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Recurrent Guillain-Barré syndrome and CNS demyelination
  1. J RÍO,
  2. C NOS,
  3. M TINTORÉ,
  4. M E MARZO,
  5. X MONTALBAN
  1. Unitat de Neuroimmnunología Clínica, Hospital General Universitari Vall d’Hebron, Barcelona, Spain
  1. Dr J Río, Unitat de Neuroimmunologia Clinica, Escola d’Enfermeria 5ª planta, Hospital Universitario Vall d’Hebron, Psg Vall d′Hebron 119-129, 08035 Barcelona, Spain.

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It is commonly assumed that multiple sclerosis exclusively affects the central nervous system but there is growing evidence that it is not so. There is evidence that dysfunction and tissue damage in Guillain-Barré syndrome, chronic idiopathic demyelinating polyneuropathy, and multiple sclerosis result from immune reactions within the peripheral or central nervous systems.1Although it is commonly assumed that multiple sclerosis exclusively affects the central nervous system, central nervous system demyelination has been suggested in chronic idiopathic demyelinating polyneuropathy.2 There are few reports of acute inflammatory demyelinating neuropathy associated with multiple sclerosis.3 4

Here, we report a patient with a recurrent Guillain-Barré syndrome who developed a first episode of central nervous system demyelination several years later.

A 26 year old woman was admitted to our hospital because of an acute right hemiparesis. In 1977, at the age of seven, the patient gradually developed a tetraparesis with arreflexia. The neurological deficit reached a peak two weeks after the onset of symptoms. Her CSF showed 217 mg/dl protein without cells. The motor and sensory nerve conduction study included median, ulnar, peroneal, and sural nerves. The motor nerve conduction velocities (NCVs) and distal motor latencies were 46 ms and 5 ms respectively in the median nerve, 50 ms and 4 ms in the ulnar nerve, 39 ms and 7 ms in the peroneal nerve. Sensory nerve conduction and EMG studies did not disclose abnormalities. She slowly improved and was discharged three weeks later without symptoms. The patient was asymptomatic until 1991, when she was readmitted because of an ascending weakness and distal paraesthesiae, two weeks after a flu syndrome with fever. Neurological examination disclosed paresis in all four limbs, more severe distally in the upper limbs and proximally in the lower limbs. She was areflexic. Sensory examination showed a mild deficit to pin prick, and vibration and reduced propioception was found distally in her four limbs. Lassegue sign was positive in both legs. The CSF contained 148 mg/dl protein and two leucocytes/mm3. Nerve conduction studies disclosed an absence of F responses and dispersion of motor nerve compound action potentials. Median, radial, ulnar, peroneal, and sural nerves were examined. Motor and sensory NCVs were normal except in the peroneal nerve (40 ms) and median nerve (48 ms). Distal motor latencies were 5 ms, 4 ms, and 8 ms in median, ulnar, and peroneal nerves respectively. The neurological deficit reached a peak three weeks after onset and she had recovered fully in two months and was thereafter asymptomatic. A control electrophysiological study was performed several months later and no abnormalities were found .

In 1996, she was readmitted because of an acute right hemiparesis. Neurological examination showed a right pyramidal weakness, with right Babinski’s sign and without sensory impairment. The haematological and blood chemical values were normal. A CSF analysis showed 24 mg/dl protein with three leucocytes/mm3 and oligoclonal bands were detected. Brain MRI showed multiple T2 weighted hyperintense white matter lesions in both cerebral hemispheres, brain stem, and cerebellum. One of the lesions located in the left corona radiata displayed enhancement after gadolinium administration. High dose intravenous steroids were started and there was a pronounced clinical improvement. When the patient was discharged 10 days later the neurological examination was normal. Motor NCVs were greater than 50 ms in the arms and 45 ms in the legs, and no prolonged distal motor latencies were found. Transcranial magnetic stimulation showed a delay in central motor conduction in the right arm. An EMG examination did not show abnormalities. A new MRI performed two weeks later showed a decrease in inflammatory activity.

Our patient had a recurrent Guillain-Barré syndrome with subsequent development of a first attack of a central nervous system inflammatory demyelinating disease. Recurrent Guillain-Barré syndrome consists of multiple episodes of typical acute Guillain-Barré syndrome with CSF findings and EMG abnormalities during each individual episode similar to those described in the acute monophasic disease.5 Although our patient does not fulfil criteria of clinically definite multiple sclerosis, MRI and CSF findings make the diagnosis very probable. Cranial MRI lesions have been found in chronic inflammatory demyelinating polyneuropathy although without clinical symptoms suggestive of multiple sclerosis.2 Acute inflammatory demyelinating neuropathy associated with multiple sclerosis has not been described very often.3 4 As far as we know, this is the first case of recurrent Guillain-Barré syndrome associated with a central nervous system demyelinating disease. In our patient both peripheral and central nervous myelin involvement were clinically symptomatic.

It seems that dysfunction and tissue damage in Guillain-Barré syndrome and multiple sclerosis result from impairment of immune regulation within the peripheral or central nervous system. Peripheral and central nervous system myelins exhibit a similar macromolecular organisation and some protein molecules are present in myelin from both systems; thus it is likely that, under certain circumstances, immune processes may be directed selectively against either peripheral nerves or CNS white matter. In the animal model, experimental allergic encephalomyelitis, the induction of the disease is accompanied by different degrees of peripheral involvement. Because closely related epitopes may be expressed in peripheral and central myelin, it is not unexpected that in some people there may be cross reactivity between peripheral and central nerve white matter.6 On the other hand, it has been suggested that activated T lymphocytes may function as effector cells that exert cytotoxic activity towards Schwann cells or myelin.1

Although caution is appropriate in interpreting the few reports in the literature supporting the idea that peripheral nerves are involved in multiple sclerosis, it seems likely that at least in some patients both peripheral and central nerve myelin may be involved in an acute demyelinating attack. Perhaps the peripheral and central nervous systems share antigenic properties.

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