High resolution sonography in the evaluation of the peripheral nervous system in polyneuropathy--a review of the literature.

Clinical, laboratory and electrodiagnostic studies are the mainstay in the diagnosis of polyneuropathy. An accurate etiological diagnosis is of paramount importance to provide the appropriate treatment, prognosis and genetic counselling. High resolution sonography of the peripheral nervous system allows nerves to be readily visualized and to assess their morphology. Ultrasonography has brought pathophysiological insights and substantially added to diagnostic accuracy and treatment decisions amongst mononeuropathies. In this study the literature on its clinical application in polyneuropathy is reviewed. Several polyneuropathies have been studied by means of ultrasound: Charcot-Marie-Tooth, hereditary neuropathy with liability to pressure palsies, chronic inflammatory demyelinating polyneuropathy, Guillain-Barré syndrome, multifocal motor neuropathy, paraneoplastic polyneuropathy, leprosy and diabetic neuropathy. The most prominent reported pathological changes were nerve enlargement, increased hypo-echogenicity and increased intraneural vascularization. Sonography revealed intriguingly different patterns of nerve enlargement between inflammatory neuropathies and axonal and inherited polyneuropathies. However, many studies concerned case reports or case series and showed methodological shortcomings. Further prospective studies with standardized protocols for nerve sonography and clinical and electrodiagnostic testing are needed to determine the role of nerve sonography in inherited and acquired polyneuropathies.

Treatment of the sacroiliac joint in patients with leg pain: a randomized-controlled trial.

PURPOSE: The sacroiliac joint (SIJ) may be a cause of sciatica. The aim of this study was to assess which treatment is successful for SIJ-related back and leg pain. METHODS: Using a single-blinded randomised trial, we assessed the short-term therapeutic efficacy of physiotherapy, manual therapy, and intra-articular injection with local corticosteroids in the SIJ in 51 patients with SIJ-related leg pain. The effect of the treatment was evaluated after 6 and 12 weeks. RESULTS: Of the 51 patients, 25 (56 %) were successfully treated. Physiotherapy was successful in 3 out of 15 patients (20 %), manual therapy in 13 of the 18 (72 %), and intra-articular injection in 9 of 18 (50 %) patients (p = 0.01). Manual therapy had a significantly better success rate than physiotherapy (p = 0.003). CONCLUSION: In this small single-blinded prospective study, manual therapy appeared to be the choice of treatment for patients with SIJ-related leg pain. A second choice of treatment to be considered is an intra-articular injection.

Occurrence of CCSVI in patients with MS and its relationship with iron metabolism and varicose veins.

BACKGROUND AND PURPOSE: A new treatable venous disorder, chronic cerebrospinal venous insufficiency (CCSVI), has been proposed in patients with multiple sclerosis (MS). Its relationship with iron metabolism is suggested, but has not been examined prospectively. METHODS: We performed extra- and transcranial echo colour Doppler (ECD) in 90 patients with MS and 41 healthy controls (HC). Indices of iron metabolism and the presence of peripheral signs of impaired venous flow were also examined. RESULTS: The ECD examination showed CCSVI in 8 (9%) of the 90 patients with MS and 0 HC (P = 0.11). The 8 CCSVI-positive MS patients were older (P = 0.02), had less often relapsing-remitting-MS (P = 0.02) and had more neurological disability assessed by expanded disability status scale (EDSS, P = 0.001) and longer duration of disease (P = 0.02) in comparison with the 82 CCSVI-negative MS patients. Multivariate analysis revealed that EDSS remained an independent factor associated with CCSVI (odds ratio 1.89, 95%CI 1.17-3.05, P-value = 0.009). CCSVI MS patients more often had bilateral telangiectasia at the legs (P = 0.008), reticular veins (P = 0.006) and venous stasis dermatitis (P = 0.004). No relationship was found between CCSVI and impaired iron metabolism in patients with MS. CONCLUSIONS: CCSVI is uncommon and is a secondary epiphenomenon in MS and related to more neurological disability and the presence of varicose veins at the legs.

The clinical, electrophysiological and prognostic heterogeneity of ulnar neuropathy at the elbow.

