to cover the wound.

To investigate whether or not the pattern and extent of autonomic involvement differ between the two subtypes of Guillain-Barré syndrome (GBS), namely acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN).Head-up tilt test, R-R interval variation, plasma noradrenaline concentration, skin vasomotor reflex (SVR) and sympathetic sweat response (SSwR) were used to estimate autonomic function in seven AIDP and eight AMAN patients.Heart rate and plasma noradrenaline concentration were significantly high in the AIDP group but not in the AMAN group. Skin vasomotor reflexes were generally preserved and SSwRs were impaired in patients with severe neurological deficits for both AIDP and AMAN groups.The patterns of autonomic involvement are qualitatively different between AIDP and AMAN. Acute inflammatory demyelinating polyneuropathy is characterized by cardio-sympathetic hyperactivity, excessive or reduced sudomotor function and preserved skin vasomotor function, while AMAN is not necessarily generally associated with marked autonomic dysfunction except for the sudomotor hypofunction seen in patients with severe neurological deficits.

To explore the possible histogenesis of superficially spreading carcinoma of the esophagus, the clinicopathological features of these tumors (n = 44) were compared with those of ordinary superficial carcinoma (n = 163). Tumors of a heterogeneous histological type and having in situ carcinoma components were significantly more common (P.05), and the number of residual squamous islands was significantly greater (P0.05) in the former group than the latter. Furthermore, the tumor size was not different among in situ, intramucosal, and submucosal carcinomas of the former, whereas the tumors became larger according to the depth of invasion in the latter group. These results indicate that the collision of multiple simultaneously developing superficial tumors is a plausible histogenesis of superficially spreading carcinoma of the esophagus.

To clarify the relations of the axonal form of Guillain-Barré syndrome (GBS) to anti-ganglioside antibodies and Campylobacter jejuni infection, 86 consecutive Japanese GBS patients were studied. Electrodiagnostic criteria showed acute inflammatory demyelinating polyneuropathy in 36% of the patients and acute motor axonal neuropathy (AMAN) in 38%. Frequent anti-ganglioside antibodies were of the IgG class and against GM1 (40%), GD1a (30%), GalNAc-GD1a (17%), and GD1b (21%). Identified infections were C. jejuni (23%), cytomegalovirus (10%), Mycoplasma pneumoniae (6%), and Epstein-Barr virus (3%). There was a strong association between AMAN and IgG antibodies against GM1, GD1a, GalNAc-GD1a, or GD1b. Almost all the patients with at least one of these antibodies had the AMAN pattern or rapid resolution of conduction slowing/block possibly because of early-reversible changes on the axolemma. C. jejuni infection was frequently associated with AMAN or anti-ganglioside antibodies, but more than half of the patients with AMAN or anti-ganglioside antibodies were C. jejuni-negative. These findings suggest that the three phenomena "axonal dysfunctions (AMAN or early-reversible conduction failure)," "IgG antibodies against GM1, GD1a, GalNAc-GD1a, or GD1b," and "C. jejuni infection" are closely associated but that microorganisms other than C. jejuni frequently trigger an anti-ganglioside response and elicit axonal GBS.

To examine the validity of electrodiagnostic tests to help diagnosis and to evaluate disease activity of juvenile atrophy of unilateral upper limb (Hirayama's disease), we reviewed the results of nerve conduction studies, motor unit number estimates, electromyography, and somatosensory evoked potentials in consecutive 38 patients. There was selective involvement of the C8-T1 myotomes, in which motor unit loss was more prominent in the abductor digiti minimi than in the abductor pollicis brevis muscles, and F wave persistency was decreased out of proportion to the motor unit loss. These findings may help diagnosis. With respect to the disease activity and treatment, denervation potentials in the flexor carpi radialis muscle were more frequent in patients with shorter disease duration than those with longer duration. Median F wave persistency was greater in patients treated with neck brace than in patients without the treatment. These parameters can monitor the disease activity and efficacy of treatment.

