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Pediatric radial mononeuropathies: a clinical and electromyographic study of sixteen children with review of the literature.

Sixteen pediatric radial mononeuropathies were seen among 2077 electromyograms performed in the electromyography laboratory at The Children's Hospital, Boston, during 16.5 years, 1979-1995. Eight (50%) of these radial neuropathies, including 2 in newborns with apparent prenatal onset, were atraumatic, primarily related to compression in 6 and entrapment in 2. The other 8 (50%) were traumatic related to fractures or lacerations. Electromyography documented the radial neuropathy to be localized to the proximal main radial nerve trunk in 2 (13%), distal main radial nerve trunk in 9 (56%), and posterior interosseous nerve in 5 (31%) children. Significant improvement was noted in 13 of the 16 radial neuropathies--within 6-12 weeks for demyelinating lesions and up to 17 months for axonal injuries. Rarely, a child with a chronic progressive radial neuropathy or a postfracture radial neuropathy that does not improve in 3 months may require exploration.

Adolescent↗

Rare mononeuropathies of the upper limb in bodybuilders.

Clinical and electrophysiological findings of mononeuropathies of thoracodorsal, dorsoscapular, terminal branch of suprascapular, and medial pectoral nerves are reported in 4 bodybuilders. All athletes used anabolic steroids. After about 1 year partial improvement of symptoms was evident in 2 cases and complete recovery in the other 2. The data are discussed in the light of the few other existing reports and possible pathogenetic mechanisms.

Acute Disease↗

Mononeuropathy multiplex due to infiltration of lymphoma in hematologic remission.

We report a case of progressive mononeuropathy multiplex in a patient with lymphoma in hematologic remission. At the time of presentation there was no evidence of meningeal or central nervous system metastasis. At autopsy, extensive infiltration of tumor cells was found in both femoral nerves. We review the literature pertaining to multifocal malignant lymphoid infiltration of peripheral nerves, which can occur during hematologic remission or in the absence of any evidence of systemic lymphoma.

Fatal Outcome↗

Treatment of mononeuropathy multiplex in hepatitis C virus and cryoglobulinemia.

Recent reports advocate alpha-interferon (alpha-Ifn) treatment for mononeuropathy multiplex in hepatitis C virus-associated cryoglobulinemia. We report 2 patients with this disorder to describe two underrecognized treatment outcomes--worsening of polyneuropathy with initiation of alpha-Ifn, in the absence of immunosuppression, and deterioration of liver function with prednisone, despite improvement of polyneuropathy.

Adult↗

Mononeuropathy multiplex in rhesus monkeys with chronic Lyme disease.

Peripheral neuropathy is a recognized but poorly understood manifestation of Lyme disease. We performed serial electrophysiological studies on 8 rhesus monkeys chronically infected with the JD1 strain of Borrelia burgdorferi and compared the results with those of similar studies on 10 uninfected control monkeys. Four infected and 2 uninfected animals underwent sural nerve biopsy. Five of the infected and 1 of the uninfected animals also had postmortem neuropathological examinations. Altogether, 5 of the infected monkeys demonstrated primarily axonal-loss-variety multifocal neuropathies. Only one nerve lesion exhibited findings compatible with demyelination. Pathologically, peripheral nerve specimens showed multifocal axonal degeneration and regeneration and occasional perivascular inflammatory cellular infiltrates without vessel wall necrosis. Free spirochetal structures were not seen, but several macrophages exhibited positive immunostaining with a highly specific anti-B. burgdorferi, 7.5-kd lipoprotein monoclonal antibody. In the infected animals, serial analysis of serum antibodies to B. burgdorferi showed increasing numbers of IgG specificities and new IgM specificities, suggesting persistent infection. Thus, peripheral neuropathy in the form of a mononeuropathy multiplex develops frequently in rhesus monkeys chronically infected with B. burgdorferi. The pathogenesis of these nerve lesions is not yet known, but our studies suggest an immune-mediated process perhaps driven by persistent infection with B. burgdorferi.

