Comparative study between intra-anal sponge and needle electrode for electromyographic evaluation of constipated patients.

PURPOSE: The aim of this study was to compare the intra-anal sponge electrode with the conventional needle electrode for electromyography of the pelvic floor in constipated patients. MATERIALS AND METHODS: Forty consecutive patients (27 females) with a mean age of 64.3 (range, 15-87) years who had chronic constipation were prospectively evaluated for electromyographic evidence of nonrelaxation or paradoxical contraction of the puborectalis and external anal sphincter during simulated defecation. The soft intra-anal sponge electrode and then the concentric needle electrode were used in each patient as an internal control. Furthermore, in all patients, cinedefecography was used as an independent standard to confirm the diagnosis. Agreement was calculated using the kappa statistic. RESULTS: Confirmation of needle electromyography was noted in 19 of 20 patients (95 percent) who had sponge electromyographic evidence of paradoxical activity. Similarly, concurrence was noted in 19 of 20 patients (95 percent) with normal relaxation of the puborectalis observed with the sponge electrode. Agreement between needle and sponge electromyography was very good (kappa = 0.9), between needle electromyography and cinedefecography was fair (kappa = 0.4), and between sponge electromyography and cinedefecography was moderate (kappa = 0.5). Furthermore, needle electromyography was more painful in all patients compared with sponge electromyography. CONCLUSION: The soft sponge surface intra-anal electrode is an excellent alternative to the needle electrode for assessment of puborectalis activity in constipated patients. Sponge electromyography has the advantage of being as accurate as, but less painful than, needle electromyography.

Proposed algorithm to aid the diagnosis of cerebellopontine angle tumors.

Hearing and/or balance disorders are symptoms that may be associated with tumors of the cerebellopontine angle (CPA). Between March 1988 and May 1995, 22 patients had diagnosis or evaluation of CPA tumors at Cleveland Clinic Florida. The most common presenting signs or symptoms included unilateral low-frequency tinnitus, unsteadiness, and/or asymmetric hearing loss. On the basis of our experience, we have constructed an algorithm to help identify and manage acoustic nerve dysfunction. We compare our findings with national epidemiologic data and current medical reports.

Variation in pudendal nerve terminal motor latency according to disease.

PURPOSE: The aims of this study were first to establish whether any difference among pudendal nerve terminal motor latency (PNTML) values exists relative to diagnosis, second to determine whether left and right latencies are similar, and third to assess any correlation between age and neuropathy. Latency was elicited three times on each side, and an average latency was recorded as a result. MATERIALS AND METHODS: Between June 1989 and April 1995, 1,026 patients (775 females and 251 males) underwent PNTML study. These patients were divided into four groups according to diagnosis: Group I, fecal incontinence; Group II, chronic constipation; Group III, idiopathic rectal pain; Group IV, rectal prolapse. Overall mean age was 61.5 (range, 6-95) years. Student's t-test was used to calculate statistical differences. Patients were then analyzed according to age and gender. Correlation was calculated with the nonparametric Mann-Whitney U test. RESULTS: Unilateral or bilateral prolongation of PNTML was noted in 90 patients (21.2 percent) in Group I, 80 (20.4 percent) in Group II, 22 (18.1 percent) in Group III, and 38 (42.6 percent) in Group IV. Average PNTML on the left side was 1.88 ms in Group I, 1.94 ms in Group II, 1.98 ms in Group III, and 2.12 ms in Group IV. Average PNTML on the right side was 1.85 ms in Group I, 1.94 ms in Group II, 1.99 ms in Group III, and 2.07 ms in Group IV. The only statistically significant differences in PNTML were between Groups I and IV (left, P < 0.005; right, < 0.05) and between females and males (P < 0.0001). CONCLUSION: There is no statistically significant difference between latencies of left and right pudendal nerves. Similarly, there are no statistically significant differences among patients with fecal incontinence, chronic constipation, or chronic idiopathic rectal pain. Normal latency can be expected in patients with constipation or fecal incontinence. However, patients with rectal prolapse have a more prolonged PNTML. Age is correlated with a higher incidence of pudendal neuropathy. This study reveals significant overlap among PNTML values and diagnosis.

