Biomedical subjects
Publications and source records attributed to W Tackmann.
Progressive supranuclear palsy, first described as clinical entity by Steele, Richardson and Olszewski, is a degenerative disorder of the central nervous system. Besides progressive supranuclear oculomotor disturbances, other characteristic signs are pseudobulbar paresis, axial rigidity, gait disturbances and subcortical dementia. Misinterpretation in the early stage as Parkinson's disease is frequently seen. A causal therapy is still missing.
The familial spastic paraplegia is a rare disorder of the cerebral nervous system characterized by a slowly progressive spasticity of the lower limbs with early or late onset. There are pure and complicated forms. The disease is inherited by an autosomal dominant mode and a recessive one. In this study 6 patients out of 5 families are presented. The clinical variety, genetic aspects, electrophysiological results and differential diagnosis are discussed.
The prevalence of clinical and electrophysiological signs of peripheral nerve disease was evaluated in 151 patients with chronic obstructive pulmonary disease. Patients with concomitant disorders affecting the peripheral nervous system were excluded. Thirty patients had clinical signs of a mild sensorimotor and distal neuropathy and 13 additional patients had only electrophysiological abnormalities. The rate and the severity of the neuropathy correlated with the severity of chronic hypoxaemia. Three out of 20 patients with mild hypoxaemia (PaO2 less than 15 mm Hg below normal) had polyneuropathy as compared with 15 out of 36 with severe hypoxaemia (PaO2 more than 30 mm Hg below normal (rates different at the 10% level)). PaO2 and age were the only variables discriminating between patients with and without peripheral neuropathy.
Adrenoleukodystrophy (ALD) is characterized by adrenal cortical insufficiency and progressive demyelination of the central nervous system with lethal outcome in childhood. Adrenomyeloneuropathy (AMN) represents a clinical variant of ALD with later manifestation during adolescence. The neurological symptoms of this form are slowly progressive spastic paraparesis and peripheral neuropathy. Other intermediate forms, which might be classified as adrenoleukomyeloneuropathy (ALMN), possibly represent an overlap of the two conditions. Heterozygote carriers of the X-linked disease may also show neurological symptoms, such as spastic paraparesis and peripheral neuropathy. Biochemically, all these X-linked forms of ALD/AMN are characterized by an accumulation of very-long-chain fatty acids in various tissues and body fluids indicating an impaired peroxisomal metabolism. In this study two families with ALD are presented. The two forms of ALD and AMN were observed in different members of the families including a heterozygote female carrier with neurological symptoms. The different clinical syndromes, the biochemical and genetic basis, and new therapeutical strategies are discussed.
A patient with positive serological HIV-II reactions is presented, who lived for many years in North and West Africa and on the Arabian peninsula, and who developed asthenica, incontinence, gait disturbances, impaired mental function, personality changes and finally spastic paraplegia and peripheral neuropathy. He was shown to have a chronic inflammatory process of the central nervous system with cerebral atrophy.
Fibre density and amplitudes of macro-EMG motor unit potentials were studied and compared with conventional EMG in the anterior tibial muscles from 51 patients with amyotrophic lateral sclerosis. The fibre density was increased in 46 muscles. Increased amplitudes of macro-EMG motor unit action potentials were found in 46 muscles, while the mean duration of motor unit potentials recorded with a concentric needle electrode was prolonged in only 26 muscles. Changes in the packing density of muscle fibres of surviving motor units are thought to influence the different electrophysiological parameters in different ways.
The bulbocavernosus reflex (BCR) was examined in 39 normal potent men and in 252 patients with impaired potency of varying aetiology. For BCR evaluation minimum, maximum and mean latencies, the temporal dispersion in ten successive responses, together with minimum and maximum side differences from simultaneous recordings of the left and right bulbocavernosus muscles were determined. Pathological findings were detected in 125 patients. Somatosensory evoked potentials (SSEPs) recorded from the scalp after stimulation of the penile dorsal nerves and the terminal branches of the pudendal nerve were investigated in 30 controls and in 246 patients. An abnormal SSEP was found in 63 patients.