To investigate the pathophysiological role of anti-GM1 antibody in Guillain-Barré syndrome (GBS), we reviewed sequential nerve conduction studies of 345 nerves in 34 GBS patients. Statistically significant correlation between IgG anti-GM1 antibodies and electrodiagnoses was found. Sixteen IgG anti-GM1-positive patients were classified as having acute motor or acute motor sensory axonal neuropathy (AMAN or AMSAN) (12 patients), as having acute inflammatory demyelinating polyneuropathy (AIDP) (3 patients), or as undetermined (1 patient) by electrodiagnostic criteria. Besides axonal features, there was rapid resolution of conduction slowing and block. In 3 patients initially diagnosed as having AIDP, conduction slowing was resolved within days, and 1 of them and 3 AMAN patients showed markedly rapid increases in amplitudes of distal compound muscle action potentials that were not accompanied by prolonged duration and polyphasia. The time courses of conduction abnormalities were distinct from those in IgG anti-GM1-negative AIDP patients. Rapid resolution of conduction slowing and block, and the absence of remyelinating slow components, suggest that conduction failure may be caused by impaired physiological conduction at the nodes of Ranvier. Reversible conduction failure as well as axonal degeneration constitutes the pathophysiological mechanisms in IgG anti-GM1-positive GBS. In both cases, immune-mediated attack probably occurs on the axolemma of motor fibers.

We conducted a retrospective study on 711 patients with early gastric cancer. Twenty-two patients (4.0%) developed recurrent disease and 21 died of recurrence during this study. One hundred and sixty-three patients died of unrelated or unknown causes. The recurrence patterns of 22 patients were as follows: hematogeneous metastasis to the liver in 11 patients, peritoneal dissemination in 3, recurrence in the remnant stomach in 3, and in the distant lymphonodes in 1. The mean survival period of patients with recurrent disease was 50.4 months, and 7 of these patients (31.8%) died more than 5 years after surgery. A retrospective clinicopathologic evaluation of the 22 patients and 526 patients without recurrence revealed significant differences between the two groups with respect to mean age at the time of surgery (62.5 years in the recurrent group vs 57.3 years in the nonrecurrent group), tumor size (41.2 vs 30.6 mm), depth of invasion (submucosal cancer: 19 vs 256), lymphnode metastasis (11 vs 48), lymphatic (11 vs 89) and venous (7 vs 18) invasion, and operative curability (curability B: 8 vs 48). Three patients with intramucosal cancer who died of hematogenous and/or peritoneal recurrence within 7 years after surgery had neither lymphnode metastasis nor lymphatic or venous invasion, suggesting that new techniques are needed for prediction of recurrent disease in patient with early gastric cancer.

We described a-44-year old male patient with Isaacs' syndrome occurring 8 months before the recurrence of malignant thymoma. Electrophysiological examination suggested that spontaneous muscle activities were generated at the distal terminals of the motor nerves. Double filtration plasmapheresis alleviated the symptoms of neuromyotonia for a short term. Administration of valproic acid had more prolonged effects lasting for 2 months until his death from relapsed thymoma. Our results support that autoimmune mechanisms, especially humoral factors, are playing an important role in the pathogenesis of Isaacs' syndrome and that anti-convulsants therapy is important.

