The US health care system: Part 1: Our current system.

The US health care crisis is of great concern to American neurologists. The United States has the world's most expensive health care system yet one-sixth of Americans are uninsured. The cost and volume of procedures is expanding, while reimbursement for office visits is declining. Pharmaceutical costs, durable goods, and home health care are growing disproportionately to other services. Carriers spend more for their own administration and profit than on payments to physicians. This first article on the US health care system identifies problems and proposes solutions, many of which are championed by the American Academy of Neurology through its legislative and regulatory committees.

Invited article: the US health care system: part 2: proposals for improvement and comparison to other systems.

In the search for a universal, high quality, affordable health care system, Americans seek to identify and correct a series of current problems. In part one of this two-part series, we presented problems along with some suggested actions. This second part presents other health care systems in Europe and Canada. These different systems provide universal care and at a lower cost than in the United States. Further domestic proposals are presented from the Massachusetts plan and positions from US presidential candidates. These systems and proposals raise ideas about possible changes in the US health care system. Knowledge of these issues and other health care systems will help foster a meaningful dialog about changes in the US health care system.

Acute sensory neuropathy associated with rabbit antithymocyte globulin.

Rabbit antithymocyte globulin (RATG) is indicated for the treatment of acute renal transplant rejection and has also been shown to be effective as an induction immunosuppressive agent after renal transplantation. We report a patient that developed a painful sensory neuropathy within an hour of receiving RATG. The neuropathic symptoms resolved within a month, and a careful review of his medications, exposures and comorbid conditions revealed no other causes of neuropathy. Since the administration of RATG and onset of symptoms were so closely related temporally and the symptoms resolved after the cessation of RATG, we believe it is likely this medication led to the development of neuropathy.

Topiramate vs placebo in painful diabetic neuropathy: analgesic and metabolic effects.

BACKGROUND: Using identical methods, three simultaneous placebo-controlled trials of topiramate for painful diabetic neuropathy (PDN) did not reach significance. This independent yet concurrent placebo-controlled trial used different methods to assess topiramate efficacy and tolerability in PDN. METHODS: This 12-week, multicenter, randomized, double-blind trial included 323 subjects with PDN and pain visual analog (PVA) score of at least 40 on a scale from 0 (no pain) to 100 (worst possible pain). Topiramate (n = 214) or placebo (n = 109) was titrated to 400 mg daily or maximum tolerated dose. Short-acting rescue analgesics were permitted only during the first 6 weeks. RESULTS: Baseline characteristics were comparable between groups except for mean body weight (topiramate, 101.4 kg; placebo, 95.7 kg; p = 0.028). Twelve weeks of topiramate treatment reduced PVA scale score (from 68.0 to 46.2 mm) more effectively than placebo (from 69.1 to 54.0 mm; p = 0.038). Fifty percent of topiramate-treated subjects and 34% of placebo-treated subjects responded to treatment, defined as >30% reduction in PVA scale score (p = 0.004). Topiramate monotherapy also reduced worst pain intensity (p = 0.003 vs placebo) and sleep disruption (p = 0.020 vs placebo). Diarrhea, loss of appetite, and somnolence were the most commonly reported adverse events in the topiramate group. Topiramate reduced body weight (-2.6 vs +0.2 kg for placebo; p < 0.001) without disrupting glycemic control. CONCLUSIONS: Topiramate monotherapy reduced pain and body weight more effectively than placebo in patients with painful diabetic neuropathy.

Spontaneously remitting dermatomyositis.

Dermatomyositis (DM) is an inflammatory myopathy of skeletal muscle with characteristic cutaneous findings. It is a rare disorder with a bimodal age distribution that affects almost twice as many women as men. One category of DM, normal-enzyme DM, is characterized by cutaneous changes only at baseline, normal serum muscle enzyme levels and myositis demonstrated by electromyography (EMG) and/or muscle biopsy specimens. Typically, patients with normal-enzyme DM progress to severe muscle involvement and require systemic corticosteroid therapy. The patient we report has normal-enzyme DM confirmed by serial serum enzymes, EMG, and skin and muscle biopsies but is unique in that she never experienced progression of muscle weakness although muscle involvement was documented histologically and by EMG. Follow-up examination after 1 year revealed near-complete resolution of cutaneous involvement after topical therapy and no evidence of muscle weakness.

