Mercury poisoning in a dentist

We examined a dentist with chronic elemental mercury poisoning electrophysiologically. Motor conduction in the upper and lower limbs was normal. Sensory nerve action potentials in the ulnar and median nerves were normal, but could not be elicited in the superfical peroneal nerves. Conduction velocity of the sural nerves was normal, but the action potential amplitude was abnormal. Following treatment with penicillamine, sensory conductions in the lower limbs returned to normal.     

Symmetry of normal motor and sensory nerve conduction measurements

Nerve conduction measurements in normal subjects are assumed to be symmetric, but the normal limits of symmetry have not been determined. Full data on the limits of symmetry for commonly studied nerves are important in the clinical interpretation of nerve conduction data. We selected normal electrodiagnostic studies from archived electromyographic laboratory reports that included bilateral measurements of motor and sensory nerves. Symmetry of nerve conduction measures was confirmed, and only the median and ulnar sensory nerves had significant deviations from symmetry, supporting subclinical nerve damage in the most common dominant hand. The limits of symmetry were determined by calculating the 95th percentile for the differences between sides. For motor and sensory nerves, the range of 95th percentile limits was narrower for measures in upper extremity nerves compared to lower extremity nerves. Several reasons are offered for the wider limits of symmetry in lower extremity nerves.     

Multifocal motor neuropathies with conduction blocks. 39 cases

Clinical, biological and electrophysiological features from a cohort of 39 multifocal motor neuropathies with conduction blocks (NMM with CB) have been studied. There were 29 males and 10 females with an average of 47.3. At the first evaluation, the mean duration of the symptoms was of 8 years with extremes between 1 and 28. Pain and paresthesias were present in respectively 10 and 18 p. 100 of the patients. Fasciculations and cramps were observed in more than 2/3 of the cases. Three patients had tremor at rest. Upper limb muscular weakness was the predominant initial symptom (84.6 p. 100). The weakness always affected distal and unilateral muscles. Radial and cubital nerve distribution are mainly affected and in half of the cases an unilateral motor deficit in the lower limb was associated. Muscle atrophy was frequent (74 p. 100) and rapidly developed in the first 2 years. Reflexes were decreased or absent in 64 p. 100. In 78 p. 100 of cases, biological study showed normal serum immunoelectrophoresis and CSF. IgM anti-GM1 antibodies were found in 24/36 patients. Very high titres were found in 5 cases. All patients had CB in upper limbs. The preferential localizations of the CB were equally at the median and ulnar nerves. Only 7 patients had CB localized to the lower limbs. In many cases, marked reduction of the motor amplitude prevented the detection of CB, marked reduction of the motor amplitude prevented the detection of CB. Moderate fibrillation potentials were found in 28 p. 100 of patients. Giant muscular unit potentials were frequent (21/39). F-waves in nerve with CB were always abnormal with marked increased latencies. Late responses sometimes seemed to be repeater F-waves. Axon reflexes were detected in 5 cases. The late responses abnormalities could precede the block. Clinical, biological and electrophysiological described arguments could may distinguish NMM with CB from motor neuron disease and relate them to the group of chronic demyelinating neuropathies.     

Body mass index effect on common nerve conduction study measurements

This study was performed to determine whether there is a difference in nerve conduction study (NCS) measures based on body fat (body mass index; BMI). Two hundred fifty-three subjects had the following NCS tests performed on them: median, ulnar, peroneal, and tibial motor studies; median, ulnar, radial, and sural sensory studies; median and ulnar mixed nerve studies; and H-reflex studies. BMI was calculated as weight (kg) divided by height (m) squared. A repeated measures analysis of variance was run adjusting for age, sex, and height and using BMI as both a continuous variable and by dividing BMI into upper, middle, and lower thirds. The sensory and mixed nerve amplitudes correlated significantly (P < or = 0.01) with BMI for all nerves tested, with means being approximately 20-40% lower in the obese than in the thin subjects. No correlation was noted between BMI and nerve conduction velocity, H-reflex latency, or most of the other motor/sensory/mixed measures. The correlation between increased BMI and lower sensory/mixed nerve amplitudes should be taken into account in clinical practice.     