The aim of this study was to determine whether latent viral infection is associated with idiopathic pulmonary fibrosis (IPF), an interstitial lung disease whose aetiology remains to be elucidated. Cytomegalovirus (CMV) immunoglobulin G (IgG) and complement fixation (CF), Epstein-Barr (EB) viral capsid antigen (VCA) IgG, herpes simplex virus (HSV) IgG, adenovirus CF, and parainfluenza 3 virus haemagglutinin inhibition (HI) titres were measured in the serum from patients with pulmonary diseases. The study included five subject groups: 35 normal controls (aged (mean +/- SD) 38 +/- 17 yrs); 43 IPF (63 +/- 10 yrs), seven collagen vascular disease-related interstitial pneumonitis (CVD-IP) (62 +/- 12 yrs); 22 sarcoidosis (36 +/- 14 yrs); and 17 emphysema (66 +/- 11 yrs). Levels of CMV IgG in IPF (87.6 +/- 51.7) and CVD-IP (101.2 +/- 69.9) were significantly elevated compared to those in the control (30.9 +/- 24.1), sarcoidosis (34.4 +/- 38.3) and emphysema groups (40.3 +/- 24.6), whereas CMV immunoglobulin M (IgM) was generally below the limit of detection. Similarly, CMV CF titres in IPF and CVD-IP were elevated compared to those in other diseases. EB VCA IgG titres in IPF, CVD-IP and emphysema and HSV IgG in IPF were also elevated. In contrast, adenovirus CF and parainfluenza 3 HI titres demonstrated no significant difference among all of the groups investigated. Increases in cytomegalovirus immunoglobulin G and complement fixation titres with negative cytomegalovirus immunoglobulin M suggest that latent cytomegalovirus infection may be more prominent in idiopathic pulmonary fibrosis or collagen vascular disease-related interstitial pneumonitis. Together with the elevation of Epstein-Barr virus viral capsid antigen and herpes simplex virus immunoglobulin G in idiopathic pulmonary fibrosis and/or collagen vascular disease-related interstitial pneumonitis, it is rational to assume that these viruses may be implicated in the development of pulmonary fibrosis. Further study is necessary to investigate the relationship between latent viral infection and pulmonary fibrosis.

To assess the prevalences and neurological symptoms of destructive spondyloarthropathy (DSA) and other cervical spinal diseases in long-term hemodialysis, a questionnaire survey of the medical histories and subjective sensory-motor symptoms was taken on 191 patients undergoing dialysis for 0.2-23 years (mean 8.6 +/- 6.3) with a mean age of 56.6 +/- 11.8 years (range 23-86). Furthermore, plain radiographic examinations of the cervical spine were carried out in 90% of the patients, and neurologic examinations were also performed on 29 patients. DSA was diagnosed in 18 cases (10%): moderate narrowing of the intervertebral space (NIS-I) in 8, severe narrowing (NIS-II) in 18, ossification of posterior longitudinal ligaments (OPLL) in 6, cervical spondylosis (CS) in 12 and other abnormalities in 6; 104 cases were normal. NIS-I was considered to reflect an early stage of DSA. The age of patients with DSA (mean 61.5 +/- 10.2 years), as well as with CS, was significantly higher than the normal group (52.2 +/- 10.6; p0.001), but younger than CS (68.5 +/- 10.0). The duration of dialysis in DSA patients (mean 12.1 +/- 6.0 years) was the longest and significantly longer than normal (7.7 +/- 6.5; p0.01) and (:S patients (3.8 +/- 3.0; p0.001). Through comparative evaluations of prior histories of the diseases, such as diabetes mellitus, carpal tunnel syndrome, amyloid osteoarthropathy (AOA) and parathyroidectomy, along with large calcification of the nuchal soft tissues on plain films, it was found that DSA, in conjunction with NIS-I, correlated with only AOA (p0.05). Subjective sensory symptoms, which consisted chiefly of arthralgic type followed by polyneuritic type, were noted in 60% of all patients and 56% of DSA patients. No significant differences were evident between any two particular groups. The score of subjective motor symptoms of the lower extremities in 5 patients with myelopathy was significantly higher than that in 5 other patients without neurological disorders (p0.05). Thirty-eight percent of DSA patients showed myelopathy; the frequency was higher than those of previous similar studies (0 = 5%). In conclusion, this study demonstrated that 1) DSA occurred in 10% of dialysis patients, which was also evident in previous reports (9 = 10%); this prevalence was higher than those of CS or OPLL, 2) DSA correlated with the duration of dialysis and AOA, 3) subjective motor symptoms of the lower extremities could indicate presence of myelopathy whereas subjective sensory symptoms contributed little to differential diagnosis, and 4) a careful neurologic examination can more frequently disclose myelopathy in patients with DSA than so far believed.

