[Optic neuritis caused by herpes zoster ophthalmicus. Case report and review of the literature].
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Biomedical subjects
Publications and source records attributed to W Tackmann.
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Two patients, aged 0.1 and 2 years, with homozygous hypobetalipoproteinaemia, one with PKU in addition are described. The clinical evaluation showed no neurologic abnormalities. Treatment with a fat-reduced, protein and carbohydrate-enriched diet was combined with vitamin A and E supplementation, according to the suggestions for classical abetalipoproteinaemia. In the patient with PKU the protein intake was increased by using a phenylalanine-free, amino acid mixture. Only by this, were normal growth and weight gain achieved.
A group of 37 patients (total 41 nerves) with a traumatic transection of median or ulnar nerves at the wrist were reinvestigated clinically and electrophysiologically 4-59 months after primary or secondary suture or grafting. Clinically there was no relation between the time after the operation, and sensory recovery determined according to the schedule of Nicholson and Seddon (1957), two-point discrimination, vibration threshold. There was also no relationship between the time after suture, and the motor latencies as well as amplitudes of evoked muscle action potentials from the abductor pollicis brevis or hypothenar muscles. In sensory nerve fibres there was a statistically significant increase of the maximum amplitude and of the cumulative amplitude during the period after operation, due to an increasing number of regenerated nerve fibres. Sensory nerve conduction velocities showed no relation to the time after suture. Cumulative amplitudes were significantly related to two-point discrimination and to restitution of sensibility. As indicated by the great scatter, however, this parameter is merely a moderate predictor for the degree of clinical recovery. Somatosensory evoked potentials can be helpful in some cases to indicate nerve regeneration when nerve action potentials in peripheral nerves cannot be recorded. However, latencies and amplitudes of the individual peaks did not reveal any relationship to either clinical findings or to period of time after operation. Needle electromyography also yielded highly variable findings. A constant finding was a persistent loss of motor units. Most of these were increased sin size, as indicated by prolonged duration of their action potentials during slight voluntary effort.
The application of microsurgical techniques for the removal of acoustic neuromas has evidenced greatly improved postoperative results. Therefore the early detection of an acoustic neuroma is a special need for every neurologist. The development of new electrophysiological and radiological procedures now allows a far better approach in the diagnosis of acoustic neuromas. Brain stem auditory evoked potentials as well as acoustic reflex testing have shown to be rather sensitive tools in the diagnosis of retrocochlear pathology. Other electrophysiological procedures have been widely replaced by these techniques. Gasmeatocisternography in combination with cranial computed tomography is the method of choice in the detection of small acoustic neuromas. The current status in the application of different techniques for the diagnosis of acoustic neuromas is reviewed.
A total of 103 patients (40 with pure sensory disturbances, 63 with sensory and motor deficits) with ulnar nerve lesions at the elbow were examined neurophysiologically. The measurement of motor conduction velocity across the cubital tunnel alone did not completely localize the lesion. The latency to an ulnar-innervated flexor muscle was of outstanding importance. The measurement of amplitudes was only rarely of localizing significance. The results of sensory recording proved to be important in patients with pure sensory disturbances. In cases with additional motor deficits, sensory recordings were too often abnormal in all segments of the ulnar nerve to be of substantial localizing value.
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A total of 37 patients with traumatic transection of median or ulnar nerves at the wrist (total 41 nerves) were examined clinically and electrophysiologically 4-59 months after primary or secondary suture or grafting. There was a significant increase of cumulative amplitude with the time after suture, whereas maximum sensory nerve conduction velocity and maximum amplitude of nerve action potentials did not reveal such a correlation. The recovery of two-point discrimination, vibration threshold and sensibility scored according to the scale of Nicholson and Seddon were also not related to the passage of time after operation. Though there were significant correlations between cumulative amplitude and both two-point discrimination and recovery of sensibility, electrophysiological parameters were shown to be inadequate predictors of clinical recovery.
A peculiar form of chronic spinal muscular atrophy is described, 5 unrelated young males developed muscle wasting of the left shoulder and upper arm region, mainly in the distribution of myotomes C5 and C6. This muscle atrophy remained confined to the primary site for 13-18 years, being followed in 4 cases by a mild muscle atrophy of the right upper arm or or the left forearm and hand and in 1 case by atrophy of the legs and the facial muscles. On EMG examination, muscle involvement was always found to be more widespread than on clinical examination. The repeated and extensive EMG examinations gave results typical of an anterior horn cell disease. Pyramidal signs were absent. During the observation period of 21-40 years the course was benign and at least partial working capacity was retained until retiring age.