Median and ulnar nerve conduction studies among workers: normative values

To determine normative values for nerve conduction studies among workers, we selected a subset of 326 workers from 955 subjects who participated in medical surveys in the workplace. The reference cohort was composed exclusively of active workers, in contrast to the typical convenience samples. Nerve conduction measures included bilateral median and ulnar sensory amplitude and latency (onset and peak). Workers with upper extremity symptoms, medical conditions that could adversely affect peripheral nerve function, low hand temperature, or highly repetitive jobs were excluded from the "normal" cohort. Linear regression models explained between 21% and 51% of the variance in nerve function, with covariates of age, sex, hand temperature, and anthropometric factors. The most robust models were fitted for sensory amplitudes in the median and ulnar nerves for dominant and nondominant hands. The median-ulnar difference was least sensitive to adjustment, indicating it is the best measure to use if corrections are not made to account for relevant covariates. A key point was that the magnitude of variance increased with age and anthropometric factors. These findings provide strong evidence that to improve diagnostic accuracy, electrodiagnostic testing should control for relevant covariates, particularly age, sex, hand temperature, and anthropometric factors.     

The sword of Damocles: the psychosocial impact of familial spinocerebellar ataxia in South Africa

It has been recently recognized that increased titers of serum anti-GM1 antibodies may be associated with motoneurone diseases or with multiple motor neuropathy with or without conduction block and also with chronic sensorimotor neuropathy and Guillain-Barré syndrome. Santoro et al. were the first to note that anti-GM1 antibodies were able to bind to the nodes of Ranvier of the sural nerve of a patient with clinical signs and symptoms mostly resembling amyotrophic lateral sclerosis who also showed, in nerve conduction studies, multifocal motor nerve fibers conduction block and serum IGM anti-GM1 antibodies. The two patients presented in this report had asymetrical motor neurone disease with signs and symptoms of lower motoneurone involvement, and other signs, in the first patient, which suggested the existence of upper motoneurone damage. Besides, the second patient also had clinical sensory impairment in the lower limbs. Electrophysiologically, none of them had nerve conduction block but both showed inexcitable median and sural nerve sensory fibers. Both had high titers of anti-GM1. A sural biopsy of both patients showed immunoglobulins into the sensory fibers. However, we do not know whether the anti-GM1 antibodies bind to a cross-reactive glycolipid other than the GM1 itself. In any case, it seems that the presence of anti-GM1 antibodies might be a marker signalling a potentially treatable immune disorder which may have signs of lower and upper motor neurone disease and, also, clinical and electrophysiological evidences of peripheral sensory involvement.     

Nortropane-3beta-acetic acid, tropaquinuclidine and dehydrotropaquinuclidine (author''s transl)]

The greater decrease of conduction velocity in sensory than in motor fibres of the peroneal, median and ulnar nerves (particularly in the digital segments) found in patients with chronic carbon disulphide poisoning, permitted the diagnosis of polyneuropathy to be made in the subclinical stage, even while the conduction in motor fibres was still within normal limits. A process of axonal degeneration is presumed to underlie occurrence of neuropathy consequent to carbon disulphide poisoning.     

Segmental motor conduction time for abductor of the fifth finger muscle: methods and normative values

Electrical stimulation of the ulnar nerves (60 nerves) and magnetic stimulation of the roots (C7) and motor cortex were performed on 30 normal controls. The muscle responses and F wave (peripheral stimulation) were recorded from abductor digiti minimi muscle (60 muscles). The parameters of examined potentials were measured and the central, root, peripheral motor conduction times were estimated. The normative values were established as well as formulae of linear regression within the observed correlations with height. The method may be used for electrophysiological diagnosis of patients with motor pathway impairment at the different levels.     

Pulmonary veno-occlusive disease complicating Felty''s syndrome

Correction factors exist to allow for the dramatic effect that temperature has on nerve conduction study parameters. However, these are based on normal nerves in normal individuals and may not be appropriate in the diseased nerve setting. Our clinical study showed that in carpal tunnel syndrome, the median nerve reacts differently to temperature changes compared with normal ulnar controls. Furthermore, statistically significant differences exist between the rates of change with increasing temperature in motor and sensory nerves.     