We report a 23-year-old woman, who had a relapsing-remitting multiple mononeuropathy with multifocal nerve enlargement. The patient was characterized by asymmetrical, marked enlargement of multiple nerves in the arms (median and ulnar nerves) and neck (accessory nerve) bilaterally. Sequential nerve conduction studies revealed persistent demyelinative abnormalities, especially across the segment of nerve thickening. The MRI of the proximal arm confirmed the markedly enlarged median and ulnar nerves which showed high signal intensity on T2-weighted images and partial enhancement after administration of gadolinium. We consider that the patient had the clinical features of "multifocal pseudohypertrophic neuropathy" described by Adams et al, and that chronic demyelination and inflammation associated with impairment of the blood-nerve barrier might be underlying mechanisms.

A 56-year-old man was admitted to our hospital in November, 1991 because of hyperproteinemia and anemia. Total protein showed 12 g/dl and serum immunoglobulins were as follows; IgG 974 mg/dl, IgA 142 mg/dl, IgM 9270 mg/dl. M-component was identified as IgM-kappa with immunoelectrophoresis and serum viscosity indicated 6.9. Although the patient had no history of severe streptococcal infection, his serum showed very high activity of ASLO (6890 IU/ml). Bence Jones protein was detected in the urine and determined to be of kappa-type. Plasma cells occupied 43% of bone marrow nucleated cells and their cytoplasms were stained with FITC-labeled anti-IgM and anti-kappa antibodies. X-ray examination disclosed punched out lesions in the skull. Consequently, the patient was diagnosed as having IgM-myeloma. At first, VCAP regimens were given after plasmapheresis, but had no effect. Therefore, the patient was treated with CHOP protocol and the serum IgM decreased in amount and the proportion of bone marrow myeloma cells got down to 17%. ASLO titer also decreased in parallel with IgM. These findings suggest that IgM in this case had ASLO activity.

We studied the effect of double filtration and immunoadsorption plasmapheresis in Guillain-Barré syndrome retrospectively in 70 patients. Thirty three patients had plasmapheresis, of whom 23 had double filtration and 10 immunoadsorption. The rest of 37 patients consisted of a control group. Clinical disability was evaluated using the functional grading scale by Hughes. In patients having a maximum disability of grade 4 and 5, the double filtration plasmapheresis group showed statistically significant improvement at 3 months after the treatment. There was no significant difference in prognoses between the immunoadsorption plasmapheresis and control groups. In mildly-illed patients having grade 2 and 3, the prognoses were not different between the three groups. In Guillain-Barré syndrome, double filtration plasmapheresis, as well as well-established plasma exchange, had benefit for severely-illed patients.

Several attempts have been made to detect and retrieve fetal nucleated cells including nucleated erythrocytes (NRBCs), leukocytes, and trophoblasts in maternal blood. We have recently developed a new method for non-invasive fetal DNA diagnosis from maternal blood. Peripheral blood granulocytes including NRBCs were isolated by a discontinuous density gradient method using Percoll (Pharmasia). NRBCs were found and retrieved at a single cell level using a micromanipulator under a microscope. To determine whether the origin of the NRBCs was maternal or fetal, the NRBCs were analysed by polymerase chain reaction (PCR) amplification to determine the presence of a Y-chromosome-specific repeat sequence in mothers carrying male fetuses. We were successful in predicting fetal sex accurately in 10 out of 11 samples taken from maternal blood. This new technique opens up fetal DNA diagnosis from maternal blood during the first trimester of pregnancy to the whole population because there is no risk to the fetus or the mother.