Animals↗

Gender, body mass and age as risk factors for ulnar mononeuropathy at the elbow.

Factors that predispose patients to ulnar mononeuropathy at the elbow (UME) are poorly defined. We compared 112 electrodiagnostic reports which met criteria for definite or probable UME to 104 reports which excluded UME. Male gender was strongly associated with definite (OR = 6.9, 95% CI = 2.4-20.4; P < 0.001) and all UME (OR = 2.2, 95% CI = 1.2-4.1; P = 0.010) after controlling for age and body mass index (BMI). Among men, UME was associated with increasing age (P = 0.008) but not a decreased BMI. Women, however, demonstrated an association between decreased BMI and UME (OR = 2.3, 95% CI = 1.3-4.2 for BMI < or =22.0 versus >22.0). These findings, in conjunction with gender differences in ulnar motor nerve parameters among UME subjects and controls, suggest that the pathophysiology of UME differs with gender.

Age Distribution↗

Mononeuropathy multiplex associated with acute hepatitis B infection.

Immunologic syndromes are associated with hepatitis B viral (HBV) infection. However, mononeuropathy multiplex (MM), a syndrome in which immune factors may play a role, is rare in acute HBV infection. Few cases of MM associated with HBV infection have been reported. We report a case of acute HBV infection in which MM involving the median, ulnar and lateral femoral cutaneous nerves complicated the course of the acute illness. Skin biopsy demonstrated vasculitic changes which may accompany the immunologic factors in HBV infection.

Acute Disease↗

Electrophysiological findings in localized hypertrophic mononeuropathy.

Localized hypertrophic mononeuropathy (LHM) is a rare foccal neuropathy associated with perineurial cell proliferation due to an undefined stimulus. We report a case of LHM involving the proximal ulnar nerve in a 13-year-old boy. Serial clinical and electrophysiological observations were done, including intraoperative recordings directly from the nerve on two occasions. The evolution of abnormalities, as documented by electrophysiological and immunohistochemical studies, is compatible with a process of progressive thinning of myelin sheaths, culminating in axonal degeneration in later stages. Intraoperative recording of nerve action potentials was used to guide surgical management of the lesion. Ultimately, there was a good clinical and electrophysiological response to resection of the lesion and sural nerve fascicular grafting.

Action Potentials↗

Mononeuropathy in sickle cell anemia: anatomical and pathophysiological basis for its rarity.

Peripheral neuropathy is a rare complication of sickle cell disease. We report a young black woman with sickle cell anemia who developed a proximal median mononeuropathy in the setting of sickle cell crisis. The clinical and electrodiagnostic features are consistent with an ischemic mechanism from the sickling process. The pathophysiological basis for the rarity of this complication may be related to the rich anastomotic microvasculature of peripheral nerve and the unique large size of the capillaries of this vascular network.

Adult↗

Femoral mononeuropathy induced by the lithotomy position: a report of 5 cases with a review of literature.

We report 5 young women who developed femoral mononeuropathy (FMN) after placement in the lithotomy position. Three had uncomplicated vaginal deliveries and 2 underwent exploratory laparoscopies. EMG was performed on 3 patients. The lesions were localized to the inguinal ligament and probably demyelinating, accounting for their excellent prognosis. On reviewing the literature, however, we believe that lithotomy position can cause FMN, not only by nerve compression at the inguinal ligament, but also by stretching of the nerve by excessive hip abduction and external rotation.

Adult↗

Paraneoplastic pseudo-obstruction, mononeuropathy multiplex, and sensory neuronopathy.

A patient with mononeuropathy multiplex, sensory neuronopathy, and diffuse intestinal pseudo-obstruction consisting of gastroparesis and impaired small and large bowel motility was found to have a small cell carcinoma of the lung. The constellation of findings were indicative of paraneoplastic neuropathy, which was confirmed with the appropriate antibody studies demonstrating antineuronal nuclear antibodies. Identification of paraneoplastic neuropathy is important, since early treatment of the primary cancer, which is virtually always a small cell carcinoma of the lung, may halt the progression of the disorder.