A randomized, placebo-controlled trial of topiramate in amyotrophic lateral sclerosis.

OBJECTIVE: To determine if long-term topiramate therapy is safe and slows disease progression in patients with ALS. METHODS: A double-blind, placebo-controlled, multicenter randomized clinical trial was conducted. Participants with ALS (n = 296) were randomized (2:1) to receive topiramate (maximum tolerated dose up to 800 mg/day) or placebo for 12 months. The primary outcome measure was the rate of change in upper extremity motor function as measured by the maximum voluntary isometric contraction (MVIC) strength of eight arm muscle groups. Secondary endpoints included safety and the rate of decline of forced vital capacity (FVC), grip strength, ALS functional rating scale (ALSFRS), and survival. RESULTS: Patients treated with topiramate showed a faster decrease in arm strength (33.3%) during 12 months (0.0997 vs 0.0748 unit decline/month, p = 0.012). Topiramate did not significantly alter the decline in FVC and ALSFRS or affect survival. Topiramate was associated with an increased frequency of anorexia, depression, diarrhea, ecchymosis, nausea, kidney calculus, paresthesia, taste perversion, thinking abnormalities, weight loss, and abnormal blood clotting (pulmonary embolism and deep venous thrombosis). CONCLUSIONS: At the dose studied, topiramate did not have a beneficial effect for patients with ALS. High-dose topiramate treatment was associated with a faster rate of decline in muscle strength as measured by MVIC and with an increased risk for several adverse events in patients with ALS. Given the lack of efficacy and large number of adverse effects, further studies of topiramate at a dose of 800 mg or maximum tolerated dose up to 800 mg/day are not warranted.

Randomized controlled trial of IVIg in untreated chronic inflammatory demyelinating polyradiculoneuropathy.

OBJECTIVE: To determine the efficacy of IV immunoglobulin (IVIg) given patients with untreated chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). METHODS: A randomized, double-blind, multicenter, investigator-initiated study compared IVIg (Aventis Behring LLC, King of Prussia, PA) with placebo (5% albumin). On days 1, 2, and 21, IVIg (1 g/kg) or placebo was given. The primary outcome measure was the change in muscle strength from baseline to day 42, using the average muscle score (AMS). Secondary outcome measures included change from baseline AMS at days 10 and 21, the Hughes' functional disability scale, forced vital capacity (FVC), and nerve conduction studies (NCS) of four motor nerves (median, ulnar, peroneal, and tibial). RESULTS: The patients (n = 33) were randomized. Of these, 30 (14 women, 16 men, aged 54 +/- 20 years, range 13 to 82) received IVIg and 23 were given placebo (12 women, 11 men, aged 50 +/- 18 years, range 23 to 73). Baseline AMS values of the groups were similar (IVIg 7.06 +/- 1.31 versus placebo 7.28 +/- 1.18, p = 0.53). There were two dropouts in placebo group and one in the IVIg group. Mean AMS improved at day 42 comparing IVIg with placebo (0.63 versus -0.1, p = 0.006). Improved strength was seen by day 10. The placebo group lost strength over this same interval. In the IVIg, 11 subjects improved by the functional disability scale; none worsened. This differed (p = 0.019) from those in the placebo-treated group (two improved, two got worse, remainder unchanged). Forced vital capacity did not improve with IVIg treatment. IVIg improved ulnar motor distal latency (p = 0.005), tibial distal compound muscle amplitude (p = 0.003), and peroneal nerve conduction velocity (p = 0.03). CONCLUSIONS: IVIg improves strength in patients with untreated CIDP by day 10 with continued benefit through day 42; more than one third improve by at least a functional grade on a disability scale. This study provides data supporting IVIg as the initial treatment for CIDP.