Electrodiagnostic evaluation of fecal incontinence.

The aim of this study was to assess the utility of electrodiagnostic testing (EDT) for the evaluation of fecal incontinence (FI). Over a 5-year period, 225 patients (174 females) with FI were prospectively studied with anal manometry, anal ultrasonography, anal electromyography (AEMG), and pudendal nerve terminal motor latency (PNTML) assessment. The mean age was 60 (range 12-94) years. Causes of FI identified by clinical evaluation were obstetric injuries (45), rectal prolapse (43), iatrogenic or other trauma (42), neurologic disease (23), and idiopathic (72). EDT revealed abnormalities in 76% of patients. The incidence of pudendal neuropathy (PN) was 36% (bilateral 21%, unilateral 15%). Patients with PN were older than were those with normal PNTML (mean 71 vs. 63 years; P < 0.002). No relationship between squeeze pressure and PN could be demonstrated (P = 0.9). Reduced motor unit potential (MUP) recruitment on AEMG was present in 60% and was associated with decreased squeeze pressure (P < 0.001) and increased MUP polyphasia (P < 0.001). Concurrence of AEMG and anal ultrasonographic findings was observed in 35 of 41 patients (84%). Defects were overlooked in one study but identified by the other on three occasions, each. Moreover, 8 of 22 patients with demonstrated sphincter defects had unsuspected PN or extensive sphincter injury on AEMG that precluded sphincter repair. In conclusion, EDT proved to be a valuable tool in the evaluation and subsequent treatment of patients with FI.

Pudendal neuropathy is not related to physiologic pelvic outlet obstruction.

UNLABELLED: Chronic straining because of pelvic outlet obstruction is hypothesized to cause pudendal neuropathy (PN) by stretch injury. PURPOSE: The aim of this study was to determine any association between PN and pelvic outlet obstruction. METHODS: One hundred forty-seven constipated patients were evaluated by cinedefecography and pudendal nerve terminal motor latency assessment. PN was defined as a latency longer than 2.2 ms. Pelvic outlet obstructive patterns of evacuation included paradoxic contraction, nonrelaxation of the puborectalis muscle, or failure of opening of the anal canal. RESULTS: Median length of constipation including straining during evacuation was eight (range, 1-47) years. Ninety-one (62 percent) subjects had a normal pattern of evacuation, and 56 (38 percent) had an obstructed pattern. Both groups had a similar median age (68 vs. 69 years, respectively; P > 0.05) and same median length of symptoms. Overall incidence of PN was 23.8 percent (10.9 percent unilateral and 12.9 percent bilateral). There was no difference in the incidence of PN between patients with normal evacuation and patients with obstructed evacuation (24.2 percent vs. 23.2 percent, respectively; P > 0.05). Patients with PN had a similar incidence of physiologic pelvic outlet obstruction as did patients without PN. However, median age of patients with PN was higher than those without PN (73 vs. 66 years, respectively; P > 0.05). CONCLUSION: There was a difference in the incidence of PN between normal and obstructed patterns of evacuation. Therefore, the espoused theory that obstructed defecation causes PN appears flawed.

Pudendal neuropathy in evacuatory disorders.

PURPOSE: Aims of the present study were to assess frequency of pudendal neuropathy in patients with constipation and fecal incontinence, to determine its correlation with clinical variables, anal electromyographic assessment, and anal manometric pressures, and to determine usefulness of the pudendal nerve terminal motor latency assessment in evaluation of these evacuatory disorders. METHODS: From 1988 to 1993, 395 patients (constipated, 172; incontinent, 223) underwent pudendal nerve terminal motor latency, electromyography, and anal manometry. Pudendal neuropathy was defined as a pudendal nerve terminal motor latency greater than 2.2 ms. RESULTS: Patients were a mean age of 60.7 (range, 17-88) years. Overall incidence of pudendal neuropathy was 31.4 percent (constipated, 23.8 percent; incontinent, 37.2 percent; P < 0.05). Incidence of pudendal neuropathy dramatically increased after 70 years of age in both groups (22 percent vs. 44 percent; P < 0.05). Moreover, subjects with pudendal neuropathy were older than those without pudendal neuropathy (mean age, 67 vs. 57 years; P < 0.05). The presence of pudendal neuropathy was associated with decreased motor unit potentials recruitment in patients with incontinence (P < 0.01). Patients with and without pudendal neuropathy had a similar mean squeezing pressure in both groups. CONCLUSION: Pudendal neuropathy is an age-related phenomenon. Although pudendal neuropathy is associated with abnormal anal electromyographic findings in patients with incontinence, no association with anal manometric pressures was found. Pudendal nerve terminal motor latency assessment is a useful tool in the evaluation of patients with fecal incontinence, but its role in the assessment of constipated patients remains unknown.

