The nervous system in early HIV infection: a prospective study through 7 years.

In this study 28 patients with early stage HIV infection (CD4 >280 x 10(6)/l) were subjected to yearly examinations of the nervous system up to 7 years in order to detect any deterioration. Clinical neurological as well as neuropsychological performance was evaluated. The patients also underwent yearly neurophysiological tests (digital EEG, visual evoked potentials, somatosensory evoked potentials, auditory evoked potentials P300 and electroneurography). Every other year, SPECT with (99m)Tc-D,L-hexamethylpropylene amine oxime and brain MRI were performed. Originally, 38 patients were included in the study but only the results of 28 patients who complied with three of more yearly check-ups are presented. The results of yearly investigations of cerebrospinal fluid (CSF) have been presented earlier [Eur J Neurol4 (1997) 1]. All the patients showed signs of HIV in the CSF. Yet, no major deterioration in the neurological, psychological performance, neurophysiological or neuroimaging examinations could be discerned.

Urethral sensitivity in incontinent women.

OBJECTIVES: The aim of this study was to ascertain whether frequent voiding and urge incontinence are associated with supersensitivity to electrical stimulation in the posterior urethra. METHODS: Current perception thresholds (CPT) were tested at four stimulus frequencies (1, 3, 20, and 100 Hz; duration 0.5 ms) using a square-wave constant current electrical stimulator connected to ring electrodes on a urethral catheter. The strength of the current at the first tingling sensation was regarded as the CPT. CPT analysis and cystometry were performed on 61 women (ages 28-89 years). RESULTS: CPTs were significantly higher at lower than at elevated stimulus frequencies, and they were also generally higher in old than in younger patients. Seven women repeated the CPT test after two months, and the thresholds were unchanged. There were no significant differences in sensitivity between patient groups with stress incontinence, urge, or mixed symptoms. Moreover, CPT was not significantly related to bladder volume at first sensation of filling. CONCLUSION: Measuring CPT is an easy and reproducible method of testing urethral sensibility, but our results do not support the suspicion that urethral hypersensitivity is involved in increased voiding frequency and urge incontinence.

Peripheral neuropathy in acrodermatitis chronica atrophicans - effect of treatment.

Forty-seven patients with the late borrelial manifestation acrodermatitis chronica atrophicans (ACA) and with objective neurological and/or neurophysiological findings were followed up after antibiotic treatment with dermatological, serological, neurological and neurophysiological controls. Despite a good therapeutic effect on ACA lesions, specific antibody values and symptoms of irritative nerve lesions, the objective neurological and neurophysiological findings of nerve deficit remained unchanged. There was no progress of neuropathy findings during the follow-up time. Our interpretation of the results is that the remaining neuropathy signs after treatment of ACA are neurological sequelae and not manifestations of persisting Borrelia infection.

Durability of success after rectocele repair.

The purpose of the study was to determine the durability of success after rectocele repair and to evaluate parameters that might influence long-term results. Twenty-five patients with rectocele were prospectively evaluated both clinically and physiologically. Follow-up was performed twice, at 1 and 5.1 years postoperatively. Twenty-four patients had a long-term follow-up; 21/23 patients (91%) with preoperative symptoms of rectal emptying difficulty reported improvement of their symptoms and 9 of 12 (75%) with preoperative symptoms of pelvic heaviness reported relief at long-term follow-up. All 5 patients with preoperative pathologic transit study had various degrees of rectal emptying difficulty at long-term follow-up. Three of 5 patients with preoperative paradoxical sphincter reaction (PSR) at electromyography (EMG) reported improvement of the symptoms of rectal emptying difficulty at long-term follow-up. Surgery for rectocele is associated with improved symptoms in a majority of patients which are sustained long term. Patients with pathologic transit study may have a less favorable symptomatic outcome. The clinical significance of PSR needs further study.

Elevation of sensory thresholds in the prostatic urethra after microwave thermotherapy.

OBJECTIVES: To determine whether transurethral microwave thermotherapy (TUMT) affects the sensory threshold in the posterior urethra and whether such an effect influences urinary storage symptoms. PATIENTS AND METHODS: The sensory threshold was measured before and at 3 and 12 weeks after TUMT in 13 men with minor obstructive symptoms caused by benign prostatic hyperplasia. Sensations were evoked by electrical stimulation at different frequencies, using a bipolar ring-electrode mounted on a urethral catheter. Changes in sensory thresholds were evaluated in the patients both as a group and individually. The patients were interviewed about their symptoms at each measurement. RESULTS: After TUMT, 12 patients were satisfied and reported decreased irritative symptoms, primarily less frequent nocturnal micturition; two patients were cured of urgency incontinence. In 11 of the satisfied patients, and the unsuccessful patient, decreased urge accompanied increased sensory thresholds. Thresholds elevated by >/= 30% were correlated with decreased irritative symptoms. CONCLUSIONS: TUMT decreases sensitivity in the posterior urethra, which may alleviate storage symptoms.

