The spectrum of chronic inflammatory demyelinating polyneuropathy.

Research criteria for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) were proposed by an Ad Hoc Subcommittee of the American Academy of Neurology (AAN) in 1991, and since then these criteria have been widely used in clinical studies. We have been impressed by the frequent finding of electrophysiological changes of a demyelinating neuropathy in patients whose clinical presentation does not conform to the usually accepted clinical phenotype of CIDP. To determine the clinical spectrum of CIDP, we conducted a retrospective review of patients of the peripheral electrophysiology laboratory of the University of Miami-Jackson Memorial Medical Center. Diagnostic criteria for acquired demyelination of an individual nerve were adapted from the AAN research criteria for the diagnosis of CIDP (1991). Patients were accepted for inclusion when such evidence was demonstrated in at least one motor nerve or at least two sensory nerves. We then reviewed the clinical phenotype and the underlying etiology of the neuropathy in these cases. Eighty-seven patients, 63 male and 24 female, age of onset 4-84 (mean 49.3) years, met these inclusion criteria. Forty-seven patients (54%) had distinct features outside the usual clinical presentation of CIDP. Of these, 15 (17%) had predominantly distal features, 13 (15%) had exclusively sensory polyneuropathy; seven (8%) had markedly asymmetric disease, seven (8%) had associated CNS demyelination, four (5%) had predominant cranial nerve involvement, and one (1%) had only the restless legs syndrome. An associated medical condition that may have been responsible for the acquired demyelinating neuropathy was present in 60% of the patients. We conclude that spectrum of CIDP is broader than would be indicated by the strict application of the AAN research criteria, and that many of the cases meeting more liberal criteria frequently respond to immunosuppressive therapy.

Sympathetic skin response and heart rate variability in patients with Huntington disease.

OBJECTIVE: To examine the autonomic nervous system functions in patients with Huntington disease. BACKGROUND: Although patients with Huntington disease frequently experience vegetative symptoms, it is not clear if there is dysfunction of the autonomic nervous system. METHODS: Sympathetic skin response (SSR) latency and amplitude from both palms and soles and R-R (heart rate) interval variation (RRIV) at rest and during the Valsalva maneuver were examined in 22 patients and 21 age-matched controls. Unified Huntington's Disease Rating Scale scores were determined in all the patients. RESULTS: Our data are reported as means +/- SEMs. The SSR latencies in patients (mean palm latency, 1835.8+/-110.7 milliseconds; mean sole latency, 2625.3+/-226.9 milliseconds) were prolonged compared with controls (mean palm latency, 1359.5+/-28 milliseconds [P<.01]); mean sole latency, 2038.1+/-44.9 milliseconds [P<.01]) and amplitudes in patients (mean amplitude, 1063.1+/-237.7 microV) were smaller compared with controls (mean amplitude, 1846.3+/-251.2 microV [P<.05]). The RRIV in patients both at rest (mean RRIV in patients, 3.7%+/-0.4% vs. controls, 9.7%+/-0.6% [P<.01]) and during the Valsalva maneuver (mean RRIV in patients, 6.3%+/-1.6% vs. controls, 14.5%+/-1.2% [P<.01]) was lower compared with controls. Furthermore, the prolonged SSR latencies, smaller amplitudes, and lower RRIV in patients compared with controls closely correlated with the various components of the Unified Huntington's Disease Rating Scale scores (total behavior score and SSR latency, R = 0.6 [P<.01]; total behavior score and SSR amplitude, R = -0.5 [P<.05]; total behavior score and RRIV, R = -0.4 [P<.05]; verbal fluency and SSR latency, R = -0.5 [P<.05]; verbal fluency and SSR amplitude, R = 0.5 [P<.05], verbal fluency and RRIV, R = 0.5 [P<.05]; total functional capacity and SSR latency, R = -0.6 [P<.01]; total functional capacity and SSR amplitude, R = 0.5 [P<.05]). CONCLUSION: These results suggest that there is autonomic nervous system dysfunction in patients with Huntington disease.