Subacute multifocal painful neuropathy with spontaneous remission

We present the cases of two patients with subacute onset of multifocal painful neuropathy with spontaneous remission and no relapse. The distribution of pain in patient 1 was hands (median > ulnar nerve region) and feet (peroneal and terminal tibial nerve regions), and in patient 2, hands (ulnar nerve region) and feet, left worse than in right. Both patients experienced facial numbness. Deep tendon reflexes were intact except for absent ankle jerks in patient 2. Motor nerve conduction studies demonstrated a marked prolongation of the distal motor latencies with normal proximal segment conduction velocities, suggesting distal demyelination. Cerebrospinal fluid protein concentration was elevated in patient 2, but no definite abnormality was found on sural nerve biopsy. A demyelinating neuropathy with a monophasic self-limited course may be consistent with Guillain-Barre syndrome (GBS). However, the multifocal painful sensory symptoms with facial numbness and the marked distal nerve conduction slowing in our cases are not consistent with GBS.     

A case of hereditary motor and sensory neuropathy type I with a new type of peripheral myelin protein (PMP)-22 mutation

A 16-year-old school boy suffered from an insidious foot deformity. Slight degrees of symmetrical muscular weakness of the distal lower limb muscles were observed. In addition, slight degrees of atrophy of the anterior tibial muscles with moderate degrees of pes cavus deformity and flexion contracture of the toes of both feet were observed. In the upper and lower limbs muscle stretch reflexes were decreased and absent, respectively. Vibratory and touch sensations were moderately and slightly decreased, respectively, in the toes. The median and ulnar motor conduction velocities (m/sec) were 21.1 and 13.2, respectively, with markedly prolonged distal latencies. The median and ulnar sensory conduction velocities (m/sec) were 21.5 and 10.1, respectively. No M-waves were recorded by stimulation of the tibial and peroneal nerves. Also no nerve action potential was elicited by stimulation of the sural nerve. A fascicular biopsy of the sural nerve was performed. The myelinated fibers showing segmental de- and re-myelination were frequently found in teased fiber preparations. The density of myelinated fibers was markedly decreased, and both demyelinated axons and onion-bulbs were also observed by light and electron microscopy in the Epon-embedded sections. Based on the neurological examination and nerve conduction studies, although other family members were not examined, a diagnosis of HMSN type I was made. To clarify the genetic abnormality, a systematic study of the genomic DNA was made. A DNA duplication in the chromosome 17p11.2-12 was not observed. The single-strand conformational polymorphism method showed an abnormal extra band in the exon 3 encoding peripheral myelin protein (PMP)-22 gene of the patient compared with the control. The direct sequencing analysis of the exon 3 revealed a guanine to cytosine substitution that caused a substitution of arginine for glycine at amino acid position 93 of PMP-22. The digestion of the exon 3 with Sty I showed the presence of a mutant and normal allele of the PMP-22 gene indicating autosomal dominant heredity. This type of PMP-22 gene mutation is different from any type of PMP-22 mutations reported in the literature. The mutation is located in the intracellular domain of PMP-22. The mechanism by which the mutation induce demyelination of the peripheral myelin remains to be elucidated. Reports of patients with a point mutation of amino acids of PMP-22 are rare in the literature. This is the first Japanese patient with a new type of mutation of the PMP-22 gene.     

Scheuermann's kyphoscoliosis associated with Charcot-Marie-Tooth syndrome

Much confusion and disagreement exists regarding the classification and characteristics of inherited disorders manifesting neurogenic muscular atrophy. Many authors consider Charcot-Marie-Tooth syndrome (CMTS) and Roussy Levy syndrome (RLS) forme fruste or variants of Friedreich's ataxia (FA). Familial kyphoscoliosis has often been described in FA and RLS but not with CMTS. The purpose of this paper is to present detailed clinical and laboratory findings in a family with three cases of Scheuermann's kyphoscoliosis and CMTS in three generations. In all cases Scheuermann's kyphoscoliosis was associated with pes cavus, markedly diminished vibratory and position sensation in the lower extremities, absent deep tendon reflexes and muscular atrophy, predominantly of the distal muscles. Fine rhythmic tremor of outstretched hands and positive Romberg sign were present in one case only. Serum creating phosphokinase was elevated in two cases. Motor nerve conduction studies revealed impaired function in the median, ulnar, tibial and peroneal nerves. Sensory nerve conduction wal also impaired in median and ulnar nerves. There was evidence of left ventricular hypertrophy in one case only. The nosology and relationship between CMTS, RLS and FA are discussed.     