We investigated excitability of cervical lower motoneurons in 18 patients with juvenile muscular atrophy of the distal upper limb (Hirayama's disease), by F wave analysis and electrophysiological estimation of the number of motor units in the abductor pollicis brevis muscles of the affected side. In all the 18 patients, F wave persistencies and numbers of motor units were decreased, whereas amplitudes of single motor unit potentials were increased, compared to those of age-matched normal controls. Patients in a progressive phase showed markedly decreased F wave persistencies, overwhelming the degree of decreases in numbers of motor units. In half of them, F waves were significantly increased in number during neck flexion. On the other hand, patients treated with neck brace and patients having stabilized symptoms with longer duration showed F wave persistence reduction corresponded to the degree of the motor unit loss, and no changes in F wave persistencies in a posture of neck flexion. These results suggest that the disorder is characterized by decreased excitabilities of survived cervical anterior horn cells, as well as denervation and reinnervation, presumably resulting from local compression or circulatory failure by anterior shift of the posterior dural wall during neck flexion.

We found an electromyographical proof of reconstruction of the motor nerve terminals following hyperbaric oxygen therapy. A 38-year-old man who had been partially recovered for thirty four months from acute onset paraplegia following a gastrointestinal infection developed progressive muscular atrophy and weakness of the lower limbs, and was first admitted to our hospital. Cerebrospinal fluid examination was normal and nerve conduction studies showed small compound muscle action potentials without an evidence of segmental demyelination. There were ample fibrillation potentials on electromyography. Single fiber electromyography (SFEMG) showed increased fiber density, abnormal jitter and blockings without neurogenic jitter, which were similar to findings in post-poliomyelitis syndrome. He was treated by hyperbaric oxygen consisting of two hour exposures to pressures of two atmospheres breathing 100% oxygen. These exposures continued for a month daily, and thereafter once a week for one year. Clinical improvement of the weakness and a decrease in amount of fibrillation potentials occurred on and after a month after treatment. We found significant changes on SFEMG a year later. There were increased fiber densities and decreased mean values of consecutive differences. These changes indicate diminished degeneration and enhanced regeneration of the terminal axons. We think that hyperbaric oxygen has a beneficial effect on oxygen metabolism of remaining motoneurons which may not be able to maintain excessive metabolic demands of all their sprouting axons.

The authors report a case of cavernous hemangioma in the occipital region, which resembled sinus pericranii, protruded in the recumbent posture. A 28-year-old male was admitted with a chief complaint of an occipital fluctuating mass, 5cm in diameter, accompanied by slight pain. The skull X-P was normal. A direct puncture revealed that the lesion was a blood cyst. A cystogram by percutaneous needle puncture revealed paramedian blood pooling with some draining veins but did not show any transcranial communicating vessels. A T2 weighted MR image demonstrated a well demarcated high intensity lesion just beneath the corium. The subtotally removed specimen turned out to be a cavernous hemangioma. We discerned a conceptual confusion of pseudosinus pericranii with scalp cavernous hemangioma, based on the literature review. And we propose that scalp cavernous hemangioma, even if it changes its size according to posture, should not be simply designated as sinus pericranii.

This report is concerning a case of acute spontaneous paralysis of the posterior interosseous nerve (P.I.O.N.), possibly caused by twists in the nerve trunk, in a 23-year-old woman. The subject felt a tingling pain over the lateral epicondyle of the right forearm when grasping and lifting a basin, and noticed that the fingers of the right hand could not be extended three days later. The fingers of the left hand also experienced paralysis 3 months after the first injury when she pronated and extended left forearm. Neurological examination revealed bilateral P.I.O.N. paralysis. When, after a period of time, the bilateral P.I.O.N. paralysis had not improved, surgical exploration of both P.I.O.N. was performed. It revealed that the right P.I.O.N. underwent a severe constriction at 2 cm proximal to the superficial portion of the supinator muscle, and that the proximal portion of the right nerve was swollen. The constricted portion of the right nerve was resected 5 mm in length, and nerve suturing was performed. The left P.I.O.N. was also constricted at the same location, and was found to be a sausage-like neurinoma with two constrictions. In the histological examination of the right resected P.I.O.N., edema of the interstitial tissue and a great number of regenerating cluster formations, including swollen axons, were observed proximal to the constriction. Distal to this, severe Wallerian degeneration was found. These histological findings were the same as those of chronic compression neuropathy. The authors reviewed and analysed reports on 20 other cases of P.I.O.N. paralysis that had compressions at 2 cm proximal to the superficial portion of the supinator muscle.(ABSTRACT TRUNCATED AT 250 WORDS)