Role of pudendal nerve terminal motor latency assessment in constipated patients.

UNLABELLED: The importance of pudendal nerve terminal motor latency assessment for the evaluation of incontinence is well established. However, its role in constipated patients remains unclear. PURPOSE: The purpose of the present study was to assess the incidence of pudendal neuropathy in constipated patients and its correlation with others variables including age, sex, anal pressures, and anal electromyography. RESULTS: From 1988 to 1993, 161 patients with chronic constipation underwent pudendal nerve terminal motor latency assessment, anal electromyography, and anal manometry. The overall incidence of pudendal neuropathy was 23.6 percent; females and males had a similar incidence (24 percent vs. 23 percent, respectively; P > 0.05). Patients over 70 years old had a significantly higher incidence of pudendal neuropathy than did patients under 70 years (37 percent vs. 12 percent, respectively; P < 0.01). Patients with paradoxical puborectalis contraction on anal electromyographic assessment had a higher incidence of bilateral neuropathy, paradoxical puborectalis contraction (+)23 percent vs. paradoxical puborectalis contraction (-)8 percent, P < 0.05. Patients with pudendal neuropathy also had a higher incidence of decreased motor units potential recruitment than did patients without pudendal neuropathy (31.5 percent vs. 17 percent, respectively; P > 0.05). CONCLUSIONS: Pudendal nerve terminal motor latency assessment was able to detect unsuspected pudendal neuropathy in 24 percent of patients. This finding correlated with age and with the presence of paradoxical puborectalis contraction but not with manometric anal pressures, motor unit potentials recruitment, or the presence of polyphasia. However, the often espoused relationship between pudendal latency and external sphincter function could not be demonstrated.

Anorectal manometry in the diagnosis of paradoxical puborectalis syndrome.

This prospective study was undertaken to compare the utility of anorectal manometry (ARM) with that of anal electromyography (EMG) and cinedefecography (CD) in the diagnosis of paradoxical puborectalis syndrome (PPS). One hundred sixteen consecutive patients with a history of chronic constipation were prospectively assessed. These 35 males and 81 females were of a mean age of 60 years, ranging from 18 to 84 years. The incidences of PPS were 63 percent for ARM, 38 percent for EMG, and 36 percent for CD. The correlations of PPS were suboptimal: ARM and EMG, 70 percent; and ARM and CD, 61 percent. A two-tiered system for the manometric classification of PPS was developed. First, the evacuation pressure curve pattern was classified as a normal relaxed downward (Type A; n = 43), a nonrelaxed flat or equivocal (Type B; n = 36), and a paradoxical upward (Type C; n = 37). PPS was noted with increasing incidence within curve types (21 percent in Type A, 64 percent in Type B, and 95 percent in Type C). Second, an evacuation index (EI = evacuation pressure/squeeze pressure) was defined: Group I (EI < 0; n = 43), Group II (0 < or = EI < 0.25; n = 24), Group III (0.25 < or = EI < 0.5; n = 27), and Group IV (EI > or = 0.5; n = 18). The finding of PPS also correlated with the EI group: 21 percent in Group I, 67 percent in Group II, 74 percent in Group III, and 100 percent in Group IV. This subdivision of curve types and EI groups may provide a role in the diagnosis of PPS.

Cinedefecography and electromyography in the diagnosis of nonrelaxing puborectalis syndrome.

A prospective study was undertaken to assess the correlation between electromyography (EMG) and cinedefecography (CD) for the diagnosis of nonrelaxing puborectalis syndrome (NRPR). Clinical criteria for NRPR included straining, incomplete evacuation, tenesmus, and the need for enemas, suppositories, or digitation. EMG criteria included failure to achieve a significant decrease in electrical activity of the puborectalis (PR) during attempted evacuation. CD criteria included either paradoxical contraction or failure of relaxation of the PR along with incomplete evacuation. In addition, other etiologies for incomplete evacuation, such as rectoanal intussusception or nonemptying rectocele, were excluded by proctoscopy and defecography in all cases. One hundred twelve patients with constipation, 81 females and 31 males, with a mean age of 59 (range, 12-83) years were studied by routine office evaluation, CD, and EMG. Forty-two patients (37 percent) had evidence of NRPR on CD (rectal emptying: none, 24; incomplete, 18). Twenty-eight of these patients (67 percent) also had evidence of NRPR on EMG. EMG findings of NRPR were present in 12 of 70 patients (17 percent) with normal rectal emptying. Conversely, 14 of 72 patients (19 percent) with normal PR relaxation on EMG had an NRPR pattern on CD. The sensitivity and specificity for the EMG diagnosis of NRPR were 67 percent and 83 percent, and the positive and negative predictive values were 70 percent and 80 percent, respectively. Conversely, if EMG is considered as the ideal test for the diagnosis of NRPR, CD had a sensitivity of 70 percent, a specificity of 80 percent, and positive and negative predictive values of 66 percent and 82 percent, respectively. In summary, sensitivity, specificity, and predictive values of EMG and CD are suboptimal. Therefore, a combination of these two tests is suggested for the diagnosis of NRPR.

Neurophysiologic assessment of the anal sphincters.

One hundred twenty consecutive patients with either fecal incontinence (60 patients), chronic constipation (41 patients), or idiopathic intractable pelvic pain (19 patients) were prospectively assessed. Patients underwent concentric needle electromyography (EMG), bilateral pudendal nerve terminal motor latency evaluation, anorectal manometry, and cinedefecography. The most common EMG finding in patients with fecal incontinence was decreased recruitment of motor units with squeezing and polyphasic motor unit potentials; these are consistent with an injury pattern. The most common EMG finding in the constipated patients was paradoxical puborectalis contraction. This latter abnormality was also a frequent finding in patients with rectal pain, as was prolongation of pudendal nerve latency. Paradoxical puborectalis contraction was diagnosed more frequently with EMG than with cinedefecography. Inter-examination correlation was best in the incontinent group between EMG and manometry. Cinedefecography had poor correlation with EMG in all patient groups but was valuable in the detection of additional pathology such as rectoanal intussusception and anterior rectocele. Electromyography including pudendal nerve terminal motor latency assessment is a valuable adjunct in the evaluation of disorders of evacuation. The information it yields is complementary to that offered by more routine physiologic examinations.

Thyrotropin-releasing hormone (TRH) in murine motor neuron disease (the wobbler mouse).

Clinical benefits of thyrotropin-releasing hormone (TRH) were tested in wobbler mice, an animal model of motor neuron disease. After the disease was clinically recognized at 3-4 weeks, the animals were divided into two groups, each group consisting of 5 pairs of wobbler mice and normal littermates. TRH (50 mg/kg) and normal saline (NS) were injected intraperitoneally daily, 6 times per week for 9 weeks, in a double-blind study. Weekly assessments consisted of front paw grip strength, push walking, body weight, and semiquantitative grading. At the end of the trial, the brain and spinal cord were sampled to measure TRH and cyclo (His-Pro) concentrations. Progression of motor neuron disease was evident in wobbler mice, regardless of treatment. Descriptive semiquantitative gradings showed the tendency of improvement in TRH-treated wobbler mice. In saline-injected controls, TRH levels in the cervical spinal cord were significantly increased (P less than 0.01) in wobbler mice compared to littermates. However, with TRH treatment, there was no significant difference in TRH and cyclo (His-Pro) levels in any neural tissue between wobbler and controls. The lack of clinical benefits with TRH in wobbler mice may be due to increased TRH levels found in diseased spinal cord in murine motor neuron disease.