Aged↗

Pediatric median mononeuropathies: a clinical and electromyographic study.

Seventeen children, 6 girls and 11 boys, aged 5-17 years with pediatric median mononeuropathies (PMM) were identified among 1809 who had EMGs primarily in the electromyographic laboratory at The Children's Hospital, Boston, between 1979 and 1993. Electromyography documented the PMM to be at the wrist in 7 children, including 3 children with idiopathic carpal tunnel syndrome (CTS)--1 whose symptoms were accentuated by skiing--2 with a systemic illness (mucolipidosis III and scleroderma), and in 1 child each the distal PMM was secondary to a cast or laceration. A proximal PMM was identified in 10 children, including 8 with trauma, 1 with an osteoid osteoma, and 1 with juvenile cutaneous mucinosis. Five children (3 with CTS and 1 each with mucolipidosis III and juvenile cutaneous mucinosis) had bilateral disease. The localization (59% proximal) and cause of these PMMs differed greatly from our experience with adult median neuropathies.

Adolescent↗

Isolated femoral mononeuropathy to the vastus lateralis: EMG and MRI findings.

A 16-year-old female track athlete experienced sudden onset of right anterolateral thigh pain, initially thought to be cramping. After 2 months of continued postexercise pain she sought medical evaluation. A 3-cm thigh circumference discrepancy was noted on physical exam. She was referred for electromyography (EMG) and magnetic resonance imaging (MRI) studies. Femoral and screening nerve conduction studies were normal. Needle EMG revealed acute neuropathic changes confined to the right vastus lateralis only. These findings were confirmed by MRI, including short T1 inversion recovery (STIR) sequences. All other medical work-up was normal, including lumbar and pelvic MRI, and complete serologic studies. This case represents a unique presentation of an idiopathic femoral mononeuropathy isolated to the vastus lateralis only, which has not been previously reported. The highly unusual anatomical presentation of this case illustrates the emerging complementary usefulness of EMG and MRI in delineating neuromuscular pathology.

Action Potentials↗

Cutaneous nerve terminal degeneration in painful mononeuropathy.

Nociceptive nerves innervate the skin and play an important role in the generation of neuropathic pain. However, it remains elusive whether and how nociceptive nerve terminals degenerate in neuropathic pain conditions. To address this issue, we investigated cutaneous innervation in a model of painful mononeuropathy, the chronic constriction injury (CCI). The hind paws of rats were immunocytochemically stained with a pan-axonal marker, protein gene product 9.5 (PGP 9.5). Within 2 days after CCI, rats exhibited thermal hyperalgesia, and there was a partial depletion of epidermal nerves. The extent of reduction in epidermal nerves after CCI was variable with an epidermal nerve density of 3.65 +/- 1.97 fibers/mm (compared to 15.39 +/- 1.58 fibers/mm on the control side, P < 0.02). There was a mild but concomitant increase in PGP 9.5 (+) Langerhans cells in the epidermis of the skin with CCI (10.19 +/- 1.99 vs 7.75 +/- 1.36 cells/mm, P < 0.05). In the skin denervated by tight ligation of the sciatic nerve, epidermal nerves were completely depleted (0 fibers/mm vs. 12.26 +/- 1.44 fibers/mm on the control side, P < 0.001). Animals with tight ligation of the sciatic nerve exhibited thermal anesthesia. These findings suggest that the epidermis is partially denervated in CCI, and that a partial injury of nerves is correlated with the development of neuropathic pain.

Animals↗

The antinociceptive effect of moclobemide on the vocalization threshold to paw pressure in a rat model of unilateral mononeuropathy.