Polyneuropathy in late Lyme borreliosis - a clinical, neurophysiological and morphological description.

In a prospective study, detailed clinical and neurophysiological examinations were performed in 17 patients with polyneuropathy associated with the late borrelial manifestation acrodermatitis chronica atrophicans (ACA). Similar clinical and neurophysiological signs were found in most of the patients. The findings were those of a sensory polyneuropathy, mainly affecting large nerve fibres. Marked abnormality of vibration threshold was a common finding and in 4 patients this raised a suspicion of spinal cord engagement, in addition to a polyneuropathy. Sural nerve biopsy, performed in 3 of the patients, showed a mainly axonal neuropathy. Biopsy findings did not confirm earlier reports of vasculitis of epineural vessels in ACA-associated polyneuropathy.

Beneficial effects of dietary supplementation in a disorder with defective synthesis of cholesterol. A case report of a girl with Smith-Lemli-Opitz syndrome, polyneuropathy and precocious puberty.

In 1993 the Smith-Lemli-Opitz (SLO) syndrome, known as a malformation syndrome characterized by certain stigma, turned out to be a metabolic disease with a defect in the last step of cholesterol biosynthesis. This led to the possibility of identifying affected individuals by biochemical methods and of increasing understanding of pathogenic mechanisms. Hopes of influencing the effects of the metabolic defect by dietary supplementation were raised and reports with some benefits of treatment have been published. This is a report of a 12-y-old girl with the SLO syndrome in an apparently progressive form. In addition to typical signs and well-known symptoms she has a verified polyneuropathy and precocious puberty. She has been treated with cholesterol and bile acids for 3 y, during which time the progressive course has been arrested. A notable effect has been the improvement of her polyneuropathy, verified by measurement of nerve conduction velocities. Possible mechanisms involved in the pathogenesis of her precocious puberty are discussed.

Electromyography of the external anal sphincter: comparison between needle and surface electrodes.

PURPOSE: Electromyography of the external anal sphincter is frequently used when investigating patients with defecation disorders. Investigations are often performed using an invasive technique by perineal insertion of a needle or wire electrode. The aim of the present study was to investigate whether surface electromyography, with electrodes applied to the perineal skin, is a reliable method in the diagnosis of paradoxical anal sphincter reaction. METHODS: Seventy-one patients with defecation disorders participated in the present study. They were investigated with electromyography of the external anal sphincter using surface and needle electrodes. RESULTS: In 65 of 71 (92 percent) patients the electromyography recording showed the same result during straining using surface electrodes when compared with needle electrodes. Twenty-two of these 65 patients had paradoxical anal sphincter reaction, and 43 patients had decreased electromyography activity. In 6 of 71 (8 percent) patients the electromyography recording showed a different pattern during straining using surface electrodes when compared with needle electrodes. CONCLUSION: The present study demonstrates a good correlation between surface electrodes applied to the perineal skin and concentric needle electrodes in the diagnosis of paradoxical anal sphincter reaction. Noninvasive electromyography recordings of the external anal sphincter are often preferred in the diagnosis of paradoxical anal sphincter reaction.

Valproate monotherapy in juvenile myoclonic epilepsy: dose-related effects on electroencephalographic and other neurophysiologic tests.

A neurophysiologic test battery (consisting of a 24-hour, seven-channel electroencephalogram [EEG], EEG spectral analysis, multiple sleep latency test, visual evoked potentials, critical flicker fusion, and visual contrast sensitivity) was administered twice to 16 patients with juvenile myoclonic epilepsy (JME) in a double-blind, randomized, crossover study comparing two daily doses of sodium valproate (VPA), 1000 mg and 2000 mg. Clinical observation time was 6 months for each dose. Mean total VPA concentration during low-dose treatment was 470.4 mmol/L and during high-dose treatment was 700.0 mmol/L. Ten patients had seizures during low-dose treatment, but only three of these showed spike-wave activity on EEGs. During high-dose treatment, nine patients had seizures; five of these had spike-wave activity. EEG power spectrum did not change between doses. The other tests also showed no change between doses. Our results suggested that EEG and our selection of other neurophysiologic tests were of limited value for monitoring seizure frequency and clinical effects of VPA.

Diabetic neuropathy in elderly Type 2 diabetic patients: effects of insulin treatment.

OBJECTIVES: To study the occurrence of diabetic neuropathy and the effect of insulin treatment in elderly Type 2 diabetic patients. MATERIAL AND METHODS: In 38 patients and 20 controls symptoms and neurophysiological examinations including electroneurography, vibration perception and temperature discrimination thresholds were investigated. Patients were randomized to insulin (n = 18) or sulfonylurea (n = 16) treatment and were re-investigated after 1 year. RESULTS: Neuropathy was present in 21/38 patients (56%). It was asymptomatic in 17/38 (45%) and symptomatic in 4/38 (11%). The occurrence of neuropathy was less common in healthy controls, 3/20 (15%) (P < 0.01). Temperature discrimination thresholds was the test that most often revealed pathology. The metabolic control after 1 year was significantly improved in the insulin treated group and unchanged in the sulfonylurea treated group. There were no changes as regards occurrence of neuropathy between or within the two treatment groups after 1 year. CONCLUSION: Diabetic neuropathy is common among elderly Type 2 diabetic patients. It is mostly asymptomatic. Improvement was not seen after 1 year of insulin treatment.

Preoperative electrophysiologic assessment cannot predict continence after rectopexy.

PURPOSE: The aim of this study was to evaluate preoperative electrophysiologic assessment for prediction of anal continence after rectopexy. METHODS: Forty-three patients with rectal prolapse (n = 26) or internal rectal intussusception (n = 17) underwent concentric-needle electromyography, fiber density determination by single-fiber electromyography of the external anal sphincter, and pudendal nerve terminal motor latency evaluation before Ripstein rectopexy. A detailed history was obtained from each patient preoperatively and postoperatively. RESULTS: Anal continence was improved after rectopexy, both in patients with rectal prolapse (P = 0.06) and in those with internal rectal intussusception (P = 0.003). Abnormal results were registered in one or several aspects of the electrophysiologic assessment in 31 (72 percent) of the patients. However, functional outcome with respect to continence was not predicted by preoperative electromyography or pudendal nerve terminal motor latency assessment results. CONCLUSION: Electrophysiologic examinations in the preoperative assessment of patients with rectal prolapse and internal rectal intussusception do not predict continence after the Ripstein rectopexy. The routine use of electrophysiologic assessment requires further definition.

Paradoxical sphincter reaction is influenced by rectal filling volume.

PURPOSE: Paradoxical sphincter reaction is frequently found in constipated patients but sometimes also in incontinent patients and in asymptomatic subjects. Its significance in defecation disorders has, therefore, been debated. The aim of the present study was to investigate whether paradoxical sphincter reaction is influenced by rectal filling volume. PATIENTS AND METHODS: Eighteen patients with defecation disorders and paradoxical sphincter reaction shown by electromyography were reinvestigated with an extended electromyographic investigation while in the lying position and while in the sitting position, with 50-ml, 100-ml, and 150-ml water-filled rectal balloons. RESULTS: All 18 patients showing paradoxical sphincter reaction in the first investigation also showed the reaction at the second investigation in the lying position with a 0-ml volume of rectal contents. In the sitting position, with a volume of 150 ml of rectal contents, the increase in electromyographic activity disappeared in seven patients (39 percent) and no longer showed paradoxical sphincter reaction. Electromyography showed decreased activity in one patient and unchanged activity in six patients during straining. A closing reflex was seen after completed straining in all of these seven patients. CONCLUSIONS: The present study demonstrates that paradoxical sphincter reaction diagnosed by electromyography is influenced by the rectal filling volume and might diminish when the rectum is filled with contents. The conventional electrophysiologic technique in the diagnosis of paradoxical sphincter reaction might, therefore, overdiagnose this condition.

Peripheral neuropathy in acrodermatitis chronica atrophicans - a late Borrelia manifestation.

Clinical and/or neurophysiological signs of peripheral neuropathy were found in 64% of 63 consecutive untreated patients with the late borrelial manifestation acrodermatitis chronica atrophicans (ACA). The neuropathy frequency was significantly higher in the patients than in 30 age- and sex-matched control persons of whom 27% had neuropathy findings. The most common neuropathy in ACA was a symmetric distal sensory polyneuropathy. In a subgroup of patients with localized or asymmetric neuropathy, the changes were found more often in extremities with than without visible ACA lesions. Neuropathy symptoms, most often pain and/or paresthesia, were present in 64% of the patients, compared to in 13% of the control persons. Thus, both symptoms and signs of neuropathy were significantly more frequent in patients with untreated ACA than in control subjects.

Ekbom's syndrome of photomyoclonus, cerebellar ataxia and cervical lipoma is associated with the tRNA(Lys) A8344G mutation in mitochondrial DNA.

We have investigated nine maternal offsprings to patients with a hereditary syndrome of cerebellar ataxia, photomyoclonus, skeletal deformities and lipoma, originally described by Ekbom. The nine family members underwent a thorough neurological examination, neurophysiological investigations and molecular genetic analysis of mtDNA from lymphocytes and muscle. Clinical examination showed a partial syndrome in one relative and minor signs and symptoms in three additional offsprings. We found the heteroplasmic tRNALys A8344G point mutation in mtDNA in all investigated maternal offsprings. The fraction of mutated mtDNA ranged from 33 to 87% in lymphocytes and from 59 to 92% in muscle tissue. Analysis of mtDNA from a lipoma showed a high level (96%) of the tRNALys A8344G mutation. We conclude that Ekbom's syndrome is a mitochondrial encephalomyopathy associated with the same heteroplasmic tRNA mutation as seen in myoclonus epilepsy with ragged-red fiber (MERRF) syndrome.

Median sensory nerve conduction block during wrist flexion in the carpal tunnel syndrome.

A new method for diagnosing the carpal tunnel syndrome (CTS) is introduced. We have determined the time (T50) necessary to reach a 50% reduction in the amplitude of the median antidromic sensory nerve action potential during wrist flexion. Ten patients with anamnestic and neurophysiological evidence of CTS (group CTS) and 20 patients with CTS suspected on clinical grounds (group CTS?) were compared with 10 normal subjects. During wrist flexion the sensory conduction in the median nerve became partially blocked in all patients, and also in 8 out of 10 controls. The T50 value in 8 out of 10 CTS patients and in 14 out of 20 CTS? patients was below the lowest recorded value in the control group. It was shown that ischemia caused the blocking of the median nerve. Determination of T50 of the median nerve SNAP during wrist flexion has the potential to add to the sensitivity and specificity of the electrophysiological diagnosis of CTS.

Results of rectocele repair. A prospective study.

PURPOSE: This study was designed to evaluate the results of rectocele repair and parameters that might be useful in selecting patients for this operation. METHODS: Twenty-five patients with symptom-giving rectoceles were prospectively evaluated with a standardized questionnaire, physical examination, defecography, colon transit studies, anorectal manometry, and electrophysiology. Patients underwent posterior colporrhaphy and perineorrhaphy. They were followed postoperatively (mean, 1.0 year) with the same questionnaire, physical examination, defecography, anorectal manometry, and electrophysiology. RESULTS: Constipation had improved postoperatively in 21 of 24 constipated patients (88 percent). At postoperative follow-up 13 patients (52 percent) had no constipation symptoms, 8 (32 percent) had occasional symptoms, and 4 (16 percent) had symptoms more than once per week. Four patients with rectocele at preoperative defecography, but not at physical examination, had favorable outcomes following surgery. The majority of patients not using vaginal digitalization preoperatively had improved with respect to constipation. All patients with pathologic transit studies had various degrees of constipation postoperatively. Constipation was not improved in two of five patients with preoperative paradoxic sphincter reaction. CONCLUSIONS: Rectocele is one cause of constipation that can be treated with good results. Preoperative use of vaginal digitalization is not mandatory for a good postoperative result. Defecography is an important complement to physical examination. Patients with pathologic transit study might have a less favorable outcome of rectocele repair with respect to constipation. More studies about the significance of paradoxic sphincter reaction in these patients are indicated.

Enterocele demonstrated by defaecography is associated with other pelvic floor disorders.

Sixty-nine patients with enterocele on defaecography, and 128 patients without enterocele but with other abnormal findings were investigated to identify concomitant colorectal disorders and their relationship with enterocele. Of the 69 patients with enterocele, 38 (55%) had concomitant rectal intususception and 26 (38%) rectal prolapse. Abnormal physiological findings on anorectal manometry and electrophysiology were more common in patients with enterocele. Previous hysterectomy increased the risk of enterocele formation. The study has demonstrated that patients with enterocele should be investigated thoroughly for other lesions before treatment is planned. Further investigation of the role of enterocele in patients with defaecation disorders is required.

Surgical treatment of carpal tunnel syndrome in patients exposed to vibration from handheld tools.

During 1985 at Sabbatsberg Hospital in Stockholm, carpal tunnel decompression was done for 50 men (58 hands) with carpal tunnel syndrome (CTS). In 1988 2.5 to 3 years after the operations, a questionnaire about their present health was answered by 43 men (50 hands). In 10/17 (59%) hands exposed to vibrations and 26/33 (79%) not exposed, carpal tunnel decompression had resulted in no recurrence of neurological symptoms at follow up. Preoperative signs of neurophysiological dysfunction of the ulnar nerve, which might indicate a more widespread neuropathy, did not correlate with poor postoperative function of the median nerve. We recommend that patients with carpal tunnel syndrome should be operated on, whether or not they have been exposed to vibration.

Serum prolactin response to thyrotropin-releasing hormone during status epilepticus.

Determination of serum prolactin can help distinguish between epileptic and pseudo-epileptic attacks since generalized tonic-clonic and complex partial seizures frequently are accompanied by a transient rise in prolactin. In status epilepticus, however, serum prolactin levels are well within the normal range: cellular depletion due to the prolonged seizure activity has been suggested as a mechanism for this finding. The control of prolactin secretion is complex. Among several possible regulators, inhibitory dopamine and stimulatory thyrotropin-releasing hormone (TRH) may take part in the regulation of prolactin levels in connection with epileptic activity. There may be subpopulations of prolactin-producing cells that react differently in response to various regulators. A dopamine receptor blocker given during status epilepticus brings forth a distinct increase in prolactin levels. In order to add to the understanding of prolactin changes in connection with status epilepticus, we injected TRH i.v. during status epilepticus in seven consecutive patients. All patients had prolactin levels within the normal range (< 25 micrograms/l) before injection of TRH which resulted in at least a two-fold increase in prolactin levels. Our results contradict the hypothesis of cellular depletion of prolactin in connection with status epilepticus. The mechanism behind prolactin values within the normal range after prolonged seizure activity remains unknown.

The effect of long-term intensified insulin treatment on the development of microvascular complications of diabetes mellitus.

BACKGROUND: A cause-and-effect relation between blood glucose concentrations and microvascular complications in patients with insulin-dependent diabetes mellitus has not been established. METHODS: We randomly assigned 102 patients with insulin-dependent diabetes mellitus, nonproliferative retinopathy, normal serum creatinine concentrations, and unsatisfactory blood glucose control to intensified insulin treatment (48 patients) or standard insulin treatment (54 patients). We then evaluated them for microvascular complications after 18 months and 3, 5, and 7.5 years. RESULTS: Mean (+/- SD) glycosylated hemoglobin values were reduced from 9.5 +/- 1.3 percent to 7.1 +/- 0.7 percent in the group receiving intensified treatment and from 9.4 +/- 1.4 percent to 8.5 +/- 0.7 percent in the group receiving standard treatment (P = 0.001). In 12 of the patients receiving intensified treatment (27 percent of those included in the analysis) and 27 of those receiving standard treatment (52 percent), serious retinopathy requiring photocoagulation developed (P = 0.01). Visual acuity decreased in 6 patients receiving intensified treatment (14 percent) and in 18 receiving standard treatment (35 percent) (P = 0.02). Nephropathy (urinary albumin excretion, > 200 micrograms per minute) developed in one patient in the group receiving intensified treatment, as compared with nine patients in the group receiving standard treatment (P = 0.01). No patient in the intensified-treatment group had nephropathy with subnormal glomerular filtration rates, as compared with six patients in the standard-treatment group (P = 0.02). The conduction velocities of the ulnar, tibial, peroneal, and sural nerves decreased significantly more in the standard-treatment group than in the intensified-treatment group. The odds ratio for serious retinopathy was 0.4 (95 percent confidence interval, 0.2 to 1.0; P = 0.04) in the intensified-treatment group as compared with the standard-treatment group. The corresponding odds ratio for nephropathy was 0.1 (95 percent confidence interval, 0 to 0.8; P = 0.04). CONCLUSIONS: Long-term intensified insulin treatment, as compared with standard treatment, retards the development of microvascular complications in patients with insulin-dependent diabetes mellitus.