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.