Electrophysiologic evaluations.

Electrophysiologic testing in concert with the neurologic history and physical examination can be useful for evaluating the patient with suspected neurotoxicity. Procedures are selected depending on whether the central or peripheral nervous system, or both, are considered to be affected. Extensive data is available on the use of nerve conduction studies to substantiate and serially follow patients with peripheral neuropathy, and the electrophysiologic findings can be used to predict the most likely focus of pathology in the peripheral nervous system. With this information in hand, the electrodiagnostician can guide the clinical neurotoxicologist towards a broad differential diagnosis of the most likely neurotoxins.

Iatrogenic superior gluteal mononeuropathy.

Injuries to the superior gluteal nerve (SGN) have been reported as a result of trauma, pyriformis muscle entrapment, injections, and lumbar lordosis and inadequate back stabilization. We report 3 patients who developed isolated SGN injuries, 1 after a partial nephrectomy and 2 following revision of a total hip arthroplasty. SGN should be suspected in anyone developing an abnormal gait after hip or pelvic surgery or after prolonged lateral decubitus positioning.

Videofluoroscopic evaluation of patients with Guillain-Barré syndrome.

We reviewed 14 patients with clinically confirmed Guillain-Barré syndrome for swallowing dysfunction. All had swallowing dysfunction varying from mild to severe. Six patients (43%) had equivalent impairment during oral and pharyngeal phases. Seven patients (50%) had more severe functional abnormalities during the pharyngeal phase than during the oral phase. One patient (7%) had moderate disorder during the oral phase and mild disorder during the pharyngeal phase. Thirty-six percent of the patients had moderate-to-severe dysfunction during the oral phase, and 71% had moderate-to-severe dysfunction during the pharyngeal phase. In 5 patients who had multiple sequential examinations, moderate or severe swallowing disorders improved to mild-to-moderate disorders within 4-8 weeks after the onset of the symptoms. Residual swallowing disorders may be seen in those who had severe swallowing dysfunction during the later phases of their disease. Further investigations are needed to determine if swallowing abnormalities persist after complete recovery from Guillain-Barré syndrome.

AAEM case report #28: monomelic amyotrophy.

Monomelic amyotrophy is a rare form of motor neuron disease usually presenting as painless asymmetric weakness and atrophy in the distal upper extremities of young adults. Only rarely are the legs involved and pyramidal findings are uncommon. Monomelic amyotrophy is most often observed in people of Japanese and Indian heritage and affects men almost exclusively. Most cases are sporadic. Laboratory testing is frequently normal or nonspecific except for electrophysiologic studies which typically demonstrate reduced compound muscle action potential amplitudes, fasciculations, and features consistent with acute and chronic denervation in distal upper extremity muscles. Necropsy in 1 patient identified anterior horn cell shrinkage, necrosis, and gliosis in appropriate spinal cord segments. Symptoms and signs often progress for several years before spontaneously arresting. The differential diagnosis for monomelic amyotrophy is broad, including processes which affect the cervical cord, roots, brachial plexus, and individual or multiple nerves in the upper extremity.

Demyelinating polyneuropathy in eosinophilia-myalgia syndrome.

Eosinophilia-myalgia syndrome (EMS) is a newly recognized disorder, characterized by myalgia, weakness, scleroderma-like changes, and eosinophilia. EMS is associated with lots of L-tryptophan allegedly contaminated with byproducts of the manufacturing process. We describe 3 patients with EMS who presented with a severe demyelinating sensorimotor polyneuropathy. Electrodiagnostic studies revealed multifocal conduction block, slowing and temporal dispersion of motor responses, and prolonged or absent F-responses. Despite plasmapheresis; corticosteroids; and, in 1 patient, cyclophosphamide, 2 patients died and the remaining patient experienced minimal recovery. Pathology revealed patchy perivascular infiltrates and fibrosis in the connective tissue of muscle and nerve. Autopsy of the central nervous system in 2 patients did not reveal changes unique to EMS. In addition to other organ involvement, EMS may manifest as a potentially fatal polyneuropathy, which initially appears to have prominent demyelinating features.

