Clinical and electrophysiological features of the 2007 Guillain–Barré syndrome epidemic in Northeast China

Guillain–Barré syndrome (GBS) generally presents sporadically. Epidemics of GBS are unusual. We reviewed the medical records of 26 patients hospitalized for GBS during the 2007 GBS epidemic in northeast China. The objective was to determine whether there were clinical and electrophysiological characteristics. All patients had drunk unboiled water, and the illness was preceded by diarrhea in 19 (73%) patients. Only 1 patient had a Campylobacter jejuni infection, whereas 14 (54%) patients exhibited features of acute motor axonal neuropathy (AMAN). The most common electrophysiological findings in early GBS included decreased compound muscle action potential (CMAP) amplitude (62%), abnormal F waves (73%), and abnormal H reflexes (62%). This epidemic of GBS appears to have been associated with consumption of contaminated water. The main subtype was AMAN, which was associated with a longer duration of illness and a worse prognosis. Electrodiagnostic evaluations are helpful for diagnosis in the primary stages of GBS. Muscle Nerve, 2010     

EEG markers of future cognitive performance in the elderly

This exploratory follow-up study investigated whether EEG parameters can predict future cognitive performance. Forty elderly subjects, ranging from cognitively unimpaired to those with Alzheimer disease underwent EEG registration at baseline and neuropsychological examination at both baseline and follow-up. We assessed relations between EEG measures and future cognitive performance (i.e., global cognition, memory, language, and executive functioning) controlling for age, follow-up time, and baseline cognitive performance. Regression models were constructed to predict performance on the Cambridge Cognitive Examination, a widely used tool within dementia screenings. Baseline EEG measures, i.e., increased theta activity (4-8 Hz) during eyes closed and less alpha reactivity (8-13 Hz) during eyes open and memory activation, indicated lower global cognitive, language (trend significant), and executive performance at follow-up. A regression model combining baseline cognitive and EEG measures provided the best prediction of future Cambridge Cognitive Examination performance (93%). EEG and cognitive measures alone predicted, respectively, 43% and 92% of variance. EEG and cognitive measures combined provided the best prediction of future cognitive performance. Although the "cognition only" model showed similar predictive power, the EEG provided significant additional value. The added value of EEG registration in the diagnostic work-up of dementia should be further assessed in larger samples.     

Red Blood Cell Distribution Width (RDW) and long-term survival in patients with ST Elevation Myocardial Infarction

Introduction

High RDW values are associated with adverse prognosis in many clinical conditions including short and medium term outcome of patients with ST Elevation Myocardial Infarction (STEMI). The aim of the present study was to evaluate the association between RDW and long term mortality in STEMI patients undergoing primary angioplasty (PPCI).

Material and methods

A cohort of 535 STEMI patients undergoing PPCI were divided into two groups (RDW > 14%, RDW ≤ 14%) using CHAID and CART methods. The association between RDW and 5-year all-cause mortality was assessed using Cox’s proportional hazards analysis.

Results

A total of 37 patients died during follow up of 5 years (mean: 1059, median: 1013, range 2–2130 days). RDW > 14% was associated with increased risk of all-cause mortality (HR = 5, CI 95% 2.7– 9.9, p < 0.001). In multivariate analysis, RDW > 14 remained significantly associated with increased risk for all-cause mortality (HR = 3.8, CI 95% 1.8– 7.99, p < 0.001). Patients with RDW above 14% did not have lower ejection fraction, higher CPK or more conventional risk factors.

Conclusion

RDW value above 14 is independently associated with increased long term all-cause mortality in patients with STEMI undergoing PPCI.     

Deficient sustained attention to response task and P300 characteristics in early Huntington’s disease

Evidence for the extent and nature of attentional impairment in premanifest and manifest Huntington's disease (HD) is inconsistent. Understanding such impairments may help to better understand early functional changes in HD and could have consequences concerning care for HD patients. We investigated attentional control in both early and premanifest HD. We studied 17 early HD subjects (mean age: 51 years), 12 premanifest HD subjects (mean age: 43 years), and 15 healthy controls (mean age: 51 years), using the sustained attention to response task (SART), a simple Go/No-go test reflecting attentional and inhibitory processes through reaction time (RT) and error rates. Simultaneously recorded EEG yielded P300 amplitudes and latencies. The early HD group made more Go errors (p < 0.001) and reacted slower (p < 0.005) than the other groups. The RT pattern during the SART was remarkably different for early HD subjects compared to the other two groups (p < 0.005), apparent as significant post-error slowing. P300 data showed that for early HD the No-go amplitude was lower than for the other two groups (p < 0.05). Subjects with early HD showed a reduced capacity to effectively control attention. They proved unable to resume the task directly after having made an error, and need more time to return to pre-error performance levels. No attentional control deficits were found for the premanifest HD group.     

