Amyloid myopathy presenting with distal atrophic weakness

Amyloid myopathy is a rare complication of primary amyloidosis usually presenting with proximal muscle weakness. We report a woman with multiple myeloma in whom marked atrophy and weakness of finger flexor muscles were the first manifestations of systemic amyloidosis. Muscle biopsy revealed amyloid angiopathy with deposits of lambda light chains in vessel walls. The recognition of amyloid myopathy is important because clinical symptoms may respond to chemotherapy.     

Chronic daily headache with medication overuse: a randomized follow-up by neurologist or PCP

Several studies have shown the benefit of withdrawal therapy when medication overuse headache (MOH) is suspected. Our aim was to compare the effect of withdrawal therapy in patients followed by a neurologist (group A, n  = 42) and a primary care physician (PCP) (group B, n  = 38). Patients were randomized to A or B, and follow-up was at 3, 6 and 12 months. Calculated mean headache (MH at 6 months + MH at 12 months)/2 (primary end-point) was similar; A 1.04 (0.87, 1.21) and B 1.02 (0.82, 1.21) ( P  = 0.87). The number of patients with 50% improvement of headache days was also similar; 14/42 in group A vs. 12/34 in B ( P  = 0.86) at 3 months, 15/42 vs. 11/33 ( P  = 0.83) at 6 months and 15/42 vs. 14/38 ( P  = 0.92) at 12 months. Days without headache during the last 9 months of follow-up were 123 (96, 150) in group A and 137 (112, 161) in B ( P  = 0.62). After 3 months one-third were classified as MOH. Patients with MOH improved similarly in group A and B, and so did patients without MOH. Within 1 year 7/42 in A and 9/38 in B had recurrent medication overuse ( P  = 0.43). In summary, there were no significant differences in follow-up results between the two groups.     

Poor reproducibility of PIRADS score in two multiparametric MRIs before biopsy in men with elevated PSA

Purpose

Since January 2015, all men referred to urologists in Norway due to elevated PSA or other suspicion of prostate cancer underwent multiparametric MRI (mpMRI) before prostate biopsy. At our hospital, patients and the initial MRI were assessed by an urologist and if deemed necessary, patients were referred to another institution for MR/US fusion biopsies. Before MR/US biopsy, patients underwent a second mpMRI. Since we noticed disagreement of these two mpMRIs before biopsy, we retrospectively assessed the level of agreement between the two mpMRIs from the two institutions.

Methods

During the first 6 months of 2015, 292 patients were referred to our outpatient clinic. We referred 126 patients of these to the other institution for MR/US fusion biopsy. The 2 mpMRIs were performed within 4 weeks. We analyzed MR reports and schematics for number of lesions and highest PIRADS score per side of the prostate and histological result of the biopsies. Bland–Altman’s plot was used to compare the level of agreement between the two mpMRIs of the same patient before biopsy.

Results

There was a poor level of agreement between the two mpMRIs and a statistically significant difference in PIRADS scores. Regression analysis showed that there was no proportional or systematic bias.

Conclusion

In unselected patients with elevated PSA, there seems to be a significant variation of mpMRI results across institutions. The PIRADS scoring system needs to be validated with regards to MR equipment, mpMRI protocols and inter-reader variability of radiologists.

     

The phenomenon of ‘chronic Lyme’; an observational study

Purposes: To chart clinical, laboratory, and psychometric profiles in patients who attribute their complaints to chronic Lyme disease. Methods: We assessed the patients by clinical examination, laboratory tests, and questionnaires measuring fatigue, depression, anxiety, health‐related quality of life, hypochondriasis, and illness perceptions. Results: We found no evidence of ongoing Borrelia burgdorferi (Bb) infection in any of the 29 included patients using current diagnostic guidelines and an extended array of tests. Eight (28%) had other well‐defined illnesses. Twenty‐one (72%) had symptoms of unknown cause, of those six met the suggested criteria for post‐Lyme disease syndrome. Fourteen (48%) had presence of anti‐Bb antibodies. The patients had more fatigue and poorer health‐related quality of life as compared to normative data, but were not more depressed, anxious, or hypochondriacal. Their beliefs about the illness were characterized by negative expectations. Conclusion: Our patients, who all attributed their symptoms to chronic Lyme disease, were heterogeneous. None had evidences of persistent Bb infection, but whether current diagnostic criteria are functional in patients with longstanding complaints is controversial. Other well‐defined illnesses or sequelae from earlier Lyme disease were probable as main explanatory factor in some cases. The patients were not more depressed, anxious, or hypochondriacal than the normal population, but they had poorer health‐related quality of life, more fatigue, and negative expectations about their illness.     

Approach to the patient with chronic polyneuropathy

Background –  Chronic polyneuropathy is a common disorder with heterogenic clinical presentation and many different etiologies. Diagnostic investigation is challenging.
Materials and methods –  An algorithm for diagnostic investigation of chronic polyneuropathy is presented. It was designed to secure practical usefulness for general neurologists, identification of the most common causes with an adequate number of laboratory tests, and more focused investigation when necessary. The proposal is based on review of articles found by PubMed search using the terms 'peripheral neuropathy, cause, and investigation', relevant book chapters, and own clinical experience.
Results –  All patients should undergo a routine investigation for the most common causes of polyneuropathy by asking for diabetes, heredity, alcohol abuse, toxic medications and agents, symptoms of Sjögren's syndrome, renal failure, and the following laboratory tests; glucose, haemoglobin, leucocytes, thrombocytes, ESR, creatinin, ALAT, GT, vitamin B12, serum electrophoresis, TSH and thyroxin. If routine investigation is negative, a targeted approach based on clinical type and electrophysiological findings is recommended. The most common type with slowly progressive, symmetric sensory symptoms beginning in the feet can often be classified as cryptogenic without further investigation. Polyneuropathies with subacute onset, progressive weakness, asymmetric symptoms, proximal weakness, selective involvement of sensory fibers or demyelinating pathology, or other organ manifestations, have various etiologies that necessitate individual focused investigation.
Interpretation –  Diagnostic investigation of polyneuropathy can be simplified and systematized.     

Autoimmunity against the ryanodine receptor in myasthenia gravis

Some myasthenia gravis (MG) patients have antibodies against skeletal muscle antigens in addition to the acetylcholine receptor (AChR). A major antigen for these antibodies is the Ca²⁺ release channel of the sarcoplasmic reticulum the ryanodine receptor (RyR). These antibodies are found mainly in MG patients with a thymoma MG and correlate with severe MG symptoms. The antibodies recognize a region near the N‐terminus on the RyR, which seems to be of importance for RyR regulation. The antibodies cause allosteric inhibition of RyR function in vitro, inhibiting Ca²⁺ release from sarcoplasmic reticulum.