Impairment of the peripheral nervous system in Creutzfeldt-Jakob disease.

Background: The clinical manifestations of Creutzfeldt-Jakob disease (CJD) primarily reflect involvement of the central nervous system. The coexistence of CJD with peripheral nervous system involvement has also been reported.

Objective: To analyze peripheral neuron electrophysiologic changes and to compare these data with neuropathologic features of spinal motor neurons in patients with definite CJD.

[Clinical picture of spinocerebellar ataxia type I (SCA1)].

Spinocerebellar ataxia is a group of diseases with autosomal dominant inheritance heterogenous both clinically and genetically. So called dynamic mutations underlie most these nosological units. The clinical patterns of various SCA types have not yet been defined completely.

Motor evoked potential studies in Creutzfeldt-Jakob disease.

Objectives: Motor evoked potentials (MEPs) were recorded in 7 cases of Creutzfeldt-Jakob disease (CJD) to asses the involvement of pyramidal motor pathways in these cases. The diagnosis of CJD was confirmed by autopsy in 5 cases and based on clinical data in two cases.

Methods: Transcranial (MEP-cortex), root magnetic (MEP-root) and electrical stimulation of peripheral nerves (F-wave, direct M-response) were performed.

Peculiarity of soleus motor potentials evoked by transcranial magnetic stimulation and electrical stimulation of tibial nerve.

Objective: To reveal and discuss the peculiarities of soleus muscle in comparison with electrophysiological features of other leg muscles.

Methods: Vastus lateralis (L3), tibialis anterior (L4), extensor digitorum brevis (L5) and soleus (S1) muscles were tested at rest. Transcranial magnetic stimulation (TMS) combined which electrical stimulation of relevant peripheral nerves were applied.

[Motor segmental conduction times for extensor digitorum brevis muscle: method and normative values].

Electrical stimulation of the peroneal nerves (60 nerves) and magnetic stimulation of the roots (L5) and motor cortex were performed on 30 normal controls. The muscle responses and F wave (peripheral stimulation) were recorded from extensor digitorum brevis muscle (60 muscles). The parameters of examined potentials were measured and the central, root, peripheral motor conduction times, were estimated.

[Segmental motor conduction time for abductor of the fifth finger muscle: methods and normative values].

Electrical stimulation of the ulnar nerves (60 nerves) and magnetic stimulation of the roots (C7) and motor cortex were performed on 30 normal controls. The muscle responses and F wave (peripheral stimulation) were recorded from abductor digiti minimi muscle (60 muscles). The parameters of examined potentials were measured and the central, root, peripheral motor conduction times were estimated.

Cortical excitability threshold for distal limb muscles in transcranial magnetic stimulation. Method--normative values.

Transcranial magnetic stimulation was carried out in 30 healthy subjects, aged 20 to 55 and 156 to 180 cm tall, in whom cortical excitability threshold was determined for musculus (m.) abductor digiti minimi (ADM) and m. extensor digitorum brevis (EDB).

Segmental conduction times in the motor nervous system.

The study was performed in 30 normal subjects (24 females and 6 males) with an age range between 18 and 55 years and a height range between 154 and 188 cm. Electrical stimulation was applied to the peroneal nerve with simultaneous recording of the orthodromic response (MEP-M) and F-wave (MEP-F) from the tibialis anterior (TA) and extensor digitorum brevis muscles (EDB). Magnetic stimulation of the L5 level and cerebral cortex was applied using different coils with simultaneous recording of responses from the right and left TA and EDB muscles.

[Segmental conduction times in motor pathways for the soleus muscle--methodology and normal values].

The study performed in 30 normal subjects (24F, 6M) with age range 18-55 years and height range 154-188 cm. Electrical stimulation was applied to the tibial nerves and direct response M and F-wave was recorded from right and left SOLEUS (60 examinations). Transcranial and paravertebral (S1 level) magnetic stimulation was applied with simultaneous recording of responses from both SOL.

The F-wave in the vastus lateralis M. and the segmental motor conduction times for L2/L4 motoneurons.

The study was performed in 30 normal subjects. Electrical stimulation was applied to the femoral nerves and the muscle evoked potential (CMAP) together with their late components were recorded from the vastus lateralis muscle bilaterally (60 nerves). Features of the late component demonstrating its antidromic nature (F-wave) were proved.

[Dermatomal somatosensory evoked potentials from L3, L4, L5 and S1 spinal roots in healthy subjects].

Dermatomal somatosensory evoked potentials (DSEPs) were recorded in 62 healthy volunteers aged from 15 to 65 years (mean 36.8 +/- 12.9 years) with height from 1.

[Somatosensory evoked potentials in vascular damage to central sensory pathways].

Somatosensory evoked potentials from median nerve (SEP-M) were examined bilaterally in 43 patients with a well delimited vascular foci in central somatosensory pathways. Scalp, far field, cervical, Erb potentials and conduction times were recorded in each patients. Five subgroups based on localization of foci (parietal cortex, corona radiata, internal capsule, thalamus and extensive subcortical--cortical hemispheric focus) were distinguished.

[Routine study of somatosensory evoked potentials in healthy persons. II. Tibial nerve].

60 healthy volunteers ranging in age from 15 to 75 years participated in the study. Tibial nerve was stimulated unilaterally or bilaterally at the ankle and SEPs were recorded along the somatosensory pathway. Determination and readability of the potentials were evaluated according to the stimulating and recording method.

[Routine study of somatosensory evoked potentials in healthy persons. I. Median nerve].

The study was performed on 62 healthy volunteers (123 nerves) using standard technical parameters. In every case the median nerve was stimulated at the level of II + III fingers and at the wrist. This enabled us to compare potentials evoked by sensory versus mixed nerve stimulation.

[Computer-assisted evaluation of the status of the peripheral nervous system in alcoholics].

The authors present the principles of work of a computer system destined for evaluation of the results of routine electrophysiological examination of two muscles and four sensory nerves in alcohol-dependent subjects. The range of examinations and the programme based on discrimination analysis make possible, as shown in own investigations, an objective and complete evaluation of the state of the peripheral nervous system in these subjects. The programme is owned by the EMG laboratory of the Institute of Psychiatry and Neurology in Warsaw where it was introduced for clinical diagnosis, but it may be made available to other persons interested in these problems.

[Comparison of electrophysiologic changes in persons with alcoholic polyneuropathy and polyneuropathy caused by carbon disulfide].

A group of 39 patients with alcoholic polyneuropathy (Alc) and another group of 33 patients with carbon disulphide polyneuropathy were studied by electrophysiological methods using an identical programme, and the results were compared. In both groups evidence was found of damage to the peripheral neuron of the type of axonal neuropathy with signs of axonal loss, with moderate slowing down of conduction velocity and with associated signs of regeneration. The dynamics of changes in the Alc group was greater.

[Process of retrograde axonal degeneration in amyotrophic lateral sclerosis in the light of clinical electrophysiological studies].

Correlation analysis was performed comparing the parameters of 30 electrophysiological records obtained in a group of 28 SLA patients. The obtained results suggest that in this primary neuronal disease there is evidence of retrograde degeneration of axons (dying back). Differences in the degree of progression of this pathological process at different levels of spinal innervation enable the recognition of the sequence of electrophysiological changes taking place in the peripheral parts of the axons.