[The endonasopalpebral reflex]. / Ob éndonazopal'pebral'nom reflekse.

The authors describe the endonasopalpebral reflex which is evoked from the nasal mucosa. Reduction of this reflex may be used as a fine index of involvement of the trigeminus in patients with acoustic neuromas. This makes it possible to differentiate this tumour at early stages of its development from neuritis of the acoustic nerve. This is of great importance for this pathological situation.

Sports medicine for wheelchair athletes.

Participation in wheelchair athletic competitions has increased, and a growing number of sports have become available for disabled athletes. Benefits of participation include increased mobility and improved self-image. Common problems include soft tissue injuries, blisters, pressure sores and temperature regulation disorders.

[Differential diagnosis of Friedreich ataxia]. / Zur Differentialdiagnostik der Friedreich Ataxie.

The clinical records of 72 patients with spinocerebellar ataxias which had manifested before the age of 20, were examined in a retrospective study. Depending on whether the muscle stretch reflexes in the legs were positive or negative, two groups were distinguished, that of early onset cerebellar ataxia with retained tendon reflexes (EOCA) (13 P.) and that of Friedreich's ataxia (FA) (59 P.). The clinical course was much worse in the FA patients compared with EOCA. Cardiomyopathy and diabetes mellitus were not a feature of EOCA. Scoliosis and a disturbed position sense in the toes were more frequent in FA cases. The striking differences in clinical signs and course of the diseases justify the differentiation of EOCA from FA, as suggested by Harding in 1981.

Urologic aspects of tethered cord.

Tethered cord syndrome, a form of spinal dysrhaphism, may involve vesical neurologic dysfunction. We present herein 60 cases of tethered cord syndrome, including 24 patients who underwent preoperative urodynamics studies. Preoperative cystometrography revealed areflexic bladders in 71 percent of cases and hyperreflexic bladders in 29 percent. Eight patients had serial pre- and postoperative urodynamics testing. Slight improvement was noted in postoperative urodynamics studies performed on 4 of 6 areflexic bladders and in the only hyperreflexic bladder studied. In addition, of the 3 patients in the serial urodynamics groups who had urinary symptoms preoperatively, 2 noted clinical improvement postoperatively. The urologist's role is important in the early detection, evaluation, and treatment of tethered cord syndrome and the neuropathic bladders that may result.

[The neurologic examination in the 1st year of life. 2]. / Zur neurologischen Untersuchung im ersten Lebensjahr.

The author gives a review on normal neurological development in the first year of life. In the second part the author describes the special reflexes and reactions laying the foundation of the normal beginning of locomotion.

Carotid sinus hypersensitivity in patients with unexplained syncope: clinical, electrophysiologic, and long-term follow-up observations.

To assess the incidence and clinical characteristics of carotid sinus hypersensitivity and the relationship to electrophysiologic findings, 76 patients with unexplained syncope underwent carotid sinus massage during electrophysiologic studies for syncope evaluation. Twenty-one patients (28%) were found to have carotid sinus hypersensitivity. Of these 21 patients, 11 (52%) had coronary artery disease, two (10%) had hypertensive heart disease, and eight (38%) had no organic heart disease. During electrophysiologic studies, abnormal sinus node function was found in three patients (14%), abnormal atrioventricular (AV) node function was noted in four (19%), and combined abnormal sinus node and AV node functions were seen in three (14%). Eleven patients (53%) had a normal electrophysiologic study. During carotid sinus massage, sinus arrest alone was observed in 12 patients (57%), and combined sinus arrest and AV nodal block was seen in nine (43%). Thirteen patients were treated with a permanent pacemaker, in whom either carotid sinus massage reproduced the symptom or concomitant sinus node or AV node abnormality, or organic heart disease was present. With a mean follow-up of 42 +/- 19 months, none of these 13 patients had recurrent syncope. However, one of eight patients (13%) who did not receive a pacemaker had recurrence of syncope. Subsequently, this patient has done well after implantation of a pacemaker. These observations suggest that there is a significant incidence of carotid sinus hypersensitivity in patients with unexplained syncope. Permanent pacing appears to be beneficial in selected patients based on clinical and electrophysiologic findings.

Three patients with Fisher's syndrome and normal MRI.

