Comorbidity profiles among patients with bullous pemphigoid: a nationwide population-based study.

BACKGROUND: Bullous pemphigoid (BP) has been associated with neurological and psychiatric diseases; however, large-scale population-based study of different comorbid diseases in patients with BP is quite limited. OBJECTIVES: We sought to analyse the prevalence of neurological, psychiatric, autoimmune and inflammatory skin diseases prior to the diagnosis of BP and their associations with BP among patients with BP from a nationwide database in Taiwan. METHODS: A total of 3485 patients with BP and 17,425 matching controls were identified from the National Health Insurance Database in Taiwan from 1997 to 2008. Conditional logistic regression analyses for a nested case-control study were performed to examine the prevalence of comorbidities prior to the diagnosis of BP between these two groups. RESULTS: Overall, our results showed that stroke [odds ratio (OR) 3·30; 95% confidence interval (95% CI) 3·03-3·60], dementia (OR 4·81; 95% CI 4·26-5·42), Parkinson disease (OR 3·49; 95% CI 3·05-3·98), epilepsy (OR 3·97; 95% CI 3·28-4·81), schizophrenia (OR 2·56; 95% CI 1·52-4·30) and psoriasis (OR 2·02; 95% CI 1·54-2·66) were significantly associated with BP. Among them, the association with schizophrenia and psoriasis was predominant in female and male patients, respectively, with BP. It remains for all these comorbid diseases to be independently associated with BP by multivariate analysis. CONCLUSIONS: Patients with BP are more likely to have various neurological diseases, schizophrenia and psoriasis prior to the diagnosis of BP, supporting associations found in other studies. Further research is required to elucidate the tentative causal association with BP.

Effect of sacral-root stimulation on the motor cortex in patients with idiopathic overactive bladder syndrome.

AIMS OF THE STUDY: It is presumed that idiopathic overactive bladder syndrome (OBS) is due to visceral hypersensitivity. Sacral-root stimulation can restore the bladder function, but its mechanism remains uncertain. It is well-known that long-term peripheral stimulation can induce brain plasticity. Hence, we investigated whether brain reorganization occurred along with clinical improvement after sacral-root stimulation. MATERIAL AND METHODS: Because toe flexion is the index for monitoring wire placement, we used the flexor hallucis brevis (FHB) as the target muscle. Transcranial magnetic stimulation (TMS) was applied to study motor cortex excitability and the brain mapping of the muscle. RESULTS: Six patients with idiopathic OBS were included in the study. All demonstrated clinical improvement after sacral-root stimulation. Motor cortex excitability and the area of representation for the flexor hallucis brevis muscle increased for at least 30 min after sacral-root stimulation had terminated. CONCLUSION: Our results showed that cerebral activities changed after sacral-root stimulation. The improvement in urinary urgency and urgency perception was probably due in part to brain reorganization.

MR findings of decerebrate rigidity with preservation of consciousness.

We describe a case of decerebrate rigidity, with preservation of consciousness, caused by a discrete pontine tegmentum lesion identified on MR imaging. Lesions within a certain brain stem region are responsible for decerebrate rigidity in animal studies, but there has been a lack of MR imaging evidence in humans. This report also implies that a discrete lesion was responsible for the decerebrate rigidity, while consciousness was preserved.

HMPAO brain SPECT in acute carbon monoxide poisoning.

UNLABELLED: Technetium-99m-hexamethylpropylene amine oxime (HMPAO) brain images with fanbeam SPECT, in combination with surface three-dimensional display, were used to detect basal ganglion and cerebral cortex anomalies in the acute phase of carbon monoxide (CO) poisoning. METHODS: Ten patients, aged 16-29 yr, with acute CO poisoning and no past history of neurologic disorders were enrolled in this study. After oxygen treatment, all 10 patients were investigated using 99mTc-HMPAO brain images with fanbeam SPECT and surface three-dimensional display. Meanwhile, 6 of 10 patients also received a brain CT scan. RESULTS: CT scan findings were negative in all 6 patients. Fanbeam SPECT demonstrated unilateral or bilateral hypoactivity of basal ganglia in 6 patients. Local hypoactivity anomalies were found in the brain cortex of 7 patients, using surface three-dimensional display of the brain. Only 2 of 10 patients had normal 99mTc-HMPAO brain images. CONCLUSION: This study suggests that, in comparison with traditional brain imaging techniques, 99mTc-HMPAO brain imaging with fanbeam SPECT in combination with surface three-dimensional display is a better tool for early detection of regional cerebral anomalies in acute CO poisoning.

