1/8. association of spondylarthropathies with lumbar synovial cysts.Intraspinal synovial cysts presenting with lower back pain and radiculopathy are well known but rare. They are associated with facet joint arthopathy, generally degenerative in nature. Spinal synovial cysts have not been described in spondyloarthropathies (SpA). We report a case of a 66-year-old man with a chronic undifferentiated SpA who presented with severe weakness of both legs. A centrally located spinal cyst was encountered on MRI and led to excision of a highly inflammatory synovial cyst. This association may not be fortuitous and be related to inflammation of the facet joint in SpA.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/8. Unilateral calf hypertrophy seen in lumbosacral stenosis: case report and review of the literature.STUDY DESIGN: A case report of a patient with neurogenic unilateral calf hypertrophy and review of the literature are reported. OBJECTIVES: To provide further evidence that S1 radiculopathy is predisposed to develop neurogenic muscle hypertrophy. SUMMARY OF BACKGROUND DATA: Calf hypertrophy, specifically hypertrophy of the gastrocnemius muscle, is a rare but recognized presentation of S1 and less commonly L5 radiculopathies. The pathophysiology of this is incompletely understood. methods: We present a 59-year-old patient with painless progressive distal right leg weakness and calf enlargement. Electrodiagnostic studies and magnetic resonance imaging scanning were performed to evaluate the extent and cause of radicular damage as the etiology for unilateral calf hypertrophy. RESULTS: Examination and electrodiagnostic studies revealed right L5, right S1, and left L5 radiculopathies. Imaging studies demonstrated lumbar stenosis at L3-L4, L4-L5, and L5-S1 vertebral levels as well as L4-L5 and L5-S1 foraminal stenosis. After decompressive surgery the progressive nature of the patient's symptomatology halted, and he had partial resolution of his deficits. CONCLUSION: Although the patient had bilateral L5 radiculopathies, he only had hypertrophy in the distribution of his right S1 radiculopathy. This supports the hypothesis that dysfunction of the S1 nerve root or its distribution is a predisposing factor to develop neurogenic muscle hypertrophy. Furthermore, patients presenting with unilateral calf hypertrophy need a careful diagnostic evaluation for S1 radiculopathy as well as to exclude asymmetric presentation of systemic neuromuscular conditions.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/8. Rocking bed and prolonged independence from nocturnal non-invasive ventilation in neurogenic respiratory failure associated with limb weakness.A 40 year old mother of three with autosomal dominant scapuloperoneal muscular dystrophy presented with severe neurogenic respiratory failure requiring nocturnal non-invasive ventilation (NIV). Because of the development of profound proximal muscular weakness as a consequence of the progressive nature of her neurological disease, she eventually was unable to apply and remove the facial interface to set up her NIV circuit. She therefore became dependent on her children and carers to start and stop NIV during the night. A rocking bed was successfully employed as an alternative to nocturnal NIV. ventilation was facilitated by the passive movement of the diaphragm as a consequence of the movement of the abdominal contents under the effect of gravity. Benefit was demonstrated objectively by pulse oximetry and subjectively by the improvement in the patient's symptomatology and continued independence at night. The ease of use of a rocking bed should be borne in mind when the necessity for nocturnal ventilatory support in neuromuscular disease results in the potential loss of independence for a patient.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/8. Myopathic changes associated with severe acute respiratory syndrome: a postmortem case series.BACKGROUND: The March 2003 outbreak of the severe acute respiratory syndrome (SARS) resulted in significant morbidity and mortality. muscle weakness and elevated serum creatine kinase levels are commonly encountered in patients with SARS. However, the nature and cause of myopathy associated with a SARS infection are unknown because, to our knowledge, there has been no report of histological or postmortem examination of the skeletal muscle from SARS-infected patients. OBJECTIVE: To determine the exact nature of the myopathy associated with SARS. METHOD: Postmortem skeletal muscles from 8 consecutive patients who died of SARS in March 2003 were studied under light and electron microscopy as well as immunohistochemistry. RESULTS: Focal myofiber necrosis was identified in 4 of 8 cases. Macrophage infiltration and regenerative fiber were scanty. All 4 patients treated with a steroid had significant myofiber atrophy. in situ hybridization for coronavirus was negative in all subjects. Viral cultures for coronavirus and examination for viral particles under electron microscopy were performed in 2 patients. The viral culture yielded no organisms and there were no viral particles seen on electron microscopic examination. CONCLUSIONS: There is a spectrum of myopathic changes associated with a SARS infection. Focal myofiber necrosis is common and possibly is immune mediated. critical illness myopathy and superimposed steroid myopathy may also play an important role in SARS.- - - - - - - - - - ranking = 2keywords = nature (Clic here for more details about this article) |
5/8. Multifocal motor sensory demyelinating neuropathy: inflammatory demyelinating polyradiculoneuropathy.The authors present two cases that provide the first autopsy findings in multifocal acquired demyelinating sensory and motor neuropathy (MADSAMN). Both cases documented multifocal but asymmetric demyelinating neuropathy with rare axonal degeneration. One case clearly documented an inflammatory polyradiculoplexoneuropathy, confirming the inflammatory nature of this neuropathy. This study showed that MADSAMN is an inflammatory demyelinating polyradiculoneuropathy that shares histologic features observed in chronic inflammatory demyelinating polyradiculoneuropathy and multifocal motor neuropathy (MMN), suggesting a similar immunopathogenesis for these entities.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/8. Seeing the invisible: painless aortic dissection in the emergency setting.Acute dissection of the aorta can be one of the most dramatic cardiovascular emergencies. Classically, aortic dissection presents as sudden, severe chest, back, or abdominal pain that is characterised as ripping or tearing in nature. However, a timely diagnosis can be elusive in the event of an atypical presentation. In this report, the authors present two patients with painless aortic dissection who were misdiagnosed during their initial evaluation in the emergency department.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/8. A case of Lewis-Sumner syndrome with conduction abnormalities only in the brachial plexus and roots.We present a case of subacute weakness of one hand with unusual sensory involvement including the upper thorax. Despite normal distal conduction studies, a clinical diagnosis of Lewis-Sumner syndrome was made and the patient responded well to intravenous immunoglobulins. Repeated studies after clinical exacerbation finally proved the demyelinating nature of the neuropathy using proximal magnetic nerve stimulation. This case underlies the importance of seeking proximal conduction blocks in patients with suspected demyelinating neuropathy.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/8. myotonia congenita--a cause of muscle weakness and stiffness.BACKGROUND: A 56-year-old woman was referred to a neurogenetic clinic with a history of stiffness and transient weakness. A previous needle electromyogram had confirmed the presence of myotonia, but a muscle biopsy had revealed no evidence of dystrophy. INVESTIGATIONS: Neurological examination, electrophysiological studies and genetic testing. diagnosis: Recessive myotonia congenita (Becker's disease). MANAGEMENT: Explanation of the nature of the disease and treatment with mexiletine 200 mg twice daily.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |