[Interpersonal behavioral style as predictor of treatment outcome]. / Interpersoonlijke gedragsstijl als voorspeller van behandelresultaat.

BACKGROUND: Interpersonal behavioral style (IPBS) refers to the way in which someone behaves in social contact. IPBS affects the type and quality of different social interactions including the therapeutic relationship. The therapeutic relationship correlates with treatment outcome, IPBS possibly too. The direction of that relationship remains unclear. AIM: To examine the predictive value of IPBS on treatment outcome in group treatment. METHOD: 149 patients were divided in different types of IPBS: degree of affiliation (kindness vs. hostility), degree of dominance (dominant vs. submissive), quadrants (combination of affiliation and dominance) and vector length (strength of IPBS). Treatment outcome was measured in experienced psychopathology, social anxiety and frequency of social contact. Groups were compared by using one-way analysis of variance (ANOVA). Significant differences were analyzed using Tukey's post-hoc analysis. RESULTS: Vector length predicted all treatment outcome measures. The degree of dominance predicted only scores on social anxiety and frequency of social contact. The degree of affiliation did not predict any treatment results. CONCLUSION: Findings suggest that adult patients with stronger IPBS and a more submissive, sub-assertive IPBS experienced lower social anxiety scores and increased frequency of social contact after treatment. A stronger IPBS also predicts lower experienced psychopathology post-treatment. The degree of kindness/hostility does not affect treatment outcome.

Encephalopathic attacks in a family co-segregating myotonic dystrophy type 1, an intermediate Charcot-Marie-Tooth neuropathy and early hearing loss.

OBJECTIVE: To report new disease components in a unique myotonic dystrophy type 1 (DM1) family previously described by us in which all affected members also had a sensorimotor neuropathy that co-segregated with markers flanking the DM1 locus. METHODS: Clinical observations, electrophysiology, audiometry, DNA studies. RESULTS: During a follow-up period of over 25 years, the following were observed: (i) co-segregation of a striking new encephalopathic phenotype. In middle age, five patients were admitted on multiple occasions with attacks of impaired consciousness, psychomotor agitation, fever and, in about half of the cases, focal neurological signs, including unilateral weakness, sensory deficits and dysphasia. Reported onset phenomena consisted of confusion, headache, focal neurological symptoms and nausea; (ii) many patients show an early and severe sensorineural hearing loss; (iii) although they have mothers with the adult onset type, the four affected subjects from the youngest generation do not show any signs or symptoms of childhood or congenital myotonic dystrophy; (iv) the neuropathy meets the criteria of an intermediate type Charcot-Marie-Tooth (CMT), and is more severe in males; and (v) patients presented with an expanded fragment at the DM1 CTG repeat but this allele was refractory to PCR amplification and triplet repeat primed PCR at the 3' end of the array, indicating the existence of an additional lesion at the 3' end. CONCLUSIONS: The phenotype in this unique family extends beyond myotonic dystrophy and CMT to include encephalopathic attacks and early hearing loss, and is associated with an atypical mutation at the DM1 locus.

[Carpal tunnel syndrome: diagnosis, treatment, prevention and its relevance to dentistry]. / Diagnostiek en behandeling van het carpaletunnelsyndroom.

Nocturnal pins and needles and other sensory disturbances in the median nerve innervated fingers are caused by local pressure on this nerve in the carpal tunnel. Carpal tunnel syndrome is the most frequently encountered peripheral nerve entrapment. In The Netherlands, the prevalence of carpal tunnel syndrome is estimated 9% among adult women and 0.6% among adult men. Several risk factors have been identified. For dental professionals, the most relevant seem forceful use of the hand during scaling and extractions, use of vibrating ultrasonic equipment and frequent working with the wrist in flexion or in extension. The diagnosis of carpal tunnel syndrome is based on the characteristic complaints, confirmed preferably by abnormal electrophysiological tests. Depending on the degree of impact on daily functioning, treatment for carpal tunnel syndrome may be expectative, conservative or surgical. Adjustment of the working conditions may prevent the development of a carpal tunnel syndrome.

[Guideline 'Diagnosis and treatment of carpal tunnel syndrome']. / Richtlijn 'Diagnostiek en behandeling van het carpale-tunnelsyndroom'.

--Carpal tunnel syndrome (CTS) is the most frequently encountered peripheral nerve entrapment: about 10% of adult women and less than 1% of adult men in the Netherlands have a clinically and electrophysiologically confirmed CTS. --All medical and paramedical disciplines involved in the diagnosis and treatment of CTS in the Netherlands contributed to the development of a guideline for the diagnosis and treatment ofCTS. --Clinical diagnosis of CTS is based on a history of nocturnal pins and needles, numbeness and/or pain in the median nerve innervated area of the fingers and hand, which often causes the patient to awake. --Provocative tests do not contribute to the clinical diagnosis of CTS. --If invasive therapy is considered, such as corticosteroid injection or surgery, the clinical diagnosis must be confirmed by abnormal findings in electrophysiological tests. --Ultrasound or MRI of the wrist may be of diagnostic value when structural abnormalities in the carpal tunnel are suspected. Given the special expertise needed for ultrasound testing and the limited availability of MRI for CTS diagnostic purposes, these methods are not the first preference. --Depending on the degree of impact on daily functioning, treatment for CTS may be expectative, conservative (wrist splint or local steroid injections) or surgical (endoscopic or open techniques). --If CTS does not restrict daily functioning, adjustment of the working conditions will do. --Furthermore measures aimed at CTS prevention and treatment of an already existing work-related CTS are discussed.

Interobserver variation in the interpretation of SSEPs in anoxic-ischaemic coma.

OBJECTIVE: To study interobserver variation in the interpretation of median nerve SSEPs in patients with anoxic-ischaemic coma. METHODS: SSEPs of 56 consecutive patients with anoxic-ischaemic coma were interpreted independently by 5 experienced clinical neurophysiologists using guidelines derived from a pilot study. Interobserver agreement was expressed as kappa coefficients. RESULTS: Kappa ranged from 0.20 to 0.65 (mean 0.52, SD 0.14). Disagreement was related with noise level and failure to adhere strictly to the guidelines in 15 cases. The presence or absence of N13 and cortical peaks caused disagreement in 5 cases each. For recordings with a noise level of 0.25 microV or more, mean kappa was 0.34; for recordings with a noise level below 0.25 microV mean kappa was 0.74. CONCLUSIONS: Interobserver agreement for SSEPs in anoxic-ischaemic coma was only moderate. Since the noise level strongly influenced interobserver variation, utmost attention should be given to its reduction. If an artefact level over 0.25 microV remains, absence of N20 cannot be judged with sufficient certainty and the SSEP should be repeated at a later stage. SIGNIFICANCE: Because of its moderate interobserver agreement, great care has to be given to accurate recording and interpretation of SSEPs before using the recordings for non-treatment decisions.

Genetic linkage with chromosome 19 but not chromosome 17 in a family with myotonic dystrophy associated with hereditary motor and sensory neuropathy.

We identified a large kindred that shows classical myotonic dystrophy (MyD), together with hereditary motor and sensory neuropathy (HMSN) in some individuals, and HMSN alone in others. A previous study of this family has shown cosegregation of the MyD and HMSN phenotypes with the Lutheran and secretor loci in some branches of the family, indicating linkage to chromosome 19. We reanalyzed this family with 2 recombinant DNA marker systems from the ApoC2 locus on chromosome 19. Our results demonstrate that all affected individuals have inherited a unique ApoC2 haplotype that was not found in their clinically and electrophysiologically normal sibs. We also obtained evidence against involvement of the HMSN I locus on chromosome 17. In this family, a moderately severe neuropathy may be the only clinical sign of MyD for many years. Our results are consistent with an unusual neuropathic mutation at the MyD gene. However, involvement of 2 closely linked genes (1 for MyD and the other for HMSN) can also explain our findings.

Effects and side effects of a percutaneous thermal lesion of the dorsal root ganglion in patients with cervical pain syndrome.

Twenty consecutive patients with intractable chronic pain in the cervical region were treated with a radiofrequency lesion of the dorsal root ganglion on level C4, C5 or C6. Electromyography (EMG) and sensory evoked potentials (SEP) were recorded before and 3 weeks after the radiofrequency lesion. Side effects were studied 3 weeks, 6 weeks and 3 months after the procedure. Pain scores were evaluated on Numeric Rating Scales (NRS) before and 6 weeks after treatment. The patient was interviewed 3, 6 and 9 months after the radiofrequency lesion. The most common side effect was burning pain in the dermatome of the treated nerve root. Hyposensibility in the dermatome was noticed in 35% of patients. Except in 1 patient, these side effects had disappeared 6 weeks after treatment. The EMG showed no signs of denervation. One SEP recording remained abnormal after treatment. There was initial pain relief in 75% of patients after 3 months and in 50% of the patients after 6 months. In conclusion, this study did not reveal any signs of motor denervation after a percutaneous partial rhizotomy. There were no long-term signs of deafferentation. Initial pain relief was found in 75% of patients, but there was a marked tendency for pain to recur in a period from 3 to 9 months after treatment.