Three patients with Fisher's syndrome had normal MRI scans of the brainstem 6 days, 7 days, and 5 weeks, respectively, after the onset of illness. This further supports a peripheral origin for the ataxia and ophthalmoplegia in this illness and distinguishes it from brainstem encephalitis.

The usefulness of head-up tilt testing and hemodynamic investigations in the workup of syncope of unknown origin.

To enhance the clinical evaluation of patients suffering from recurrent syncope of unknown origin, the integrity of mechanisms controlling blood pressure was examined in 151 patients utilizing a screening tilt test. Ninety-eight patients had an abnormal blood pressure and/or heart rate response to tilt testing, including provoked syncopal attacks in 63 patients. Whenever indicated, the screening tilt test was followed by blood volume and hemodynamic determinations, as well as autonomic nervous system testing to identify contributing pathophysiological abnormalities (hypovolemia, venous pooling, autonomic dysfunction). Detailed analysis of this battery of tests allowed us to conclude that: (1) The tilt test is commonly a provocative tool in the workup of patients with recurrent syncope due to vasovagal - vasodepressor reactions and other abnormalities of blood pressure regulation; (2) Its usefulness is augmented by associated hemodynamic and blood volume evaluations; (3) The identification of contributory pathophysiological mechanisms of blood pressure control facilitates specific therapeutic interventions.

Bethanechol supersensitivity test, rhabdosphincter electromyography and bulbocavernosus reflex latency in the diagnosis of neuropathic detrusor areflexia.

A total of 57 patients with neuropathic or nonneuropathic detrusor areflexia was studied with the bethanechol supersensitivity test, electromyography of the urethral rhabdosphincter and bulbocavernosus reflex latency. The sensitivity of these tests in detecting neuropathic areflexia was 90, 87.5 and 78.1 per cent, respectively, and the specificity was 95.6, 76 and 80 per cent, respectively. When all 3 tests were performed together the combined accuracy approached 100 per cent. These combined tests are useful in the diagnosis of patients with equivocal bladder neuropathic conditions and in those with subtle neurological lesions.

Neurophysiological investigations in potent and impotent men. Assessment of bulbocavernosus reflex latencies and somatosensory evoked potentials.

A multidisciplinary investigation was carried out on 130 patients with erectile dysfunction. The bulbocavernosus reflex (BCR) latency times were evaluated in all patients and in 116 the somatosensory evoked potentials (SSEP) were also assessed. BCR latency times were examined in 39 potent control patients and the SSEP were measured in 30 members of this group. There were no abnormal findings in the control group. Of the 130 patients with erectile dysfunction, 65 had anomalies in the BCR measurements and 36 of 116 patients had abnormal SSEP findings. When compared with the control group, 76 of 130 patients with erectile disorders (66%) had irregularities in BCR latency times and/or SSEP. As a result of these findings, lesions of the somatic penile innervation are considered to be one of the many causes of erectile dysfunction.

Predictive criteria for failed sphincterotomy in spinal cord injury patients.

Nine patients with chronic high spinal cord injury who failed sphincterotomy secondary to detrusor hypocontractility underwent neurourological evaluation. Testing revealed an abnormality involving the sensory limb of the somatic sacral reflex arc (abnormal R wave) or at the level of the spinal interneuron pool (abnormal or absent S wave). This study strongly suggests that the sensory input from the bladder to the spinal cord, together with alteration in the sensory motor integration that occurs at the level of the interneuron pool, is important in influencing the characteristics of the detrusor contraction following high spinal cord injury. Predictive criteria for failed sphincterotomy are a slow increase to maximum intravesical pressure before voiding and abnormal lumbosacral sensory evoked potentials.

The EEG and clinical evolution in Creutzfeldt-Jacob disease.

A total of nine EEGs were recorded serially from a typical, pathologically verified case of Creutzfeldt-Jacob disease (CJD). Typical periodic sharp waves (PSW) showed a progressive increase in duration, but the interval of PSW stayed fairly constant during the course of illness. Early in the illness there were PSW in approximately one-quarter of the records and this reached one hundred percent when clinical CJD was fully developed. Diagnosis of CJD on the basis of the EEG alone is difficult in the early phase of the disease due to the short duration of the PSW.