Proximal slowing in carpal tunnel syndrome resulting from either conduction block or retrograde degeneration.

In the carpal tunnel syndrome (CTS), decreased conduction velocity (CV) of the median nerve in the forearm segment has been ascribed to an electrodiagnostic artefact rather than pathophysiological changes. Standard CV of the forearm segment is calculated by subtracting the distal latency, which may not represent an exact assessment of CV in the proximal median nerve. A new technique modified from the method of Stoehr et al. and Pease et al. can exactly measure CV over the forearm. Using this new technique, the forearm nerve action potentials (FNAP) amplitude and forearm nerve conduction velocity (FNCV) proximal to the wrist can be directly determined. Normal subjects and patients with CTS were studied by both the standard and the new FNAP methods. Patients were divided into subgroups according to the severity derived from standard electro-diagnostic findings. By comparing the normal control and patient subgroups, the results show that there was a significant decrease in FNAP amplitudes proportional to severity, but FNCV was reduced to a lesser extent. In addition, the standard forearm median motor CV (MMCV) correlated well with severity, but the reduced MMCV did not correlate with the decreased FNCV. These findings suggest that retrograde degeneration of the median nerve does exist in CTS; however, retrograde degeneration contributes little to the reduced forearm MMCV which substantially results from the block of faster conduction fibres at the wrist. Therefore, technique artefact plays a major role in causing the proximal slowing in the standard electrodiagnosis.

Lack of cervical paraspinal muscle involvement in juvenile distal spinal muscular atrophy: an electromyographic study on 15 cases.

Electromyography (EMG) of the lower cervical paraspinal muscles was performed in 15 young Chinese males with distal spinal muscular atrophy of the upper extremities. The lack of fibrillation and positive sharp waves in all patients, both in early or active and chronic or steady stages, did not correlate with the EMG status in the affected upper extremity on the same side. This finding is in striking contrast with that in amyotrophic lateral sclerosis.

Effect of stimulation of an upper limb on motor evoked potentials in lower limb muscles to transcranial magnetic stimulation in normal subjects and patients with thalamic infarction.

The effects of conditioning stimulation of an upper limb on motor evoked potentials (MEPs) of relaxed muscles in both lower limbs were studied in 7 normal subjects and two patients with left thalamic infarction. A possible mechanism for the Jendrassik maneuver (JM) is that induced proprioceptive input ascends supraspinally to facilitate the descending volleys. In order to mimic the JM with a more controlled influence, we used an electrical conditioning (C) stimulation (4 times sensory threshold) delivered to the left index finger preceding the transcranial (T) magnetic stimulation at C-T intervals of 0-200 ms. The MEP facilitation of bilateral tibialis anterior (TA) and gastrocnemius medialis (GC) was within C-T 70-110 ms. The peak facilitation was at C-T 80 ms for ipsilateral TA (309%) and GC (405%) and at C-T 90 ms for contralateral TA (207%) and GC (283%). In the two thalamic infarction patients with right-sided sensory loss, the facilitation did not occur when the conditioning stimulation was delivered to the affected index finger. Therefore, it is likely that the peripheral volley must be transmitted supraspinally to facilitate MEPs of the lower limbs. This method for studying sensory facilitation is more quantitative and reproducible than the JM and technically better than other previously described methods for somatosensory conditioning.

Modulation of symptomatic palatal tremor by magnetic stimulation of the motor cortex.

OBJECTIVES: Magnetic stimulation of the motor cortex can be used to determine the involvement of the cortex in rhythmic movement disorders. Symptomatic palatal tremor (SPT) is thought to come from a pacemaker that is relatively resistant to internal and external stimulation. In this study, we investigated the effect of magnetic stimulation of motor cortex on SPT. METHODS: Five male patients, aged 67-79 years, with SPT after brain stem infarction or hemorrhage, all had a synchronous mouth angle twitch with the palatal movement. Electromyographic activity was recorded with a monopolar needle electrode from orbicularis oris. In experiment 1, transcranial magnetic stimulation (TMS) was delivered at 200% motor threshold (MT) to reset SPT. In experiment 2, the effect of TMS intensities was studied at 80-240% MT in two SPT patients. To determine the influence of the TMS, we used the resetting index (RI). RESULTS: TMS reset the tremor in all 5 SPT patients at 200% MT with RIs of 0.86-0.96. The latency of the tremor reappearance after TMS was longer than the pre-stimulus tremor interval, and the intervals between the subsequent tremor bursts were also prolonged. The degree of tremor resetting was closely correlated with the magnetic stimulus intensity and the latency of the tremor reappearance after TMS. CONCLUSIONS: Stimulation of the motor cortex may modulate the generator of SPT.

