Long-term recovery of diaphragm strength in neuralgic amyotrophy.
Diaphragm paralysis is a recognized complication of neuralgic amyotrophy that causes severe dyspnoea. Although recovery of strength in the arm muscles, when affected, is common, there are little data on recovery of diaphragm function. This study, therefore, re-assessed diaphragm strength in cases of bilateral diaphragm paralysis due to neuralgic amyotrophy that had previously been diagnosed at the authors institutions. Fourteen patients were recalled between 2 and 11 yrs after the original diagnosis. Respiratory muscle and diaphragm strength were measured by volitional manoeuvres as maximal inspiratory pressure and sniff transdiaphragmatic pressure. Cervical magnetic phrenic nerve stimulation was used to give a nonvolitional measure of diaphragm strength: twitch transdiaphragmatic pressure. Only two patients remained severely breathless. Ten of the 14 patients had evidence of some recovery of diaphragm strength, in seven cases to within 50% of the lower limit of normal. The rate of recovery was variable: one patient had some recovery after 2 yrs, and the rest took 3 yrs or more. In conclusion, in most patients with diaphragm paralysis due to neuralgic amyotrophy, some recovery of the diaphragm strength occurs, but the rate of recovery may be slow. (+info)
Motor root conduction in neuralgic amyotrophy: evidence of proximal conduction block.
OBJECTIVE: To determine the presence and role of proximal conduction block in neuralgic amyotrophy. METHODS: Percutaneous electrical stimulation of cervical roots and brachial plexus was employed in eight patients with neuralgic amyotrophy. Root to Erb's point compound muscle action potential amplitude ratios for abductor digiti minimi, extensor digitorum communis, biceps, and deltoid muscles were compared with results obtained from 10 healthy controls. RESULTS: Conduction block in the nerve to one muscle was found in three of eight patients (38%) suggesting focal proximal demyelination. Repeat studies showed axonal degeneration, resolution, and persistence of conduction block in these three patients respectively. CONCLUSION: Focal conduction block plays a significant part in the pathogenesis of neuralgic amyotrophy, which is generally regarded as an axon loss process. Therapeutic intervention should be directed to patients with persistent conduction block, with the aim of eradicating the block and possibly minimising subsequent axon loss. (+info)
Genetic refinement of the hereditary neuralgic amyotrophy (HNA) locus at chromosome 17q25.
Hereditary neuralgic amyotrophy (HNA) is an autosomal dominant, recurrent focal neuropathy. HNA is characterised by episodes of painful brachial plexus neuropathy with muscle weakness and atrophy, as well as sensory disturbances. Single episodes are commonly preceded by non-specific infections, immunisations or parturition. Mild dysmorphic features and short stature are present in some HNA families, but absolute co-segregation with HNA has not been described. To refine the previously described HNA locus on chromosome 17q25, we performed a genetic linkage study in five HNA families with different geographic origins. Significant linkage was obtained with chromosome 17q24-q25 short tandem repeat (STR) markers in three HNA families and suggestive linkage was found in the other two HNA families. Analysis of the informative recombinations in affected individuals allowed us to reduce the HNA linkage interval to a candidate region of 3.5 cM. (+info)
The natural history of hereditary neuralgic amyotrophy in the Dutch population: two distinct types?
On investigation of 101 attacks in 24 patients with hereditary neuralgic amyotrophy (HNA) from nine different families, we found that HNA can run two distinct courses: a 'classic' relapsing-remitting and a chronic undulating type with exacerbations. Only one type occurred per family, suggesting genetic heterogeneity. This is supported by the finding that only in a family with 'classic type' HNA are data of linkage analysis compatible with linkage to the 17q24-q25 interval which harbours a locus for the disease. The average number of attacks per patient during a follow-up of 26 years was four in the classic form of HNA and five in the chronic undulating type. All patients suffered from residual symptoms on follow-up, with a median Rankin score of 2 in both groups, showing that long-term prognosis is less favourable than previously reported. (+info)
Neuralgic amyotrophy as a presenting feature of infective endocarditis.
