Contribution of the pudendal nerve to sensation of the distal rectum.

Background: Anal and rectal sensory mechanisms and pudendal nerve function are important in the control of faecal continence. The contribution of the pudendal nerve to sensation of the distal rectum was investigated.

Methods: Heat thresholds in the anal canal, distal and mid rectum were measured using a specially designed thermoprobe.

The anal reflex elicited by cough and sniff: validation of a neglected clinical sign.

Background: It is unclear whether contraction of the external anal sphincter (EAS) following a voluntary cough is an integral component of the cough response itself, or a reflex response to the abdominal and pelvic floor dynamics induced by the cough. Clinical experience suggests a reflex origin for this response.

Objective: To compare motor latencies for intercostal, abdominal, and EAS muscle contraction after transcranial magnetic stimulation with those following voluntary coughing and sniffing.

Acral paresthesias in the Andes and neurology at sea level.

Background: Thirty-nine percent of permanent altitude dwellers in the Andes experience acral paresthesias.

Methods: Clinical examinations, sural nerve biopsies, and electrodiagnostic studies on peripheral nerves were performed on 15 men. Ten Cerro de Pasco (CP) natives living at 4,338 meters were biopsied.

The clinical and laboratory features of chronic sensory ataxic neuropathy with anti-disialosyl IgM antibodies.

The clinical and laboratory phenotype of a paraproteinaemic neuropathy syndrome termed chronic sensory ataxic neuropathy with anti-disialosyl IgM antibodies is described in a series of 18 cases. Previous single case reports have outlined some features of this syndrome. All 18 cases were defined by the presence of serum IgM antibodies which react principally with NeuAc (alpha2-8)NeuAc(alpha2-3)Gal-configured disialosyl epitopes common to many gangliosides including GDlb, GD3, GTlb and GQlb.

Induced myelination and demyelination in a conditional mouse model of Charcot-Marie-Tooth disease type 1A.

Charcot-Marie-Tooth disease type 1A, a hereditary demyelinating neuropathy, is usually caused by overexpression of peripheral myelin protein 22 (PMP22) due to a genomic duplication. We have generated a transgenic mouse model in which mouse pmp22 overexpression can be regulated. In this mouse model, overexpression of pmp22 occurs specifically in Schwann cells of the peripheral nerve and is switched off when the mice are fed tetracycline.

Long-term clinical and neurophysiological follow-up of patients with peripheral, neuropathy associated with benign monoclonal gammopathy.

The incidence of hematological malignancy in patients with monoclonal gammopathy of undetermined significance (MGUS) has been assessed as 17% to 25%. To ascertain whether this is true of neuropathy associated with MGUS, a long-term (5-42 years) retrospective clinical and neurophysiological follow-up was conducted in 50 cases (immunoglobulin M [IgM], n = 38; IgG, n = 11; IgA, n = 1). Only three patients developed hematological malignancy.

Medial (cutaneous) branch of deep common peroneal nerve: recording technique and a case report.

An isolated lesion of the medial branch of the deep common peroneal nerve is rare and so far there do not appear to have been any reports of a recording method or normative data for this sensory action potential (SAP). Just such a case is described along with an appropriate recording technique. Control data were subsequently obtained from 50 normal volunteers.

Posterior paroxysmal discharge: an aid to early diagnosis in Lafora disease.

Lafora body disease is a rare neurometabolic disorder of autosomal recessive inheritance. Symptoms begin in the second decade with progressive myoclonic epilepsy and survival is unusual beyond the age of 30. We report an electroencephalographic study in four cases of histologically proven Lafora body disease.

Neonatal diaphragmatic paralysis caused by chest drains.

A boy delivered at 32 weeks' gestation developed bilateral pneumothoraces that required multiple chest drains. He was dependent on the ventilator for 52 days because of bilateral diaphragmatic paralysis. Electrophysiological studies confirmed phrenic nerve damage.

Sensory conduction in medial and lateral plantar nerves.

A simple and reliable method of recording medial and lateral plantar nerve sensory action potentials is described. Potentials are recorded with surface electrodes at the ankle using surface electrodes stimulating orthodromically at the sole. The normal values obtained are higher in amplitude than those obtained by the method described by Guiloff and Sherratt and are detectable in older subjects aged over 80 years.

Neurological complications following liver transplantation.

17 (33%) of 52 patients who underwent 56 consecutive orthotopic liver transplants had serious neurological complications postoperatively. The commonest complication was fits, which occurred in 13 (25%) patients. 50% of patients had their onset of fits within the first week.