The Guillain-Barre syndrome is a distinctive neuropathy characterized pathologically by the presence of inflammatory lesions which occur scattered throughout the peripheral nervous system. The lesions consist of circumscribed areas in which myelin is lost in the presence of lymphocytes and macrophages. Myelin damage of effected largely by macrophages, which penetrate the basement membrane around nerve fibers and strip what appears to be normal myelin away from the body of the Schwann cell and off the axon. While there is evidence that this activity is immune mediated, the precise mechanism that leads macrophages to seek out and amputate a specialized region of the Schwann cell plasma membrane remains unexplained.

Pathology of the Guillain-Barré syndrome.

Electrophysiological studies were performed on 114 patients with Guillain-Barre syndrome and the literature was reviewed. Abnormalities of motor and sensory conduction were present in about 90% of the patients. Because of the patchy distribution of demyelinating lesions, the likelihood of detecting electrophysiological abnormalities increases with the number of nerves studied. Marked slowing of conduction or an abnormally prolonged distal motor latency consistent with demyelination was found in one or more nerves in about 50% of the patients. Abnormalities of conduction not present in the early stages of the illness may become evident at the peak of disability or during the recovery phase. The application of F-wave studies and somatosensory evoked potential recordings may assist in detecting demyelination in roots and proximal segments of the peripheral nervous system. There is no correlation between the degree of slowing of conduction and the duration of illness or the long-term prognosis. Abnormalities of conduction at follow-up do not correlate with the clinical disability. On the other hand, electromyographic findings of abundant spontaneous fibrillation and other evidence of axonal degeneration are associated with a poor prognosis for complete recovery and also with a slower rate of recovery from the initial illness.

Electrophysiological studies in the Guillain-Barré syndrome

Acute idiopathic polyneuritis. A clinical and electrophsiological follow-up study.

Seventeen patients, treated for Wilson's disease (WD), underwent a set of neuropsychological tests and were compared with a closely matched control group. There were clear differences between the groups (chi 2-test, p less than 0.0001). Wilson patients with only hepatic involvement, however, did not at all differ from their controls. Wilson patients with neuropsychiatric signs differed from controls on a reasoning test (p = 0.0016), and the entire WD group differed on a perceptual speed task (p = 0.0025). Compared to normal test values, however, the patients' group means were all within plus or minus one standard deviation from the normal mean. Special testing procedures and construction of the test battery excluded a factor of motor deficits as a major cause for the differences. The neuropsychological findings are viewed in relation to other findings in patients with motor disorders and predominantly subcortical lesion sites. Wilson's disease may be a dementing condition, but not when treated adequately.

Neuropsychological findings in treated Wilson's disease*

Background Recognition of Wilson disease (WD) is sometimes difficult because of its diverse manifestations. Peripheral neuropathy is rarely reported in the context of WD. Objective To report an unusual patient with WD whose initial manifestation was peripheral neuropathy. Design Case report. Setting Neurology department in a tertiary referral center. Method Personal observation. Result A 17-year-old man, who was eventually diagnosed with WD, was initially seen with polyneuropathy at least 6 months prior to developing more typical symptoms of WD. Electrophysiological and pathological studies suggested a neuropathy of mixed type. Treatment for WD resulted in clinical and electrophysiological improvement. Conclusion Wilson disease may initially appear as a treatable polyneuropathy.

https://jamanetwork.com/journals/jamaneurology/articlepdf/789540/NOB30213.pdf

Wilson disease with an initial manifestation of polyneuropathy.

The sural nerve from a patient with'Wilson's hepato-lenticular degeneration was examined by electron microscopy. The myelin sheaths showed remarkable changes and the axons secondary changes, while the unmyelinated nerve fibres were intact. These findings demonstrate that pathological changes of peripheral nerves occur in Wilson's disease. The changes are considered to be primary degeneration of the myelin sheaths.

A biopsy case of Wilson's disease. Pathological changes in peripheral nerves.

N. suralis taken from a patient showing the typical Landry-Guillain-Barre Syndrome was examined electron microscopically and showed degenerative changes of the myelin sheaths, Schwann cells, axons and unmyelinated nerve fibres. The changes in the unmyelinated nerve fibres might be concerned with disturbances of autonomic function.

A biopsy case of Landry-Guillain-Barré syndrome.

N. suralis and M. quadriceps femoralis, taken from a patient with peripheral nerve paralysis following severe diarrhoea, were examined by electron microscopy. In the nerve fibres myelin sheaths were swollen and degenerated: axons in myelinated fibres also were degenerated. Degeneration was also observed in Schwann cells. In muscle the noteworthy findings were focal irregularity of Z-bands and disappearance of thick filaments. In all parts of the muscle, glycogen granules were remarkably increased in number.

Kohei Miyakawa

Papers

A biopsy case of Wilson's disease. Pathological changes in peripheral nerves.

A biopsy case of Landry-Guillain-Barré syndrome.

Ultrastructural study of a nerve and muscle biopsy from a case of “subacute myelo-optico-neuropathy”