Bilateral neurotrophic keratopathy complicating Vidian neurectomy.

PURPOSE: To present a case of bilateral neurotrophic keratopathy with miosis after bilateral Vidian neurectomy for vasomotor rhinitis. METHODS: Observational case report. RESULTS: A 68 year-old man presented with irritation and blurred vision in both eyes 2 weeks after bilateral Vidian neurectomy. Slit-lamp examination revealed a large epithelial defect, typical of neurotrophic keratopathy, in the inferior two-thirds of cornea in both eyes. Corneal sensitivity test with a Cochet-Bonnet anesthesiometer and electrical study of the blink reflex indicated bilateral trigeminal dysfunction. Both pupils were miotic, and tests with hydroxyamphetamine 1% and epinephrine 0.1% showed postganglionic sympathetic nerve damage. The corneal epithelial defects healed after 2 months of treatment with systemic prednisolone, vitamin B(12) and tarsorrhaphy. CONCLUSION: Neurotrophic keratopathy associated with dry eye syndrome may be a serious complication of Vidian neurectomy.

Cutaneous reflexes in patients with acute lacunar infarctions.

Cutaneous reflex (CR) was measured in 15 patients with acute lacunar stroke and in 15 age- and sex-matched normal subjects. EMG activities were recorded from the first dorsal interosseous muscle with surface electrodes, rectified and averaged. Intensity of the stimulation on the index finger was four times the sensory threshold. Aided by audio- and visual-feedback monitoring, the subjects abducted the index finger. Side-to-side comparison was made on all the subjects. CR abnormalities were found in all of our patients. The early inhibitory (I1) and second excitatory (E2) components were attenuated in 11 of 15 patients. The I1-E2 interpeak amplitude (A2) was significantly reduced in all the patients. Prolonged latency of I1-E2 components was found to be pronounced in six of eight patients with sensory symptoms. Eight patients were followed up 1 month later. By that time, the abnormal CR had reversed to some degree in all of them. CR alternation was still present in two fully recovered patients. Our results implied a thalamic relay in the afferent limb of the CR. CR could be a valuable adjunct for assessing long tract dysfunction and monitoring functional recovery.

Swallowing difficulty in primary progressive aphasia: a case report.

A 71-year-old right handed man showed insidious and progressive decline of language skills, in contrast with relatively preserved performance on tests and ability to be independent in daily living. Three years later, he developed swallowing difficulties. MRI scans showed widening of the left sylvian fissure. A SPECT scan demonstrated focal hypometabolism restricted to the left temporal lobe. Videofluoroesophagography revealed marked hesitation of oral preparation and oral phases, while the reflex phases were unimpaired. The patient failed to perform oral tasks on command and imitation without motor and sensory deficit. We believe that his swallowing difficulty was due to oral apraxia. This case adds to the heterogeneity of patients with primary progressive aphasia.

Orientation landmarks of endoscopic transaxillary T-2 sympathectomy for palmar hyperhidrosis.

The identification of the T-2 ganglion through a narrow operative viewfield is the greatest challenge in performing endoscopic transaxillary T-2 sympathectomy, especially for a surgeon who is unfamiliar with the technique. The authors describe a simple anatomical method for identifying the T-2 ganglion during the operation, based on a study of 17 adult cadavers. First, a similar clinical procedure was performed along the anterior or middle axillary line via the second to fourth intercostal spaces to measure the aiming angles and intrathoracic depth needed. Second, the regional anatomical structures and their relationship to bilateral T-2 ganglia were delineated. It was discovered that the superior intercostal artery, a branch of the subclavian artery, was an accessible landmark. This small vessel existed in 87.5% of the cadavers studied. It consistently runs lateral to the parallel sympathetic chain at an average distance of 10 mm. Most important is that it can be easily distinguished where it runs across the inner part of the second rib. The authors emphasize that the superior intercostal artery should be a very beneficial landmark for surgical orientation.

Botulism: heart rate variation, sympathetic skin responses, and plasma norepinephrine.