Procainamide therapy, physical performance and energy expenditure in the Schwartz-Jampel syndrome.

The effects of procainamide administration were assessed in a 5-yr-old boy with Schwartz-Jampel syndrome (chondrodystrophic myotonia). Without procainamide the resting metabolic rate was found to be significantly higher than in an age-matched control group. With a serum level of 3.8 mg l-1 procainamide a reduction of the resting metabolic rate of 22% was observed, and times needed to climb stairs and to re-open eyes after forceful contraction (blepharospasm) were significantly reduced.

Carpal tunnel syndrome: prevalence in the general population.

To study the prevalence of carpal tunnel syndrome (CTS) in the general population and the value of brachialgia paraesthetica nocturna (BPN) in diagnosing CTS, an age and sex stratified random sample of 715 subjects was taken from the population register of Maastricht (The Netherlands) and surrounding villages, between September 1983 and July 1985. The response rate was 70%. Of these, 12 CTS cases had already been diagnosed. Of the remaining subjects, 64 (13 men, 51 women) woke up because of BPN. Among these subjects 1 man and 23 women were found to have CTS. The prevalence rate of undetected CTS was 5.8% [95% confidence interval (CI): 3.5-8.1%] in adult women; 3.4 percent (95% CI: 1.5-5.3%) had already been diagnosed as CTS. The overall prevalence rate for men was 0.6% (95% CI 0.02-3.4%). These figures have to be regarded as minimal estimates. The overall diagnostic value of BPN for CTS was 38%, while for women only this was 45% (95% CI: 31-60%).

The sensory innervation pattern of the fingers.

In 32 subjects without signs or symptoms of peripheral nerve disorder, sensory nerve action potentials (SNAPs), elicited by stimulation at the wrist, were recorded from the fingers of both hands with ring electrodes. Recordings from the volar proper digital nerves confirmed the standard distribution of the median and ulnar cutaneous innervation. Recordings from the dorsal digital nerves, however, showed that the contribution of the dorsal cutaneous branch of the ulnar nerve (DCUN) strongly decreases from the little finger (always a SNAP) to the middle finger (rarely a SNAP), and that the contribution of the radial nerve decreases in an identical way from the thumb to the middle finger. Anatomical studies have shown that small branches from the DCUN and the radial nerve contribute to the innervation of the dorsal aspect of the proximal part of the middle finger, but it seems likely from our data that the predominant innervation of this area is usually provided by the median nerve. Furthermore, we found that the dorsal aspect of the proximal part of the ring finger is mainly supplied by the DCUN. These findings differ from the standard cutaneous innervation pattern as depicted in neurological textbooks, but they are in agreement with earlier anatomical studies.

Neurographic assessment of lesions of single proper digital nerves.

OBJECTIVE: To investigate which strategy should be used to assess lesions of single proper digital nerves by means of nerve conduction studies. METHODS: Comparison of the results of various orthodromic and antidromic tests with surface electrodes and needle electrodes for stimulation and recording in two patients with a lesion of a single proper digital nerve of the middle finger. RESULTS: It is shown that orthodromic and antidromic nerve conduction tests over the wrist-finger segment yield misleading results because of innervation overlap, volume conduction and co-stimulation. These problems could be avoided by selective stimulation of common digital nerves distally in the palm of the hand and recording with ring electrodes around the relevant finger. Efficacy and selectivity of stimulation are verified by simultaneous recordings from the neighbouring fingers. CONCLUSIONS: Lesions of single volar proper digital nerves are by no means rare, but no nerve conduction studies have been published so far to assess such lesions adequately. A novel, simple and reliable technique for this purpose is described.

Nerve conduction studies show no exclusive ulnar or median innervation of the ring finger.