Selective mutism and abnormal electroencephalography (EEG) tracings

Epileptic discharges are not considered a part of the clinical picture of selective mutism, and electroencephalography is generally not recommended in its work-up. This report describes 6 children with selective mutism who were found to have a history of epilepsy and abnormal interictal or subclinical electroencephalography recordings. Two of them had benign epilepsy of childhood with centro-temporal spikes. The mutism was not related in time to the presence of active seizures. While seizures could be controlled in all children by medications, the mutism resolved only in 1. Although the discharges could be coincidental, they might represent a co-morbidity of selective mutism or even play a role in its pathogenesis. Selective mutism should be listed among the psychiatric disorders that may be associated with electroencephalographic abnormalities. It can probably be regarded as a symptom of a more complicated organic brain disorder.     

Acute limb ischemia due to malignant arterial embolism from a metastatic germ cell tumor

Arterial tumor embolization is a rare but serious complication of neoplastic disease. The majority of these tumors are associated with primary or secondary lung malignancies, originating from pulmonary vein metastasis or from an atrial mass. Malignant germ cell tumors primarily disseminate to the retroperitoneal lymph nodes and lung, and to the brain and liver later in the course of the disease. A germ cell tumor metastasis embolizing to the iliac-femoral arterial system has not yet been reported. We report a metastatic embolism in a patient with disseminated embryonal cell carcinoma causing acute limb ischemia, managed by surgical embolectomy. The sudden development of limb ischemia in a patient with a germ cell tumor should alert the physician to the possibility of tumor embolism. © 1995 Wiley-Liss, Inc.     

Pulmonary arterio-venous fistulae in hepatic cirrhosis

Cyanosis, clubbing, and arterial oxygen desaturation may occur in patients with liver disease, and are attributed to the presence of pulmonary or peripheral arterio-venous shunting. Cardiac catheterisation and angiocardiography in a patient with a normal heart did not demonstrate the presence of abnormal arterio-venous anastomoses. Pulmonary shunting was proved when intravenous technetium-labelled macroaggregated albumin, normally held up in capillary networks, was passed quickly through the lungs and immediately detected in high systemic blood flow organs. The opening of peripheral and pulmonary anastomoses in patients with liver disease may be owing to the presence of a vasodilatory substance such as ferritin, which was found to be abnormally increased in the patient's blood.     

Incipient CADASIL

BACKGROUND: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is caused by mutations in the NOTCH3 gene. Knowledge of disease expression in young adult NOTCH3 mutation carriers (MCs) is limited. OBJECTIVE: To characterize clinical, neuropsychological, and radiological status in NOTCH3 MCs younger than 35 years. DESIGN: Clinical characterization and blinded survey comparing MCs with non-MCs. SETTING: Referral center. PARTICIPANTS: Individuals younger than 35 years who were at a 50% risk of a NOTCH3 mutation, from our CADASIL database. Thirteen individuals, from 8 families, met the criteria. METHODS: Comprehensive clinical, genetic, neuropsychological, and radiological investigations. Magnetic resonance images were scored according to a standardized white matter hyperintensities rating scale. RESULTS: Six individuals, from 5 families, were MCs. Clinical symptoms consisted of migraine (with aura), stroke, and stroke-like episodes. We did not find evidence for psychiatric disturbances, functional disability, or cognitive dysfunction, compared with non-MCs. Radiologically, a characteristic magnetic resonance imaging lesion pattern emerged for all MCs. This comprised white matter hyperintensities in the anterior temporal lobes, the frontal lobes, and the periventricular frontal caps. CONCLUSIONS: Migraine (with aura) and stroke can present in NOTCH3 MCs younger than 35 years; however, more importantly, physical function and cognition are intact. Possible subtle cognitive dysfunction needs to be assessed in a larger study. White matter hyperintensities on magnetic resonance imaging are characteristic, and are consistently visualized from the age of 21 years and onward. Awareness of the clinical and radiological features of CADASIL in those younger than 35 years should increase early diagnosis and allow for customized counseling of young adults from families with CADASIL.     

A population-based study of help-seeking for self-harm in young adults

OBJECTIVE: To examine help-seeking for self-harm in a population-based sample of young adults. METHOD: Nine hundred and sixty-five participants aged 26 years were interviewed about help-seeking and barriers to help-seeking for a range of self-harmful behaviours. Self-harm included the traditional methods of suicide (ICD-9 self-harm) and other self-harmful behaviours such as self-battery and self-biting. RESULTS: Just over half of the 25 in the ICD-self-harm group (based on ICD-9 self-harm criteria) and one-fifth of the 119 in the other self-harmful behaviour group had sought professional help. Counsellors, psychologists, and general practitioners were the commonest sources of help. Most participants rated help received from health services favourably, however, emergency services were rated less favourably than other health services. Among 77 self-harm participants who did not seek help, just over one-third reported attitudinal barriers. CONCLUSIONS: To encourage help-seeking by young adults who self-harm, especially young men who are at high risk for self-harm and suicide, it may be necessary to identify ways to reduce attitudinal barriers.