BACKGROUND: Botulism may involve the autonomic nervous system. METHODS: We assessed the autonomic function of 6 botulism patients with heart rate variations, sympathetic skin responses, and plasma norepinephrine. RESULTS: Two weeks after onset, all the patients had absent sympathetic skin response in the palm and sole. Compared with controls, the heart rate variation of botulism patients was significantly decreased at rest (3.1 +/- 1.2% vs. 20.9 +/- 2.0%, p = 0.0018) and during deep breathing (4.3 +/- 2.3% vs. 29.7 +/- 2.6%, p = 0.0018). The botulism patients had significantly lower plasma norepinephrine levels (supine 29.2 +/- 10.1 pg/ml vs. 257.5 +/- 65.8 pg/ml, p = 0.0018; standing 40.3 +/- 13.1 pg/ml vs. 498.5 +/- 85.6 pg/ml, p = 0.0018). The heart rate variation and sympathetic skin response was greatly improved 6 months after onset. CONCLUSIONS: Heart rate variation, absence of sympathetic skin response, and low plasma norepinephrine are all manifestations of autonomic dysfunction in botulism patients.

Tongue dyskinesia as an early manifestation of Wilson disease.

A 15-year-old boy was diagnosed as having Wilson disease. He perceived involuntary tongue movement and speech disorder since March 1990. The tongue movements presented in the resting state and during action. It contracted transversely and bilaterally with an irregular frequency about 1 Hz. As a result, the sides of the tongue moved to form a narrow central groove. This was quite different from the tongue protrusion of tardive dyskinesia. His speech had imprecise consonants, monopitch, low pitch, low volume, harsh voice, and hyponasality. These suggested that tongue dyskinesia could be an early sign of Wilson disease and was not the main cause of his dysarthria.

Syncope as the presenting symptom of nasopharyngeal carcinoma.

Syncope is a rare symptom of nasopharyngeal carcinoma, a common tumor in Taiwan. We describe two patients with nasopharyngeal carcinoma who presented with frequent syncopal attacks five months before the diagnosis was made. We postulate that the mechanism of syncope is involvement of the glossopharyngeal or vagal nerve by para-pharyngeal extension of the tumor.

A trial study of leukocyte labeling with stabilized Tc-99m D,L-HMPAO by methylene blue and sodium phosphate buffer.

UNLABELLED: We attempted to label leukocytes with stabilized Tc-99m D,L-HMPAO by methylene blue and sodium phosphate buffer (S-HMPAO). METHODS: The results were compared with unstabilized Tc-99m D,L-HMPAO (U-HMPAO). U-HMPAO was obtained by reconstituting a commercial vial of D,L-HMPAO. Stabilization of the kit was performed by the addition of methylene blue. The leukocytes were labeled using a modified published method. The test samples of S-HMPAO and U-HMPAO were prepared immediately, and stood for 0.5, 1, 2, 4, and 6 h, respectively, at room temperature before analysis. RESULTS: In comparison with U-HMPAO: (1) the radiochemical purity of S-HMPAO was higher; (2) the labeling efficiencies of S-HMPAO labeled leukocytes were higher and consistent; (3) the viability of S-HMPAO labeled leukocytes was as high as the viability of U-HMPAO labeled leukocytes at any time; and (4) the percentages of disintegrated from S-HMPAO labeled leukocytes in plasma were lower. CONCLUSION: S-HMPAO is more stable than U-HMPAO and can provide higher leukocyte labeling efficiency. S-HMPAO, therefore, has the potential to replace U-HMPAO as a leukocyte-labeling agent.

Evaluation of cervical radiculopathy by cervical root stimulation.

For precise evaluation of cervical radiculopathy, cervical root stimulation (CRS), conventional electromyographic (EMG) studies, nerve conduction velocity (NCV) studies and F responses were done in 32 patients with clinical symptoms and signs of cervical radiculopathy. While performing CRS, a monopolar needle was inserted into the paraspinal muscles, and the compound muscle action potentials (CMAPs) in the biceps, triceps and abductor digiti minimi muscles were recorded. Conventional EMG was abnormal in 18 (56.2%), whereas CRS was abnormal in 25 (78.1%). Among the 25 patients with positive CRS tests, 13 received surgical decompression for cervical root compression caused either by a cervical disc or by foramen narrowing due to spondylosis. Only 10 out of 13 (76.9%) had abnormal conventional EMG findings. CRS provides a sensitive method for making a direct evaluation of proximal root conduction and is a good aid for pre-surgical evaluation of cervical radiculopathy.