20 patients who had clinical symptoms and/or signs of compression of the 7th and/or 6th cervical roots were examined. Motor and sensory nerve conduction velocities, velocities in the proximal segment of the median nerve determined by measuring F-wave latencies, cervical somatosensory evoked potentials, and needle electromyographic records of the Mm. deltoideus, biceps brachii, triceps brachii, abductor pollicis brevis, and abductor digiti quinti were investigated. Only in 11 out of the 20 patients electromyographical results coincided with the clinical findings with regard to the level of the root lesion. F-wave conduction velocities within the proximal segment of the median nerve were completely normal. Somatosensory evoked potentials revealed alterations in 8 cases; 2 of them showed an additional alteration on the clinically apparently healthy side. In our opinion the various electrophysiological methods seem to have only a limited value in the diagnosis and localization of cervical root compression syndromes.
Pattern shift visual evoked potentials (VEPs), cervical and cortical somatosensory evoked responses (SEPs) and motor conduction velocities studied by F-wave latency measurements were investigated in two family members with hereditary neuropathy with liability to pressure palsies (HN-PP). In both cases in VEPs and SEP conduction times N13-N20 were normal. A bilateral pathological increase of latencies of early SEP components, N9-N13 transit times and F-wave latencies revealed a lesion in the proximal parts of the median nerves close to the spinal cord in the older patient. These abnormalities emphasize the close relationship of HN-PP with hereditary polyradiculopathy (Mayer 1975).
Pattern shift visual evoked potentials, brain stem auditory evoked potentials, spinal and scalp recorded somatosensory evoked potentials, and electrically elicited blink reflexes were investigated in 32 patients with isolated optic neuritis. Eleven patients were shown to have one additional lesion in the central nervous system outside the optic nerve. Therefore, cases with optic neuritis of unknown origin should be considered as possible cases of multiple sclerosis.
We report on morphometric investigations of peripheral nerves in a woman, who died at the age of 69, presenting the classical symptoms of oculopharyngeal muscular dystrophy (OPMD) and a typical family history with several members (males and females) affected over three generations. Evidence for chronic axonal atrophy was found in peripheral nerves and especially in oculomotor nerves with severe axon loss in endomysial nerve twigs of extraocular, laryngeal, and tongue muscles. Whereas limb muscles presented features of neurogenic atrophy, severe changes of "myopathic" type were evident in extrinsic eye muscles, laryngeal constrictor, tongue, and diaphragma. However, we interpreted these changes as neurogenic in origin in view of the severe denervation found in those muscles. Our findings suggest that OPMD is a disease of primary neurogenic origin rather than a primary myopathic disorder.
The descriptive term "hereditary motor sensory neuropathy (HMSN)" includes a group of disorders of different nosology which besides heredity show a progressive distal symmetrical motor sensory neuropathy as a common clinical feature. The etiology of these disorders is probably different. According to electrophysiological and morphological findings two major types of HMSN can be distinguished from each other: 1. The neural type (HMSN, type I) is characterized by axonal degeneration, segmental demyelination and hypertrophic changes. The nerve conduction velocity in this type is significantly slowed. 2. The neuronal type (HMSN, type II) shows axonal degeneration with secondary segmental demyelination and no hypertrophic nerve changes. The nerve conduction velocity in these cases is normal or insignificantly decreased. This paper includes a historical review and a detailed description of the clinical, electrophysiological and morphological findings of the two major types of HMSN. The current classifications of HMSN are cited and critically discussed.
Blink reflexes elicited by electrical stimulation of the supraorbital nerves, by monoaurally presented 1 kHz sine wave bursts of 20-ms duration, and by flashes of light presented monocularly were investigated in 32 healthy controls. Absolute latencies of the individual components and side differences were measured. An outline of possible anatomical pathways which might be involved in the transmission of these reflexes is given.
Blink reflexes, elicited by flashes of light, tone bursts and electrical stimuli, as well as checkerboard reversal visual-evoked potentials and brain stem auditory evoked potentials were investigated in 55 patients with different degrees of diagnostic probability of multiple sclerosis. It is demonstrated that electrically and acoustically elicited blink reflexes are simple but rather sensitive methods of indicating brain stem lesions. They should be used complementarily with brain stem auditory potentials. Flash-elicited blink reflexes, although sensitive, are thought to be of minor importance, since they give only restricted localizing information.
Orthodromic and antidromic nerve conduction velocities were determined in sensory median nerve fibres from digit to palm and from palm to wrist in patients with the carpal tunnel syndrome. In a large number of subjects the carpal tunnel syndrome could be detected only when the palm-to-wrist segment was investigated. Comparison of the results of the corresponding orthodromic and antidromic nerve conduction velocity measurements along the different segments revealed no significant disadvantages for the antidromic method. This technique should be given preference to the orthodromic method since it is easier.
Thirty patients with diabetes mellitus were investigated. Twenty-seven had clinical signs of a symmetrical sensory or sensorimotor polyneuropathy. Features of autonomic dysfunction were found in 17 patients. Nerve conduction was studied in the femoral, deep peroneal, saphenous, sural and sensory and motor fibres of the posterior tibial nerves. The postural heart rate response was tested in all of the 30 patients. There was only a weak correlation between autonomic disturbances and sensorimotor polyneuropathy, based either on clinical or on electrophysiological results. Thus a multifactorial causation is suspected.