Evaluation and treatment of posterior cruciate ligament injuries

Twenty-eight low median nerve injuries and 23 low ulnar nerve injuries were repaired using intraneural fascicular dissection and electrical fascicular orientation. Eleven freshly lacerated nerves were seen within 48 hours after injury; 40 nerve lacerations were chronic. Fascicular orientation between sensory and motor fascicles at the proximal nerve end could be accurately differentiated in 47 nerves (92%) independent of whether it was acute or chronic. At the distal nerve end in fresh lacerations, the motor fascicles could be determined conclusively by muscle contraction with sequential electrical stimulation of the fascicles. In chronic nerve lacerations, the distal fascicles could be estimated anatomically after internal neurolysis. After fascicular orientation, nerves were repaired with end-to-end group fascicular suture or interfascicular sural nerve grafting. Twenty-four nerves repaired with end-to-end suture and 13 nerves repaired with nerve grafting were monitored more than 25 months. Satisfactory sensory results (i.e., S3+ or S4 functions) were obtained in 29 nerves (78%) and M4 or M5 motor functions were achieved in 29 nerves (78%). There were no patients who needed additional tendon transfers to reconstruct thumb opposition or to correct claw finger deformity. These results suggest that low median or ulnar nerve lacerations, whether acute or chronic, partial or complete, may be successfully repaired with the aid of electrical fascicular orientation with or without intraneural fascicular dissection.     

Peripheral nerve conduction following recovery from Guillain-Barre syndrome

The material comprises 10 cases with a history of Guillain-Barré syndrome within the last several years in which clinical investigations failed to demonstrate any abnormalities. Electrophysiological investigations of peripheral nerves including conduction velocity in motor fibres--maximal and minimal, sensory fibres, standardized terminal latency--showed presence of changes evidencing slight but persisting subclinical lesion to peripheral nerves, especially in subjects affected in early childhood. Slowing of conduction involved motor as well as sensory fibres in these nerves and was more pronounced in the ulnar nerve than in the remaining nerves (peroneal, axillary, musculocutaneous and facial).     

Lower extremity sensory nerve conduction studies

The need for routine recording of sensory potentials in the lower extremity was developed in efforts to better investigate peripheral neuropathy. Sensory nerve conduction studies (NCS) are more sensitive to many abnormalities in the peripheral nervous system than the motor NCS responses. This article provides a thorough review of sensory nerve conduction studies of the lower extremity, with special emphasis on electrodiagnostic issues.     

A comparison of traditional electrodiagnostic studies, electroneurometry, and vibrometry in the diagnosis of carpal tunnel syndrome

In 49 patients (98 hands), referred to an electrodiagnostic laboratory, assessments were made by conventional nerve conduction studies on the upper extremity and by two more portable modalities, namely electroneurometry (skin surface electrical stimulation of the motor nerve) and single-frequency (120 Hz) vibrometry. Tests were performed on median and ulnar nerves. Correlations with motor nerve conduction studies for each screening test on the median nerve were r = .81 for the electroneurometer and r = .48 for the vibrometer. When carpal tunnel syndrome was diagnosed either by clinical criteria only or by nerve conduction abnormality, the association with electroneurometry was characterized by high sensitivity and low specificity, while the opposite relationship prevailed with vibrometry. These associations were highly dependent on the methods used to select normal values from a reference population. While the manufacturer's recommended normal values offered good predictability, with thresholds that corresponded to nerve conduction studies, normal values generated in a more standard way produced much weaker and less useful associations. The selection of an appropriate electrical screening test for peripheral nerve injury, such as entrapment neuropathy, depends on the prevalence and seriousness of the target disease and the relative consequences of over- and underdiagnosis.     

Long-term hyperglycemia is related to peripheral nerve changes at a diabetes duration of 4 years. The Wisconsin Diabetes Registry

