Guillain-Barré syndrome: diagnostic criteria, epidemiology, clinical course and prognosis

Thirty-four patients were identified with Guillain-Barré syndrome (GBS) on review of 266 neuropathy cases admitted to a Copenhagen county hospital from June 1977 to January 1984. The age-adjusted incidence rate of GBS is 2.0 x 10(-5) years-1. The natural history of the disease, antecedent events, symptoms and signs, autonomic dysfunction, sequelae, CSF findings and mortality are described. Six cancer patients with GBS differed significantly from the non-cancer patients in a more protracted disease course and failure to improve. The National Institute of Neurological and Communicative Disorders and Stroke (NINCDS) criteria for the diagnosis of GBS are discussed, and it is concluded that the criteria, although useful in comparative studies, are too restrictive when used in clinical practice.     

Register for cerebrovascular events in the Area Latina, Italy. Clinical and epidemiological data during three years of surveillance

Data are presented on the cerebrovascular events collected by a surveillance system during 1983–1985 in the MONICA Project-Area Latina, on a target population of about 400,000 subjects aged 25–74. The cerebrovascular events observed in 3 years were 2245: the fatalities (within 28 days from the onset of symptoms) numbered 1016 (562 males and 454 females), and the non fatal cases 1229 (742 males and 482 females). The attack rates per 10,000 per year were 21.96 for males and 15.20 for females. By definition, all the non fatal cases received some kind of treatment in hospital or nursing home. Among the fatal cases 68% received a treatment in a hospital or nursing home, while 32% died without medical attention. The fatality rate within 28 days was 43% for males and 48% for females. Appendix Members of the MONICA Project—Area Latina Responsible investigators: S. Giampaoli, A Menotti, G Righetti, A Verdecchia. Clinical staff: E Forte, A Maietta, C Salvatelli. Technical staff: S. Barzotti, R Capocaccia, G G Di Carlo, F Dima, B Giuli, P Lombari, C Lo Noce, S Mariotti, M Pasquali, A Santaquilani     

Shin-gata utsu (a new type of depression) in Japan: Social and clinical implications of the emerging phenomenon

This article is aimed at introducing and examining recent controversies raised by mental health professionals in Japan concerning what they call shin-gata utsu (a new type of depression). While clinical depression has been a serious public health concern in Japan for several decades, a new type of patient, one whose characteristics and symptoms deviate from the conventional patient profile, is appearing, primarily in workplaces. In contrast to the typical Japanese depressive patients who are workaholics, self-blaming, and middle aged or older male workers, many shin-gata utsu patients are self-centered, eager to blame others and escape from work, and more likely to be younger women. There has also been much confusion among human resource management about how to understand shin-gata utsu: Is it a form of illness eligible for workers’ compensation or simply the problem of an immature, spoilt personality? While it has never been an official diagnostic category, the increasing use of this label is suggestive of the Japanese approach to mental disorders and potentially a fundamental change in the personality of the Japanese, which could originate from their family socialization pattern. The sociocultural backgrounds for this controversy will be explored, as well as what it could mean to Japanese society.     

Spinocerebellar ataxia type 6: CAG trinucleotide expansion, clinical characteristics and sperm analysis

Spinocerebellar ataxia type 6 (SCA6) is an autosomal dominant spinocerebellar degeneration caused by CAG repeat expansions in the human α1A voltage-dependent calcium channel subunit gene. We analyzed 16 SCA6 patients in 14 unrelated Japanese families, and documented the clinical and molecular properties correlating with the CAG repeat expansion. Three of them were sporadic. The CAG repeat number of the expanded and normal alleles was 22.7 ± 2.0 (mean ± SD, n = 15) and 13.8 ± 2.0 (n = 15), respectively, and the repeat size of the expanded alleles correlated inversely with age at onset. The patients presented here were clinically characterized by a slowly progressive cerebellar ataxia and nystagmus. In leukocytes, the strict pattern of the peak in the expanded allele on polyacrylamide gel electrophoresis did not show the presence of cell mosaicism in SCA6, in contrast to other trinucleotide disorders. Moreover, in each patient, the number of CAG repeats in sperm was the same as in leukocytes, and the expanded alleles in sperm indicated uniform peaks as well. In our geographic area, the frequency of SCA6 was as high as MJD, in contrast to the low frequency of other autosomal dominant cerebellar ataxias. Thus, a geographic difference in the frequency of autosomal dominant spinocerebellar ataxias may be present in Japan.     

Senile dementia of the neurofibrillary tangle type (tangle‐only dementia): Neuropathological criteria and clinical guidelines for diagnosis*

Senile dementia of the NFT type (SD‐NFT) is a subset of dementia in the elderly, characterized by numerous NFT in the hippocampal region and absence or scarcity of senile plaques throughout the brain. Senile dementia‐NFT has also been referred to as tangle‐only dementia, NFT‐predominant form of SD, SD with tangles, or limbic NFT dementia. Herein are proposed the criteria for neuropathological diagnosis of SD‐NFT: (i) late‐onset dementia with abundant NFT in the hippocampal region and absence or scarcity of senile plaques (amyloid β protein deposits) throughout the brain; and (ii) exclusion of other dementias with NFT. Some elderly individuals suffering from memory disorder without obvious dementia have neuropathological findings similar to SD‐NFT, and they would represent a condition in the process of formation of the SD‐NFT pathology. Guidelines for the clinical diagnosis of SD‐NFT are also proposed; development of reliable diagnostic tests is necessary to differentiate AD and other neurodegenerative dementias from SD‐NFT.     