Clinical and videofluoroscopic evaluation of swallowing in 41 patients with neurologic disease.

Forty-one patients with neurologic disease (ND) were evaluated by clinical and videofluoroscopic examination of the oral cavity and pharynx to assess location and severity of swallowing dysfunction using various bolus consistencies. Four different materials were given to each patient, and included low- and high-viscosity barium suspensions, barium paste, and paste-coated cookie. Thirty-five patients had abnormalities of both oral and pharyngeal function. Four patients had pharyngeal dysfunction only, and two patients were normal. Mild swallowing difficulties occurred in five patients (12%), moderate dysfunction in 29 patients (71%), and severe dysfunction in five patients (12%). Thirty-two patients had pharyngeal stasis, which was symmetric in 30 patients (94%) and asymmetric in two. Site of stasis was not related to the type of neurologic disease. Fifteen patients aspirated, most of them (13 of 15) with the low-viscosity barium suspension. The predominance of aspiration with the low-viscosity liquid emphasizes the importance of clinical and videofluoroscopic evaluation of swallowing in dysphagic patients with ND for appropriate feeding recommendations. Thus, videofluoroscopy complemented the clinical examination and defined the type and severity of swallowing abnormalities and aspiration, when present.

Peripheral neuropathy in cold agglutinin disease.

Cold agglutinin disease (CAD) characteristically presents as anemia and cold-induced rash. We report a man with purpura, anemia, cold agglutinins, and a sensory-autonomic polyneuropathy. Treatment with corticosteroids and plasma exchange resulted in resolution of the anemia and polyneuropathy. We propose that CAD may be associated with a reversible sensory-autonomic neuropathy in the absence of cryoglobulinemia. Although unsupported by pathologic findings, pathogenetic hypotheses for the neuropathy in CAD are similar to those proposed for cryoglobulinemic neuropathy.

Sensitivity for detecting fibrillation potentials: a comparison between concentric and monopolar needle electrodes.

The sensitivity of monopolar and concentric electrodes for detecting fibrillation potentials (FP) has never been formally compared. We studied 35 muscles with FP, sampling 20 sites each with concentric and monopolar needles. The concentric needle identified 0.88 +/- 3.44 (mean +/- standard deviation) more sites with spontaneous activity. Although statistically significant (Wilcoxon signed rank test P less than .03), this difference in sensitivity did not appreciably affect diagnostic interpretation. Subjects described the concentric needles as more painful. Needle insertions in 25 other muscles demonstrated that needle movement generated the majority of FP. We suggest that the increased tissue injury caused by concentric needles may account for both their increased sensitivity and discomfort.

AAEM minimonograph #34: polyneuropathy: classification by nerve conduction studies and electromyography.

Electrodiagnostic evaluation of patients with suspected polyneuropathy is useful for detecting and documenting peripheral abnormalities, identifying the predominant pathophysiology, and determining the prognosis for certain disorders. The electrodiagnostic classification of polyneuropathy is associated with morphologic correlates and is based upon determining involvement of sensory and motor fibers and distinguishing between predominantly axon loss and demyelinating lesions. Accurate electrodiagnostic classification leads to a more focused and expedient identification of the etiology of polyneuropathy in clinical situations.

MRI findings of sciatic endometriosis.

Endometriosis is a rare cause of sciatic mononeuropathy. We report a woman with cyclic, menstruation-related hip pain associated with right leg weakness and sensory loss. Examination and electrodiagnostic studies suggested sciatic nerve dysfunction. Magnetic resonance imaging (MRI) revealed abnormal signal consistent with endometriotic tissue in the region of the right sciatic nerve. The abnormal signal partially regressed after treatment of the endometriosis. This case further illustrates the utility of MRI in the assessment of rare pelvic disorders.