In 2047 hands of 1260 patients referred for an analysis of their brachialgia, distal antidromic sensory conduction was investigated in the median and ulnar nerves. No median sensory nerve action potentials (SNAPs) were obtained from the ring finger (D4) in 257 hands, while ulnar D4 SNAPs were lacking in 38 hands. An obvious lesion of the relevant nerve could be demonstrated in each of these cases. In all remaining cases, in which D4 SNAPs were obtained with both median and ulnar stimulation, it could be excluded that they were due to co-stimulation. These findings are not in agreement with the occurrence of mononeural innervation of D4 as a physiological variation, which has been reported in some clinical and experimental studies. Comparison of SNAP parameters in 183 hands with increased median nerve distal latencies showed conduction to be more impaired in the fibers innervating D4 than in those supplying D3.

What is the best way to assess focal slowing of the ulnar nerve?

OBJECTIVE: The study assessed the influence of the length of the across elbow (AE) segment of the ulnar nerve on the true and false positive rates of velocity measurements of the AE segment. Using a short AE length will increase effects of the measurement error (ME), and using a long distance will 'dilute' the slowing due to the focal lesion; it is not known which length is optimal to detect focal slowing. METHODS: A simulation was performed to assess diagnostic yield for AE lengths of 50, 100 and 150 mm, taking into account ME, variation in true velocity, and severity of the lesion. ME of latencies and distances were first determined in a healthy subject. RESULTS: ME proved lower than in a published study. Diagnostic yield was consistently better for an AE length of 50 mm than for 100 or 150 mm. The optimum length is therefore near 50 mm. Yield increased with severity of the lesion, smaller ME, and when interindividual variation in true velocity was small. Judging AE on its own had a slightly better yield than comparing AE velocity to forearm velocity, except for populations with a larger than normal spread in true conduction variability. CONCLUSIONS: The best balance between effects of ME and 'dilution' to detect focal nerve slowing is obtained at nerve lengths of about 50 mm. The need to incorporate all possible compression sites necessitates the use of a suboptimal length of about 80 mm.

Comparison of thermal threshold tests to assess small nerve fiber function: limits vs. levels.

OBJECTIVE: The method of limits (MLI) and the method of levels (MLE) are psychophysical stimulus procedures most commonly applied to quantify warm and cold sensation thresholds in humans. This paper evaluates basic methodological properties of both methods and investigates the correspondence between the method's results. METHODS: Warm sensation threshold was measured in 20 healthy participants using the psychophysical MLIs and MLEs. Two differently shaped kind of levels stimuli were used with triangular (TRIANG) and trapezoid (TRAP) temperature-time profile. RESULTS: A linear model of temperature response, based on threshold level-crossing, quantifies sensation threshold, independent of the MLI inherent 'reaction-time' artifact. It results from modeling MLI responses to warm stimuli with different rates of temperature change. The model also quantifies the reaction-time delay in the physiological system from thermal stimulus presentation until manual response. This study shows that using the reaction-time independent MLE, TRAP should preferably be used for optimal quantification of sensation threshold. CONCLUSIONS: Statistical testing shows that model-based MLI threshold equals MLE threshold provided MLE TRAP stimuli are used. Recommendations for optimal MLI and MLE stimulus configurations and properties are given in relation to application of quantitative sensory testing.

Abnormal warm and cold sensation thresholds suggestive of small-fibre neuropathy in sarcoidosis.

OBJECTIVE: A substantial number of sarcoidosis patients report apparently non-specific symptoms such as pain, for which no organic substrate has yet been found. Recently we observed symptoms suggestive of small-fibre neuropathy in a group of sarcoidosis patients. The aim of the present study was to verify this observation using various electrophysiological tests. METHODS: In 74 sarcoidosis patients complaining of symptoms suggestive of small-fibre neuropathy, thresholds for warm (WS) and cold sensation (CS) as well as for heat pain were determined at the thenar eminence and the foot dorsum. Furthermore, sympathetic skin responses (SSR), nerve conduction studies and concentric needle electromyography were performed. In 31 patients, cardiovascular autonomic testing was carried out. RESULTS: Thermal threshold testing (TTT) revealed abnormalities in 51 of the 74 patients. Abnormalities showed an asymmetrical distribution. WS was affected more often than CS and feet more often than hands. Nerve conduction studies in the legs showed slightly abnormal results in 6 patients; all of these had abnormal TTT results. The SSR was absent at the foot in 7 patients. Cardiovascular autonomic testing was abnormal in only a single patient. CONCLUSIONS: In a subgroup of sarcoidosis patients we found TTT abnormalities suggestive of small-fibre neuropathy. SSR and cardiovascular autonomic testing appeared to be of little diagnostic value. Small-fibre neuropathy may be the cause of a number of hitherto unexplained symptoms in sarcoidosis.