Somatic neuropathy is an independent predictor of all‐ and diabetes‐related mortality in type 2 diabetic patients: a population‐based 5‐year follow‐up study (KCIS No. 29)

Background and purpose: To investigate the relationships of diabetic neuropathy to all‐cause and diabetes‐related mortality in patients with type 2 diabetes after controlling for significant correlates. Methods: We examined 326 patients diagnosed as diabetic polyneuropathy by nerve conduction study in Keelung city, Taiwan, in 2002 and followed them up to ascertain the cause and date of death until the end of 2006. The cause and date of death were recorded for the deceased patients. Information on significant correlates in association with diabetic polyneuropathy and all‐cause and diabetes‐related mortality was also collected. Results: With median follow‐up time of 62.28 months, 44 patients with type 2 diabetes died. The cause of death related to diabetes accounted for 59% (n = 26) of the deceased. Univariate analysis shows that the presence of diabetic neuropathy confers higher risk for all‐cause mortality (hazard ratio [HR] = 4.88) and mortality from diabetes (HR = 6.58). The significant finding still persisted after adjustment for age, gender, blood pressure, smoking, history of cardiovascular/cerebrovascular disease, duration of diabetes, waist circumference, fasting plasma glucose, total cholesterol, hemoglobin, and creatinine (adjusted HR = 4.44 for all‐cause death and adjusted HR = 11.82 for diabetes‐related mortality, respectively). Conclusions: Diabetic polyneuropathy was an independent predictor for all‐cause and diabetes‐related mortality. The presence of neuropathy together with other significant prognostic factors is informative to predict all‐cause death and death from diabetes‐related disease for patients diagnosed as type 2 diabetes.     

Evaluation of phrenic nerve and pulmonary function in hereditary motor and sensory neuropathy, type I.

Phrenic nerve and diaphragmatic dysfunction has been assumed to be the cause of respiratory failure in hereditary motor and sensory neuropathy, type 1 (HMSN I). In order to determine the relationship between phrenic nerve and pulmonary function in this disease, 25 patients underwent a 4-step evaluation process consisting of: (1) bilateral phrenic nerve conduction study; (2) median, peroneal, and tibial motor conduction studies; (3) measurement of forced vital capacity (FVC) and maximal inspiratory and expiratory pressures (MIP, MEP); and (4) pulmonary-focused history and physical. Phrenic nerve motor latency was abnormally prolonged in 22 of the 23 (96%) subjects when a response was obtained. All had slowed velocity or absent peripheral motor conduction responses. Vital capacity was abnormally reduced in 6 of the 25 (24%) subjects. Eight (32%) had an abnormally reduced MIP, while 19 (76%) had an abnormally reduced MEP. Only 2 (8%) subjects had clinical evidence of pulmonary dysfunction. None of the dependent variables (FVC, MIP, MEP, peripheral nerve conduction, or clinical examination) correlated with phrenic nerve latencies. Although phrenic nerve latencies are markedly prolonged in HMSN I, these values are not useful in predicting respiratory dysfunction.     

Adult onset Niemann-Pick type C disease: A clinical, neuroimaging and molecular genetic study.

We report on a patient with adult-onset Niemann-Pick type C (NPC) disease, carrying the mutations P1007 and I1061T in the NPC1 gene, presenting with marked psychiatric changes followed by dystonia and cognitive impairment. Filipin staining, single photon emission computed tomography perfusional, positron emission tomography metabolic, conventional magnetic resonance imaging, and magnetic resonance spectroscopy findings suggested a pathophysiological correlation with phenotype expression. This case expands the clinical and genetic spectrum of the rare adult-onset NPC disease phenotype.     

The impact of omega-3 fatty acids, vitamins E and C supplementation on treatment outcome and side effects in schizophrenia patients treated with haloperidol: an open-label pilot study

Classical antipsychotics like haloperidol are suggested to increase oxidative stress and oxidative cell injury in the brain. Pro-oxidant effect of haloperidol may influence the course and treatment outcomes of schizophrenia. Dietary supplementation of either antioxidants or omega-3 fatty acids was found to improve symptoms of schizophrenia. Thus we decided to assess the impact of combining omega-3 fatty acids, vitamins E and C supplementation on treatment outcome and side effects in schizophrenia patients treated with haloperidol. Ongoing haloperidol treatment of 17 schizophrenia patients was supplemented with 1000 mg capsule of omega-3 fatty acids (180 mg EPA+120 mg DHA) bid, vitamin E 400 IU bid and vitamin C 1000 mg/day. Patients were assessed with Brief Psychiatric Rating Scale (BPRS), Scale for the Assessment of Negative Symptoms (SANS), Simpson Angus Scale (SAS) and Barnes Akathisia Rating Scale (BARS) over a 4 month period. Gluthatione peroxidase, superoxide dismutase, malondialdehyde, vitamin E and C levels were also evaluated at baseline and at the end of study. BPRS, SANS, SAS and BARS scores obtained at follow-up visits were significantly lower compared to baseline. Superoxide dismutase level was significantly lower at the end of study. No significant differences were detected in other laboratory parameters. Our results support the beneficial effect of the supplementation on positive and negative symptoms of schizophrenia as well as the severity of side effects induced by haloperidol. The effect of supplementation on akathisia is especially noteworthy and it has not been investigated in previous studies.