[A 50 year old man with progressive weakness in lower limbs].

Lambert-Eaton Myasthenic Syndrome (LEMS) is a rare neurological disorder caused by autoimmune antibodies attacking the presynaptic neuromuscular junction, in some cases caused by underlying cancer. The main clinical finding is fluctuating weakness of the extremities and a triad of symtoms can help physicians suspect the disease. A key to the diagnosis are the electrophysiological abnormalities seen in this group of diseases. Treatment with symtomatic and/or immunosuppressive therapy is important as well as a workup for possible malignancy. This article identifies the clinical features, diagnosis and treatment of this uncommon disease.

Delirium and fever - a rare but dangerous cause - Case report

Fever complicated by delirium is a common problem in emergency departments and inpatient wards. There are various possible causes that must all be contemplated. Infections are prominent causes due to acuteness and severity, but when generating a differential diagnosis, other causes may be life-threatening and require swift diagnosis and management. We present here a case of a 58 year old man presenting at the emergency department with fever and delirium. After comprehensive history-taking and examination, alongside targeted testing, the correct diagnosis was ascertained, leading to appropriate treatment.

Prevalence of symptomatic Charcot-Marie-Tooth disease in Iceland: a study of a well-defined population.

BACKGROUND/AIM: To determine the prevalence and clinical spectrum of Charcot-Marie-Tooth disease (CMT) in Iceland. METHODS: We identified all individuals with symptomatic CMT, based on information from all practicing neurologists, both neurophysiology laboratories and the only neurology department in the country. The diagnosis was based on clinical features and neurophysiological testing. DNA testing was regarded as confirmatory. RESULTS: We identified 37 individuals in 18 families, which were not linked by identifying 5 generations of ancestors. The point prevalence (January 1, 2007) for all CMT subtypes in Iceland was 12.0/10(5), 10.1/10(5) for CMT1 and 2.0/10(5) for CMT2. The clinical features include lower limb weakness (95%), impaired gait (68%), decreased or absent deep tendon reflexes (86%), pes cavus (70%) and hammer toes (46%). Clinical symptoms were similar for the 2 main CMT subtypes. CONCLUSION: We report the prevalence and clinical spectrum of CMT, which is comparable to the results of other prevalence studies, in a well-defined, total population sample.

[Tetanus: a case report and literature review]. / Stífkrampi - tilfelli og yfirlit

A 79 year old farmer was referred to the University Hospital with a three days history of difficulty in opening his mouth. On examination, both masseters were seen to be stiff, suggesting tetanus or jaw-subluxation. An attempt to reduce the joint was made, but failed. He subsequently developed progressive respiratory difficulties requiring intubation. The farmer had recently cut his finger on barbed wire. He had not received tetanus immunization for years and it was not clear whether primary immunization had been completed. Tetanus immunoglobulin and antibiotics were administered. He proceeded to develop autonomic instability and critical illness polyneuropathy requiring 45 days of ICU care. He was finally discharged eight months later. The farmer has gradually improved and is now living unaided at home. In this article we will review this case and the literature on tetanus.