BACKGROUND: Ulnar nerve neuropathy is one of the most common entrapment neuropathies and is often considered to be one entity in clinical and follow-up studies. OBJECTIVE: The aim of this study was to determine whether there is a difference in the long term outcome of patients with a severe motor conduction block (mCB) of > or =50% (50% group) in comparison with patients with an mCB of < or =20% (20% group). METHODS: In a prospective cohort of 244 patients with ulnar neuropathy at the elbow (UNE), 16% had an mCB of > or =50%. These patients were matched with respect to surgery, age and severity of muscle weakness with patients with an mCB of < or =20%. 32 patients per group were evaluated clinically and electrophysiologically. RESULTS: The median follow-up period was 25 months. Based on the patient's assessment, 26 (81%) patients in the 50% group and 14 (44%) patients in the 20% group had a good outcome (p = 0.005). The clinical examination showed a good outcome in 27 (84%) in the 50% group and in 13 (41%) in the 20% group (p = 0.0008). CONCLUSION: This study showed that the prognosis of patients with focal demyelination of the ulnar nerve presenting with an mCB of > or =50% is more favourable than those without an mCB.

Critical illness polyneuropathy and myopathy: clinical features, risk factors and prognosis.

Acquired neuromuscular weakness due to critical illness polyneuropathy and myopathy (CIPNM) frequently develops in patients hospitalized in the intensive care unit for more than 1 week. CIPNM may present with muscle weakness and failure to wean from mechanical ventilation, but is discovered more often and earlier by electrophysiological examination. In this review, the incidence, clinical and electrophysiological features, differential diagnosis and prognosis of CIPNM will be described. Risk factors for CIPNM are sepsis or systemic inflammatory response syndrome and the severity of multi-organ failure. Presence of CIPNM is associated with higher mortality rate, prolonged duration of mechanical ventilation and prolonged rehabilitation. The majority of survivors with CIPNM have persistent functional disabilities and a reduced quality of life. There is need for new therapeutic strategies to prevent or minimize CIPNM in critically ill patients.

Time to diagnosis of intraspinal tumors.

The aim of this study was to assess the time to diagnosis in patients with intraspinal tumors and to assess factors contributing to a long delay in some patients. Patients who were admitted at our hospital over a 15-year period (1986-2000) with an intraspinal (either intradural or extradural) tumor were included. Records of patients were studied for variables such as sex, age, diagnosis, date of diagnosis, initial symptoms, symptoms at diagnosis, level of tumor, original diagnosis and diagnostic technique [CT-myelography, CT-caudography or magnetic resonance imaging (MRI)]. The median time to diagnosis of 108 patients with an intraspinal tumor was 12.3 months (range: 4 days-14.4 years). Most common initial symptoms were back and/or neck pain, pain radiating to one extremity and walking disturbances. There was no distinctive clinical pattern between intramedullary and extramedullary tumors regarding initial symptoms. At the time of diagnosis, patients presented with moderate to severe neurological deficits: weakness in one extremity in 26%, sphincter disturbance (20%) and paraparesis (12%). Improved imaging of the spinal cord by MRI did not result in earlier detection of the intraspinal tumor. The time to diagnosis is explained by non-specific and slowly progressing signs and symptoms. A high rate of clinical suspicion should be present to diagnose an intraspinal tumor at an early stage.

Ulnar neuropathy at the elbow: follow-up and prognostic factors determining outcome.

OBJECTIVE: To determine the outcome in patients with ulnar neuropathy at the elbow (UNE) treated surgically or conservatively, and the prognostic value of clinical, sonographic, and electrophysiologic features. METHODS: After a median follow-up of 14 months, 69 of 84 patients initially included in a prospective blinded study on the diagnostic value of sonography in UNE were re-evaluated. The patients underwent renewed systematic clinical and sonographic examination. Patients were scored as having a poor (stable or progressive symptoms) or favorable (complete remission of symptoms or improvement) outcome. RESULTS: Of the 74 initially affected arms, 12 (16%) had a complete remission, 21 (28%) improved, 25 (34%) remained stable, and 16 (22%) had progression. Surgically treated patients (28 arms) had a more favorable outcome than those treated conservatively (p = 0.03). After surgery, the mean ulnar nerve diameter decreased from 3.2 to 2.9 mm (p = 0.03), while this was not seen after conservative treatment. Multiple logistic regression analysis showed that more outspoken nerve enlargement found during sonography at the time of the diagnosis was associated with a poor outcome (OR: 2.9, p = 0.009). Furthermore, the presence of a motor conduction block (OR: 0.2, p = 0.03) and motor velocity slowing across the elbow (OR: 0.1, p = 0.01) were associated with a favorable outcome. CONCLUSION: More pronounced ulnar nerve thickening at the time of the diagnosis is associated with poor outcome at follow-up, especially in conservatively treated cases, while electrodiagnostic signs of demyelination on testing indicate favorable outcome.

Difference in distribution of muscle weakness between myasthenia gravis and the Lambert-Eaton myasthenic syndrome.