A 35 year old man presented to his general practitioner with severe right shoulder pain and subsequent weakness and wasting of the muscles in the affected shoulder girdle three weeks after a dental filling. His symptoms persisted despite standard treatment. He developed malaise, night sweats, weight loss, a petechial rash and a microcytic anaemia. On admission to hospital three months after the start of his symptoms he had also developed splenomegaly and the murmur of aortic regurgitation. Investigations confirmed the diagnoses of infective endocarditis and neuralgic amyotrophy. In this case neuralgic amyotrophy appears to have been the presenting feature of infective endocarditis. This association has not previously been described. (+info)
Acute brachial plexus neuritis: an uncommon cause of shoulder pain.
Patients with acute brachial plexus neuritis are often misdiagnosed as having cervical radiculopathy. Acute brachial plexus neuritis is an uncommon disorder characterized by severe shoulder and upper arm pain followed by marked upper arm weakness. The temporal profile of pain preceding weakness is important in establishing a prompt diagnosis and differentiating acute brachial plexus neuritis from cervical radiculopathy. Magnetic resonance imaging of the shoulder and upper arm musculature may reveal denervation within days, allowing prompt diagnosis. Electromyography, conducted three to four weeks after the onset of symptoms, can localize the lesion and help confirm the diagnosis. Treatment includes analgesics and physical therapy, with resolution of symptoms usually occurring in three to four months. Patients with cervical radiculopathy present with simultaneous pain and neurologic deficits that fit a nerve root pattern. This differentiation is important to avoid unnecessary surgery for cervical spondylotic changes in a patient with a plexitis. (+info)
Outcome of cervical spine surgery in patients with rheumatoid arthritis.
OBJECTIVES: Cervical spine instability in patients with rheumatoid arthritis (RA) may lead to cervical myelopathy or occipital neuralgia, or both. Morbidity and mortality in patients with RA treated with cervical spine surgery during two years of follow up were evaluated. METHODS: Between 1992 and 1996 55 patients with RA underwent cervical spine surgery because of occipital neuralgia or cervical myelopathy, or both. Patients were classified according to the Ranawat criteria for pain and neurological assessment before operation and three months and two years postoperatively. For occipital neuralgia a successful operation was defined as complete relief of pain and for cervical myelopathy as neurological improvement. RESULTS: Occipital neuralgia was present in 17 patients, cervical myelopathy in 14 patients, and 24 had both. Surgical treatment in the patients with symptoms of occipital neuralgia who were still alive two years after surgery was successful in 18/29 (62%). In the surviving patients with cervical myelopathy neurological improvement of at least one Ranawat class was seen in 16/24 (67%). Postoperative mortality within six weeks was 3/51 (6%). Within two years after the operation 14 /51 (27%) of the patients had died; in most patients the cause of death was not related to surgery. The highest mortality (50%) was found in the group of six patients with quadriparesis and very poor functional capacity (Ranawat IIIB). CONCLUSION: Cervical spine surgery in patients with RA performed because of occipital neuralgia or cervical myelopathy, or both, is successful in most patients who are alive two years after surgery. However, the mortality rate during these two years is relatively high, which seems to be largely related to the severity of the underlying disease and not to the surgery itself. (+info)
Idiopathic brachial neuritis.
Idiopathic brachial neuritis is a well defined clinical condition that most commonly affects young adults, seen usually by primary care physicians, neurologists or orthopaedic surgeons. Its onset is characterized by acute, aching shoulder pain lasting a few days to weeks, followed by progressive shoulder girdle and upper extremity weakness and atrophy, with a slow but progressive recovery of motor function over 6 to 18 months. Its early recognition can help avoid unnecessary and potentially harmful diagnostic and therapeutic interventions, and avoid delays in prescribing appropriate therapies that may be helpful only early in the course of the disease. We present a case of idiopathic brachial neuritis and discuss important aspects of the disease and difficulties in reaching the correct diagnosis. (+info)