Plasmacytoma originating in the cranial bone is a rare disease. A report is presented of a case of plasmacytoma originating in the parieto-occipital region. The patient was a 62-year-old male with palpation of a parieto-occipital mass as chief complaint. He showed no neurological deficit. Ig-G in the serum was 2240 mg/dl, and M-protein (n-type) was demonstrated in the serum. Bence-Jones protein was negative and bone marrow was normal. Skull X-ray showed osteolytic change in the parieto-occipital region. CT scan and MRI demonstrated a markedly enhanced mass extending from the epidural to the subcutaneous space at the parieto-occipital region. Common carotid angiography showed remarkable tumor stain flowing from the occipital artery, the superficial temporal artery, and the middle meningeal artery. Following embolization of the bilateral occipital artery, parieto-occipital horse shoe scalp incision was done. The tumor was elastic soft and bled easily around the margin of destroyed bone. Subtotal removal of the tumor was accomplished. The removed surgical specimen of the tumor was found to be plasmacytoma. The patient's postoperative course was favorable and no neurological deficit was found. Laboratory studies revealed a remarkable reduction of Ig-G in the serum to 1170 mg/dl. Six months after the operation, no signs of recurrence were seen on CT scan. Plasmacytoma originating in the cranial bone is so rare that only 18 cases have been reported to date. In these cases, laboratory studies have shown no evidence of anemia, Bence-Jones protein in urine, and abnormality of the bone marrow which are characteristic of multiple myeloma.(ABSTRACT TRUNCATED AT 250 WORDS)

We reported a patient with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), presenting localized radiculopathy as an initial symptom. The patient, a 38-year-old man, developed muscle atrophy of right shin and calf. In April 1988, neurological examination revealed decreased superficial sensation and muscle atrophy in right L-4 distribution. MRI of the lumbar spinal cord and myelography were negative. In April 1989, he was admitted to Chiba University Hospital. There were slight weakness of right ankle extension and flexion, muscle atrophy of bilateral shin and calf (that of the right leg was more marked), decreased sensation of polyneuropathy type in all modalities and generalized hypo- or a-reflexia. Nerve conduction study revealed marked prolongation of F response and focal conduction block in various site. The CSF was acellular and total protein was 177 mg/dl. Sural nerve biopsy disclosed decreased number of large myelinated fibers and scattered thin myelinated fibers. This case indicates that CIDP may present localized radiculopathy in early phase and is important to point out various mode of onset among CIDP.

Somatosensory evoked potentials to median nerve stimulations were recorded in 21 patients with hypoxic coma within 24 hours from the onset. According to the neurologic prognosis, the patients were classified into 4 groups, such as I, complete recovery (5 patients), II, partial recovery (3 patients), III, vegetable state (8 patients), IV, brain death (5 patients). Measurements performed include central conduction time (N13-N20 interpeak latency) and amplitude ratio between N20 and P25. As N20 and P25 disappeared in 3 of 8 vegetable patients and all of brain death patients, disappearance of these waves indicates poor neurologic prognosis. Central conduction time showed no significant difference. Amplitude ratio between N20 and P25 (P25/N20) revealed significant decrease in vegetative patients for normal control subjects, complete recovery and partial recovery patients (p less than 0.05). Measurement of this ratio in early phase is useful to predict neurologic outcome in patients with hypoxic coma.

The authors describe the features of magnetic resonance (MR) images of Chiari type I and II malformations and present four illustrative cases. Downward displacement of the posterior fossa was more pronounced with type II than type I. A variety of intracranial anomalies were associated with the Chiari type II malformation, whereas type I was mainly associated with syringomyelia and craniovertebral changes. MR imaging is useful in the diagnosis of intracranial anomalies and those situated at the craniovertebral junction, including Chiari malformations.

Authors reported a case of recurrent intracerebral hemorrhage accompanied by severe orthostatic hypotension. A 51-year-old women had recurrent intracerebral hemorrhage 3 times during a period of 2 years. The first and third hemorrhages were located in the right putaminal region, and the second hemorrhage in the left thalamic region. Cerebral angiography revealed neither evidence of vascular malformation nor that of tumor vessels. At the third admission, she became unconscious for three hours after admission, and emergent fronto-temporal craniotomy was performed. Light microscopic histological investigation with congo-red stain demonstrated the absence of cerebral amyloid angiopathy. Laboratory examination revealed no hemorrhagic diathesis. During hospitalization, She complained of dizziness in the standing position. When systolic blood pressure fell from 140 mmHg in the supine position to less than 80 mmHg in the standing position, she became unconscious. Her blood pressure was very labile with orthostatic changes and her systolic blood pressure was also very labile without orthostatic changes, changing from 108 mmHg to 218 mmHg. Severe orthostatic hypotension and labile hypertension made the medical control of hypertension difficult. In conclusion, both severe orthostatic hypotension and labile hypertension were risk factors of recurrence of intracerebral hemorrhage.

We report what may be the first case of lipoma of the corpus callosum associated with a distal anterior cerebral artery saccular aneurysm. The pathogenetic correlation between the callosal lipoma and the distal anterior cerebral artery saccular aneurysm is discussed.

The following results were obtained by measuring serum and tissue concentrations of aztreonam, a new monobactam antibiotic. Penetration into each tissue was favorable, particularly, to the portio vaginalis, but there was no conspicuous difference of the penetration among other tissues. Serum concentration tended to decrease with the lapse of time, but the concentration in each tissue did not show a specific pattern of change due to a considerable irregularity of the measured concentrations. No specific side effect was noted.

T-1982 (cefbuperazone), a new cephamycin antibiotic with broad spectrum against Gram-positive, negative aerobic and anaerobic organisms, was clinically and bacteriologically evaluated on the gynecologic infectious diseases. Fourteen cases hospitalized at Kanazawa Medical University Hospital and the affiliated hospitals from October 1981 to July 1982 were treated with T-1982. By clinical symptoms, signs and bacteriological examinations the patients were diagnosed as pelveoperitonitis (1), intrauterine infection (3), adnexitis (3), infectious diseases of external genitalia (4), infectious abortion (1), vulvar hematoma (1), and rectovaginal fistula (1). T-1982 was administered intravenously or by drip infusion at a dose of 0.5-2.0 g twice a day after dissolved in a saline solution or a 5% glucose solution. Based on the improvement of clinical findings and antibacterial effect of T-1982, results were evaluated as excellent, good, poor and unknown. Clinical effects more than good were shown in 9 of 11 cases which could be followed up exactly on the therapeutic of T-1982. Two cases showing poor response were pelveoperitonitis and pyometra under carcinoma colli uteri stage IIIb, respectively. In 8 of 11 cases, antibacterial effect of T-1982 could be evaluated. Thirteen strains of aerobic and anaerobic bacteria were disappeared by T-1982 therapy, while no effect was seen on 5 strains. On the side effect of T-1982, exanthema was observed in 1 case but disappeared soon after the cessation of administration.