The aim of our study was to investigate the antinociceptive activity of moclobemide on the vocalization threshold to paw pressure in a rat model of unilateral mononeuropathy. The neuropathy was produced by ligation of the sciatic nerve and nociceptive thresholds were determined 15-21 days after surgery by a modification of the Randall-Sellito method. Group 1 (n= 10) received 0.2 ml peroral (p.o.) saline, Group 2 (n= 10) 5 mg x kg(-1), Group 3 (n= 10) 10 mg x kg(-1) and Group 4 (n= 10) 20 mg x kg(-1) p.o. moclobemide. Nociceptive pressure thresholds were then measured every 20 minutes after drug administration. Analysis of variance, Tukey's test and a paired Student's t-test were employed for statistical analysis. The perorally administered moclobemide (5, 10 and 20 mg x kg(-1)) produced an antinociceptive effect on both lesioned and non-lesioned hind paws ( P< 0.05). However, the analgesic effect on the lesioned paw was significantly more potent than the non-lesioned paw. The peak value ( p) remained constant while the maximal increment between the control threshold and the peak value ( I(max)) was significantly more pronounced for the lesioned paw ( P< 0.001). The results of this study may suggest that moclobemide can be a therapeutic alternative to treat some clinical symptoms in peripheral neuropathic conditions.

Analgesics↗

Italian multicentre study of peroneal mononeuropathy at the fibular head: study design and preliminary results.

BACKGROUND: The most common entrapment in the lower extremity is peroneal mononeuropathy (PM) at the fibular head. Several studies of this condition have been published but, until now, no wide multicenter clinical-neurophysiological studies on PM are available. In recent years, multicenter studies have been suggested; moreover it is commonly accepted that a multiperspective approach provides more comprehensive results. METHOD: The Italian CTS and other entrapments Study Group has designed a strict clinical and neurophysiological protocol to carry out a wide multicentre study on PM at the fibular head. In addition to traditional clinical-neurophysiological evaluation, the group has also adopted validated disability and patient-oriented measurements in order to obtain more comprehensive and reliable data about this entrapment. The study was designed: 1) to identify predisposing factors; 2) to better assess the clinical picture; 3) to evaluate relationships between etiological, clinical and neurophysiological findings; 4) to evaluate the natural evolution of the entrapment. Study design is described. FINDINGS: During the period from November 2002 to January 2004, 69 patients were enrolled consecutively in eleven Italian centres. Our preliminary data show that PM involves men more frequently than women (M:F = 3.9:1). With regard to the predisposing factors, PM is idiopathic (16%) or due to surgery (21.7%), prolonged posture (23.2%), weight loss (14.5%), external compression (5.8%), arthrogenic cyst at the fibula (1.4%), trauma (10.1%); it also occurred in bedridden patients (7.3%). Unexpectedly, peroneal nerve lesions were due not only to surgical operation close to the peroneal region, but were also associated with thoracic-abdominal surgery. Usually PM involves both terminal branches; patients complain of motor deficit in 99.5% of cases, sensory symptoms in 87.9% and pain in 19.7%. CONCLUSIONS: Our preliminary results provide some interesting information and confirm the usefulness of multicentre and multiperspective studies to standardise the approach to nerve entrapment.

Adolescent↗

Baroreceptor denervation presenting as part of a vagal mononeuropathy.

A 48-year-old woman presented with a history of progressive cough, dysphonia, dysphagia, and postural symptoms. Subsequent neurological investigations were consistent with a bilateral vagal mononeuropathy, and neurosarcoidosis was diagnosed after scalene node biopsy. Autonomic investigations including microneurography, neurohormones, and heart rate variability demonstrated arterial and cardiopulmonary baroreflex failure. In addition, parasympathetic control of heart rate was absent and consistent with a bilateral, nonselective lesion in the proximal vagus.

Arginine Vasopressin↗

Diabetic truncal mononeuropathy: electromyographic evaluation.

Fifteen diabetic subjects wih thoracic radiculopathy were studied wih paraspinal electromyography. The majority were elderly, mature onset diabetics presenting with unilateral hyperesthesia in a thoracic radicular pattern. None was under adequate control at onset, but control did not seem to alter the symptoms. Electromyography demonstrated localized paraspinal positive waves at the appropriate clinical level. Although not all subjects presented wih peripheral neuropathy, the majority showed electromyographic alteration. This should be an important adjunct in evaluation of diabetic patients with truncal mononeuropathy.

Adult↗