BACKGROUND: Myasthenia gravis and the Lambert-Eaton myasthenic syndrome (LEMS) may have a similar distribution of muscle weakness. Deciding on a diagnosis of myasthenia gravis or LEMS on clinical grounds may therefore be difficult. OBJECTIVE: To compare the localisation of initial muscle weakness and the distribution of weakness at the time of maximum severity in patients with myasthenia gravis and LEMS. SUBJECTS: 101 patients with myasthenia gravis and 38 patients with LEMS. RESULTS: In myasthenia gravis, initial weakness involved extraocular muscles in 59%, bulbar muscles in 29%, and limb muscles in 12% of the patients. In LEMS no patient had ocular weakness, 5% had bulbar weakness, and 95% had weakness of the limbs as the first symptom (p < 0.001). At the point of maximum severity, weakness in myasthenia gravis was purely ocular in 25%, oculobulbar in 5%, restricted to the limbs in 2%, and present in both oculobulbar muscles and limbs in 68%. At this point, none of the LEMS patients had weakness restricted to extraocular or bulbar muscles (p = 0.002). The legs were affected in all LEMS patients, whereas in 12 patients with generalised myasthenia gravis limb weakness was restricted to the arms (p = 0.024). CONCLUSIONS: In a patient suspected to have a myasthenic syndrome whose first symptom is ocular weakness, LEMS is virtually excluded. Limb weakness confined to the arms is only found in generalised myasthenia gravis and not in LEMS. Muscle weakness in myasthenia gravis tends to develop in a craniocaudal direction, and in the opposite direction in LEMS.

HLA class I and II in Lambert-Eaton myasthenic syndrome without associated tumor.

Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disorder, in which antibodies against voltage-gated calcium channels located at nerve terminals cause muscle weakness and autonomic dysfunction. In approximately half of the patients the autoimmune process is initiated by a tumor. In the other half of patients no tumor is found and the etiology is unknown. The aims of this study were to investigate the strength of HLA-associations with nontumor LEMS (NT-LEMS) and to study the relation of HLA-haplotypes with age at onset of LEMS and other clinical features. Therefore, typing of HLA class I and II was performed in 19 patients with NT-LEMS, who were clinically evaluated. NT-LEMS was significantly associated with alleles of both HLA-class I (i.e. HLA-B8) as well as -class II (i.e. HLA-DR3 and -DQ2). HLA-B8+ patients had significantly younger age at onset of LEMS and tended to be female. This study shows that HLA-class I haplotype is associated with a distinct phenotype in NT-LEMS.

Critical illness polyneuropathy and myopathy (CIPNM): evidence for local immune activation by cytokine-expression in the muscle tissue.

In a longitudinal prospective study a muscle biopsy was taken from 30/32 (33%) of the 98 patients who developed critical illness polyneuropathy and myopathy (CIPNM). Neuropathic changes were found in 37%, myopathic in 40%, and a combination in 23% of the biopsies. The immunohistopathology showed macrophages and Th-cells in 40% and 60% of the muscle biopsies respectively. Small mainly perivascular infiltrates contained macrophages and Th-cells. ICAM-1, VCAM and MAC were found on the vascular endothelium in 58%, 53% and 79% respectively. In all biopsies there was an upregulation of both HLA-I and HLA-DR. Proinflammatory cytokines and TNFalphaR75 were also produced locally (IL-1beta in 71%, IFN-gamma in 40%, IL-12 in 73%, TNFalphaR75 in 90%). The anti-inflammatory cytokine IL-10 was simultaneously expressed in 96% of the biopsies. HLA-DR, TNFalphaR75 and IL-10 differed significantly when compared with control muscle biopsies. Our data provide evidence that small numbers of activated leukocytes producing both pro- and anti-inflammatory cytokines infiltrate skeletal muscle of CIPNM patients. We propose that the local balance of leukocyte activities is of importance in the pathophysiology of muscle weakness in CIPNM.

Hemiparkinsonism in a patient with primary Sjögren's syndrome. A case report and a review of the literature.

A 55-year-old woman presented with hemiparkinsonism on the left side. Clinical features suggested primary Sjögren's syndrome. Eye tests and a salivary gland biopsy confirmed this diagnosis. Magnetic resonance imaging of the brain showed distinct linear lesions of increased intensity on the T2-weighted images in the right striatum and globus pallidum. Treatment with prednisone and azathioprine did not give any improvement of the neurological symptoms. To date, hemiparkinsonism in association with primary Sjögren's syndrome has not been reported.