Phenelzine toxicity responsive to dantrolene.

We describe a 44-year-old man with signs of phenelzine sulfate (a monoamine oxidase inhibitor) toxicity, including coma, hyperpyrexia, hypotension, tachycardia, generalized muscular rigidity, and hyperreflexia. He recovered after IV treatment with dantrolene. Dantrolene appears to be an effective treatment for phenelzine toxicity manifested by a hypermetabolic state.

Amyotrophic lateral sclerosis: effects of acute intravenous and chronic subcutaneous administration of thyrotropin-releasing hormone in controlled trials.

We performed double-blind crossover trials to assess the effects of thyrotropin-releasing hormone (TRH) on amyotrophic lateral sclerosis patients. For acute intravenous trials, 500 mg TRH or placebo with norepinephrine was given at 1-week intervals (16 patients). CSF TRH concentration increased, and clinical side effects appeared with TRH. For chronic studies, 25 mg TRH and a saline placebo were given subcutaneously every day for 3 months (25 patients). CSF TRH level increased 29-fold after a single TRH injection, and mild transient side effects occurred. Vital signs, respiratory function, semiquantitative and quantitative neurologic function, muscle strength by manual and dynamometer testing, and EMG were studied. With daily TRH, 10 patients noted subjective improvement without objective evidence, and 10 patients complained of worsening of the disease with objective decline after TRH was stopped. Statistical analysis, however, showed no beneficial effects from either acute or chronic TRH trials.

Focal inflammatory myopathy.

We report three patients with inflammatory myopathy who presented clinically with weakness and wasting of only one limb. The myopathy progressed over 6 months and 5 years, respectively, in two patients and was stable after 8 years in the third patient. One patient had a skin rash. Serum CK was elevated in the two patients with progressive disease. Electromyography showed brief duration, small amplitude motor unit potentials and fibrillations in the affected limbs. Muscle biopsy revealed variable fiber size, degenerating and regenerating fibers, and inflammatory foci. Vasculitis was seen in the patient with skin lesions and marked fibroblastic proliferation in the patient with the most chronic course. Immunosuppressive therapy has arrested the progression in the two patients treated; both have regained strength.

Adult-onset acid maltase deficiency presenting as diaphragmatic paralysis.

A 51-year-old man presented with a six-year history of gradually progressive exertional dyspnea. His complaint was attributed to increasing age, but evaluation finally led to a diagnosis of diaphragmatic paralysis. Neurological examination disclosed only minimal evidence of weakness of limb muscles. Electromyographic studies suggested a diagnosis of acid maltase deficiency, which was confirmed by electron microscopic and biochemical studies of muscle biopsy sections. Exercise stress testing was necessary to define the limits of the patient's exercise tolerance.

Hashimoto's thyroiditis, primary biliary cirrhosis, and myasthenia gravis.

A previously undescribed triad of autoimmune diseases--Hashimoto's thyroiditis, myasthenia gravis, and primary biliary cirrhosis--is reported. A possible common cell-mediated immune disorder is postulated.

Metastatic prostatic adenocarcinoma to the temporal bone.

A patient had paralysis of multiple lower cranial nerves (VII, IX, X, XI, and XII) and oculosympathetic fibers, and ipsilateral conductive hearing loss. Histologic analysis of a surgical biopsy of the temporal bone revealed metastatic adenocarcinoma of prostatic origin. This is only the second reported case in the English literature (seventh in the world literature) of prostatic adenocarcinoma metastasizing to the temporal bone.

Traumatic ulnar neuropathy from jet injection: case report.

The usefulness and relative safety of the technique of jet injection, mainly used in mass immunization, have been well established. A patient who developed a traumatic ulnar neuroma at the site of jet injection of swine flu vaccine is reported. This unusual and previously unreported complication of this procedure is thought to have occurred because of the patient's extremely small size and small muscles.