Thermal thresholds in complex regional pain syndrome type I: sensitivity and repeatability of the methods of limits and levels.

OBJECTIVES: To study whether the method of levels (MLE) or the method of limits (MLI) is preferable as a method of measuring thermal perception thresholds in patients with complex regional pain syndrome type I (CRPS I). METHODS: Perception thresholds for warmth and cold were measured twice, with both MLE and MLI, at a 1 month interval, both at unaffected and affected wrists (n=33) or feet (n=20) of patients with CRPS I of one extremity. RESULTS: (1) Sensitivity for pathology was equal for both methods. (2) The agreement between thresholds measured by both methods was low at all locations, except for the unaffected wrist. Since thresholds measured with the MLI always contain reaction time artefacts, this lack of agreement favours the MLE. (3) At both unaffected and affected wrists, the MLE showed significantly better coefficients of repeatability as compared to the MLI for both sensations. However, at both unaffected and affected feet, there was no preference for either method as far as threshold measurement repeatability was concerned. CONCLUSIONS: Abnormal thermal perception thresholds occurred in 20% (foot) to 36% (wrist) of the CRPS I patients on the affected side and in 15% (foot, wrist) on the unaffected side. The MLE is considered to be the preferable method to assess thermal perception thresholds in CRPS I.

Denervation due to lesions of the central nervous system. An EMG study in cases of cerebral contusion and cerebrovascular accidents.

Electromyographic examination of 10 patients with severe cerebral contusion revealed profuse fibrillation potentials and positive sharp waves in many muscles, proximal as well as distal, sometimes also in the paraspinal musculature. In some cases complete denervation of a muscle was found. It appeared that these findings were not due to plexus lesions or spinal root avulsions, but that they were caused by the lesion of the central nervous system. In 10 patients with slight concussion no spontaneous activity was found. Fibrillation potentials and positive sharp waves occurred in the paretic limbs in 20 out of 21 patients with hemisyndromes due to cerebrovascular accidents. The spontaneous activity appeared after 2-3 weeks (as in peripheral nerve lesions) and disappeared or diminished considerably within 6 months. The denervation caused by the central lesion was attended by normal motor and sensory nerve conduction (apart from disturbed motor conduction to totally denervated and subsequently reinnervated muscles). It is concluded that lesions of the central nervous system may cause a dysfunction of anterior horn cells which leads to axonal degeneration, probably in the form of a dying-back process. Apparently in the majority of the anterior horn cells this dysfunction is temporary, so that axonal regeneration and reinnervation soon take place.

Small fiber neuropathy: a common and important clinical disorder.

Small fiber neuropathy (SFN) is a neuropathy selectively involving small diameter myelinated and unmyelinated nerve fibers. Interest in this disorder has considerably increased during the past few years. It is often idiopathic and typically presents with peripheral pain and/or symptoms of autonomic dysfunction. Diagnosis is made on the basis of the clinical features, normal nerve conduction studies (NCS) and abnormal specialized tests of small nerve fibers. Among others, these tests include assessment of epidermal nerve fiber density, temperature sensation tests for sensory fibers and sudomotor and cardiovagal testing (QSART) for autonomic fibers. Unless an underlying disease is identified, treatment is usually symptomatic and directed towards alleviation of neuropathic pain.

Radial tunnel syndrome. An investigation of compression neuropathy as a possible cause.

Conventional electromyographic and nerve-conduction studies usually do not show abnormalities in patients who have a clinical diagnosis of radial tunnel syndrome. Therefore, posterior interosseous nerve-conduction measurements were performed during forced supination in patients who had this syndrome. Only one of sixteen patients had a major increase in latency. Our data do not support the hypothesis that the signs and symptoms in most patients who have a diagnosis of radial tunnel syndrome are caused by compression of the posterior interosseous nerve.

Unilateral spinal muscular atrophy. A case report.

A description is given of a 36-year-old man with muscular atrophy of the right upper and lower limbs. The onset of the disorder was at the age of 26, while the condition has been stationary for the past 6 years. The case has been diagnosed as a unilateral spinal muscular atrophy.