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.
Acute trichinosis can be followed by a chronic stage. Myalgia is the main clinical symptom in the chronic course of the disease. From 19 patients with a history of acute trichinosis 2-42 years ago, 15 patients suffered from chronic myalgia. Other typical complaints of our patients in the chronic stage were opthalmodynia, burning of the eyes, headache and early fatigability.
A 53-year-old man and his 18-year-old son, both suffering from benign focal muscular atrophy of upper extremities are described. To our knowledge this is the first familial case of this disease in Caucasians.
Brainstem auditory evoked potentials (BAEP) evoked by condensation, rarefaction clicks and clicks of alternating polarity were examined in 52 controls and in 95 patients with a certain or presumed diagnosis of multiple sclerosis. It was shown that the type of stimulation could influence the shape of the BAEP considerably. In multiple sclerosis patients pathological results were found in 67 ears from 44 patients. Rarefaction as well as condensation clicks independently could elicit BAEPs with increased latencies of one or more peaks, while clicks of alternating polarity failed to detect abnormalities in a great number of cases. These findings make the application of the latter type of stimulation doubtful.
Based on 12 patients with posttraumatic erectile dysfunction due to pelvic fractures, ruptures of urethra and straddle-trauma, the diagnostic and therapeutic means in the management of erectile failure are discussed. The most frequent etiology encountered in posttraumatic erectile failure were combined arterial-neurogenic lesions followed by insufficiencies of the venae profundae penis as a sequelae of pelvic fractures or symphyseal ruptures. Promising therapeutic alternatives represent injections of cavernous bodies with vasoactive drugs, penile implants and also in suitable cases penile revascularization procedures.
On 40 motor unit potentials recorded from the abductor digiti quinti muscle of 4 normal subjects the alteration of shape and duration under the influence of decreasing temperature (36 to 22 degrees C) has been examined. The mean increase of duration was about 6%/degrees C for the temperature range between 36 and 30 degrees C and about 9%/degrees C for the range between 30 and 22 degrees C. A significant alteration of shape, e.g. an increased incidence of polyphasic potentials as observed by Buchthal, could not be demonstrated. This prolongation of motor unit potential's duration probably results mainly from an increased temporal dispersion of impulse propagation in the terminal branches of motor nerve fibres and from a delayed impulse conduction over the muscle fibre membrane.
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Vestibular responses (vertigo, nystagmus-like eye movements) to acoustic stimuli are known as the "Tullio phenomenon". Detailed electro-oculographic analysis of this reaction, as observed in a 30-year-old patient, revealed the following: a maximum amplitude of eye movement (mainly vertical) was achieved by sine wave bursts of high intensity, a frequency of 500 to 1000 Hz and a duration of 100 ms. The ocular deviation was composed of a fast initial component, followed by a slower resetting movement that was often divided into two parts of different velocities. At longer stimulus durations (more than 100 ms) the electro-oculogram showed a fractionation of the eye deviation, terminating in an "off-response". Various positions of the patient's head influenced the direction of the eye motion. The possibility that the Tullio phenomenon may be due to an abnormal excitation of the statolith organs is discussed.
An overview on the diversity of neurologic complications of infections with different human herpes virus strains is given. Encephalitis, meningoencephalitis and the Guillain-Barré-syndrome are of major importance. Affection of single cranial nerves and mononeuropathies occur in a lesser frequency, while myelitis and isolated disturbances of the autonomic nervous system are rare complications. Therapeutically the application of acyclovir in herpes simplex and varicella zoster virus infections has given encouraging outlooks, whereas no convincing results exist with respect to cytomegalovirus and Epstein-Barr virus infections.
The bulbocavernosusreflex (BCR) was examined in 34 controls and in 98 patients with disturbances of potency. The shortest and longest latencies, the mean and the temporal dispersion out of ten successive reflex responses were measured. Additionally the smallest and largest side differences were determined when recording reflex responses simultaneously from the right and left bulbocavernosus muscles. BCR measurements revealed pathological results in 57 patients. Most sensitive parameters were the temporal dispersion and maximum side difference.
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