A case of arteriovenous malformation (AVM) with angiographically visualized extravasation of contrast medium on carotid angiography has been reported. The patient, a 9-year-old girl, came to our clinic with chief complaints of unconsciousness and left hemiplegia. On right carotid angiogram, done 4 hours after stroke, an AVM with a large intracerebral hematoma at the region of the right basal ganglia was recognized. The AVM was feeded from several lenticulostriate arteries and a small branch from precentral artery, and drained into thalamostriate vein. The extravasation of contrast medium was seen in a sash like fashion through arterial and venous phase. Extravasation of contrast medium from AVM has been extremely rarely reported, and this is considered as the third reported case. From the study of these 3 cases, we have discussed about the rarity of the extravasation from AVM and the causative factors.

A 66-year-old woman was admitted to the Hakodate Chuo Hospital because of abdominal discomfort. A barium enema study revealed a deformity of the middle Houston's valve of the rectum and granular lesions at the Rb region of the rectum. A colonoscopic examination showed the IIa + IIc type of early rectal carcinoma. A resected specimen also revealed IIa + IIc type early rectal carcinoma, 8 X 13 mm in diameter, and well-differentiated adenocarcinoma with no invasion of the submucosa. Infiltration into the lymphatic vessels and regional lymph node metastasis were not encountered. The deformity of the rectal Houston's valve was checked by a barium enema study. It should be emphasized that early detection of colorectal carcinoma with no invasion of the submucosa, requires careful barium enema studies and colonoscopic examination.

When Mycobacterium tuberculosis was grown on Sauton medium, intracellular tuberculin-active protein was produced. This product was purified by chromatography on DEAE-cellulose and Sephadex G-200 and was obtained in crystalline form. The crystals were plates, somewhat irregular in shape, about 50 mum in length and 25 mum in width. The yield corresponded to a 1.5% over-all recovery of total protein. Ultracentrifugal analysis showed only one major component with a calculated molecular weight of 9700. Sedimentation velocity analysis gave a sedimentation coefficient at 20 degrees (see article) of 1.73 S. The estimated specific activities of tuberculin-active protein were 6.33 times 10-9 tuberculin units per mg of protein-nitrogen for sensitized guinea pigs and 6.33 times 10-11 tuberculin units per mg of protein-nitrogen for humans. This is the most potent tuberculin-active protein that has yet been obtained.

A 53-year-old man with malignant lymphoma of the ileum is reported. He was admitted to our hospital because of lower abdominal discomfort. 67Ga citrate radionuclide imaging revealed an abnormal round uptake in his lower abdomen. CT scans showed an abnormal soft tissue density mass and a remarkably dilated small intestine in the pelvis. X-ray studies of the small intestine revealed a giant diverticulum-like lesion. Angiograms showed a hypovascular tumor with irregularity of the small vessels. A resected specimen revealed non-Hodgkin's malignant lymphoma, diffuse large-cell type. 67Ga citrate radionuclide imaging was useful in screening for neoplastic lesions in the small intestine because of its easiness and safety.

The authors studied 66 patients with hypertensive putamen hemorrhage and noted a close relationship between the CT classification according to the shape of the suprasellar cistern (S) and the third ventricle (III), and the state of altered consciousness: when S is not deformed and III is identified in CT images, the patient may be in a clear or confusional state; if S is not deformed but III is not identified or is packed with hematoma, consciousness may be somnolent or stuporous; if S is deformed and III is not identified or is packed with hematoma, the patient may be in a semicomatose or comatose state. However, within several hours after onset there are exceptional cases in which the states of consciousness do not correlate with the CT findings. In these cases, however, with conservative therapy the state of consciousness corresponded well with the CT findings. From the neurosurgical points of view, immediate removal of the hematoma is required when the patient's consciousness is relatively good despite of large hematoma. If at the acute stage the state of consciousness, which corresponded well with the CT findings at initial examination, has deteriorated, rebleeding or enlargement of hematoma must be suspected.