Relapses in the Guillain-Barré syndrome after treatment with intravenous immune globulin or plasma exchange.

To clarify the question of whether Guillain-Barré syndrome (GBS) patients treated with intravenous immune globulin (i.v.IG) relapse at a higher frequency than those treated with plasma exchange (PE), 54 patients with GBS were studied retrospectively. A higher frequency of relapses was noted in the PE-treated patients than in those receiving i.v.IG. The presence of an associated medical condition correlated with an increased risk of relapses, while earlier onset of treatment resulted in a decrease of relapses of GBS. This study found no support for prior suggestions of increased relapses in patients with GBS treated with i.v.IG as opposed to those treated with PE.

Lateral sinus thrombosis and cervical abscess complicating cholesteatoma in children: case report and review.

To call attention to complications of chronic otitis media, the case of a patient who simultaneously developed lateral sinus thrombosis and Bezold's abscess is reported. A 7 year old boy presented with fever, drowsiness, cervical mass and otorrhea not responding to medical management. Work-up revealed lateral sinus thrombosis and cervical abscess secondary to right ear cholesteatoma. After treatment with surgery and antibiotics, he had a favorable outcome. We review the literature regarding the diagnosis and management of these complications and concluded that although less frequent they remain a clinical challenge.

Marked improvement of severe polyneuropathy associated with multifocal osteosclerotic myeloma following surgery, radiation, and chemotherapy.

We describe a patient with a 3 year history of progressive polyneuropathy that rendered him severely quadriparetic and bedridden. Work up revealed an IgG lambda monoclonal spike and multifocal osteosclerotic myeloma. Remarkable improvement followed combined treatment with surgical excision, radiation therapy, and chemotherapy using chlorambucil, danazol, and hydrocortisone. Hence, we believe that aggressive local therapy associated with systemic chemotherapy should be considered in severely affected patients with multifocal osteosclerotic myeloma and peripheral neuropathy.

Skull base metastases causing acute bilateral hypoglossal nerve palsy.

Acute bilateral tongue palsy is a rare clinical presentation. We report one such case caused by skull base metastases from prostate cancer. There were no other cranial nerves involved or associated neurological deficit. Mild improvement was seen following radiation therapy. We review the anatomy of the nerve and differential diagnosis of its disfunction.

Undernutrition during suckling and latent learning ability of rehabilitated adult male rats.

The present report investigates the effects of early undernutrition on the latent learning ability of rehabilitated adult male rats in a simple maze task. Rats were undernourished during suckling by feeding their dams an 8% casein diet. Well-nourished dams received a 25% casein diet during the same period. Rats were weaned at 21 days of age and nutritionally rehabilitated until they became adults, when behavioral task was conducted. Under a nonappetitive condition, rats were exposed either to an open field or to a maze apparatus. They were thereafter deprived of water and tested in the maze apparatus. Both well-nourished and undernourished rats that had been previously exposed to the maze performed better than those exposed to the open field. Nutritional treatment had no effect on performance of either the latent learning or of the open-field groups. These results suggest that rehabilitated adult rats are able to learn about the environment when no immediate reinforcement is involved. The discrepancy between our findings and results reported by others may be due to differences in task complexity and/or perhaps to the fact that nutritional rehabilitation also plays a role in reversing some of the deleterious effects of early undernutrition on learning ability of rats.

Early undernutrition blocks the effect of naltrexone on rat exploratory behavior.

1. Young rats were undernourished by feeding their dams an 8% protein diet (w/w) from birth until weaning (21 days); the dams of control rats were fed a 20% protein diet (w/w) until weaning. All offspring were fed a 20% protein diet from 21 to 120-150 days of age, when they were tested. 2. The involvement of endogenous opioids in the exploratory activity (rearing response and crossing frequency in a 2-compartment open-field test) was evaluated by examining the effects of a single injection of naltrexone (0.75 mg/kg, ip) 6 min prior to testing. 3. Naltrexone decreased exploratory activity (rearing) by approximately 50% in normal rats but had no effect on undernourished rats. 4. These data provide additional evidence for an alteration in the opioid system of undernourished rats.