OBJECTIVE: To examine longitudinal hyperglycemia and peripheral nerve responses in a population-based incident cohort. RESEARCH DESIGN AND METHODS: A sample from an incident cohort of young people was comprehensively followed from diagnosis of IDDM. Participants were invited to submit blood samples three times per year for central testing of GHb. During their 4th year of diabetes, nerve conduction studies were performed on the median sensory and motor, peroneal motor, and sural sensory nerves. Relationships between mean GHb and nerve latencies, velocities, and amplitudes were explored. RESULTS: GHb was positively related to all nerve latencies and negatively related to all nerve velocities. The relationships between mean GHb and nerve conduction latencies and velocities differed by sex for the peroneal nerve latency (beta = 0.17 male subjects, beta = -0.01 female subjects; P < 0.001). Pubertal participants had lower velocities and longer latencies than prepubertal participants (beta = 0.37; P = 0.05 peroneal latency), after adjustment for GHb, height, and extremity temperature. Sensory and motor nerve amplitudes were related to GHb, and these relationships did not differ by sex. CONCLUSIONS: Our study indicates that sustained hyperglycemia is related to functional changes, at the minimum, in peripheral sensory and motor nerve conduction at a diabetes duration of 4 years. Our findings are consistent with a dying-back neuropathy, and there is some suggestion that chronic hyperglycemia may be more detrimental to nerves in male subjects than in female subjects.     

Histomorphometric study in children with idiopathic nephrotic syndrome

There is agreement on the clinical diagnostic criteria for acute inflammatory demyelinating polyneuropathy (AIDP/GBS) however, there is lack of consensus for detection of demyelination. In order to critically evaluate the prevailing criteria, sixty-six patients who fulfilled NINCDS criteria and had typical features of GBS were studied for electrophysiological abnormalities of peripheral nerves by using standard methods (median, common peroneal, sural and ulnar) between 1 to 12 weeks after the onset of symptoms. The commonest abnormality on motor nerve conduction study was prolonged distal latency (75%-83%) followed by reduction in CMAP amplitude (63%-82%), decreased velocity (48%-62%), conduction block (17%-39%) and f-wave abnormalities (37.8%-59%). Sensory conduction abnormalities were detected in over 20% of median, 25% of ulnar and 33% of sural nerves. All the patients had abnormality of at least two motor conduction parameters in one nerve when values beyond 2 SD of the mean were considered abnormal and over 70% of patients had three abnormalities in two nerves or two abnormalities in three nerves. Comparison with the prevailing criteria for demyelination revealed that the number of patients fulfilling them varied widely: Albers et al. (1985): 74.2%, Albers et al. (1989): 40.9% and Cornblath: 30.3%. We believe that the current criteria for detection of demyelination in acute neuropathy are too strict, underestimate the underlying pathology in GBS and need reassessment.     

A family with neurogenic atrophy of the distal muscles of the upper limbs: clinical and electrophysiological studies

A Chinese family manifested mild neurogenic atrophy of the distal muscles of the upper limbs. None of the affected members had sensory abnormalities, or pyramidal tract or bulbar involvement. The onset of the illness was in the middle of the second decade of life. The muscle atrophy was more severe in the female members. Electromyographic examination of the atrophic muscles showed evidence denervation. One female patient demonstrated slow motor conduction velocity in the right median nerve.     

A case of X-linked recessive bulbospinal muscular atrophy with demyelinating neuropathy and hypertrophy of the calves

We report a 53-year-old man of X-BSMA with neuropathy. The patient developed slowly progressive muscular weakness and wasting over a 2-year period with an accompanying numbness in the finger tip. He can run normally but not so fast. When he was aged 52-year old, difficulty in running progressed. General physical examination revealed nothing particular except for hypertension and gynecomastia. He showed muscular weakness and atrophy in the tongue, shoulder girdle, and upper and lower limbs. Calf muscle hypertrophies were prominent on both sides. The tendon reflexes were absent. Slight sensory impairment for vibration and pin-prick was present distally in all limbs. Autonomic nerve dysfunction was not observed. Hyperglycemia, elevated HbA1c and elevated serum CK (1,242 IU/l) were seen. The computed tomographic analyses on skeletal muscle showed hypertrophic changes in the calf muscles with a few fatty infiltrations. Electromyography showed a systemic neurogenic pattern. Motor nerve conduction velocities were slightly delayed in the lower limits. Sensory nerve action potentials were not elicited in all nerves tested. Sural nerve biopsy disclosed marked reduction of myelinated fibres for that of large diameter with thin myelin. Teased fibre studies showed a definite increase in the incidence of fibres with segmental demyelination and remyelination. In electron microscopic examination, typical or atypical onion bulb formation was observed on individual fibres. Axonal changes were minimum. We believe that segmental demyelination observed in this patient is not secondary to axonal damage. We, also, investigated AR gene abnormality by polymerase chain reaction (PCR) in this patient.(ABSTRACT TRUNCATED AT 250 WORDS)