Miller Fisher Syndrome: The Localisation of Pathology

Jamal, Goran A (1987) Miller Fisher Syndrome: The Localisation of Pathology. MD thesis, University of Glasgow.

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The syndrome described by Miller Fisher (1956) comprises an acute ophthalmoplegia (or ophthalmoparesis) associated with severe ataxia, predominantly of gait and trunk, and a mild to moderate increase in the cerebrospinal fluid protein level unassociated with pleocytosis. As in the Guillain-Barre syndrome, there is usually an antecedent infection commonly of the upper respiratory tract. The illness has a benign course with rapid and usually complete recovery. The precise nature, aetiology and main site of pathological changes in the Miller Fisher syndrome are not well understood and have been the subject of some controversy. Since neuropathological evidence from typical cases of the syndrome is lacking, due to the benign nature of the illness, hypotheses have been based on clinical observations. Opinions have suggested either that the syndrome is related to acute inflammatory demyelinating polyradiculoneuropathy, or, that it is due to a brainstem inflammatory lesion, or, that both such types of component are present. This thesis is an attempt to address the question of identification of the main site of action of the pathological process in the Miller Fisher syndrome. Comprehensive multimodal and serial neurophysiological investigations, testing both the peripheral and central nervous system, have been applied to a group of seven typical patients with the syndrome at standardised intervals from onset of the illness up to and after full recovery. The results are compared with those from 20 patients with classical Guillain-Barre syndrome systematically investigated in a similar way, and with those previously reported in the literature. The findings are then discussed in the context of peripheral versus central nervous system dysfunction. In the first chapter, a review is presented of a small number of reports of similar cases described incompletely prior to Fisher's original 1956 account, followed by an analysis of 84 patients with the syndrome reported in the literature and by an outline of some of the controversies concerning the underlying pathology. Chapter 2 describes the clinical findings together with the course, laboratory investigations and case analysis of 7 patients with typical Miller Fisher syndrome. In chapter 3, the neurophysiological methods and the timing of their application are detailed, together with a critical appraisal of their reliability. The investigations used included: 1. electromyography. 2. nerve conduction studies. 3. late response (H-reflex and F-wave) studies. 4. estimation of motor unit numbers and motor unit potentials analysis. 5. peripheral facial nerve and blink reflex studies. 6. computerised quantitative sensory (thermal and vibration) threshold measurements. 7. muscle silent period studies. 8. multimodal evoked potentials (somatosensory, brainstem auditory and visual) studies. 9. electroencephalography. 10. Quantitative pupillometric and pharmacological observations on the pupils. The results of the neurophysiological investigations and their evolution with time are presented in chapter 4. The findings indicated the presence of a significant dysfunction in the peripheral nerves of the limbs, the facial nerves and in the postganglionic parasympathetic fibres subserving the pupils. They also provided support for a peripheral disturbance as the underlying mechanism for the ataxia observed in patients with the Miller Fisher syndrome. The results of comparably timed, similar comprehensive neurophysiological investigation in 20 patients with the Guillain-Barre syndrome for a total period of 18 months are described in chapter 5. The results are compared with those of other studies from the literature. In chapter 6, the lack of any significant neurophysiological or brain imaging evidence for central nervous system involvement in the group of patients with the Miller Fisher syndrome is outlined. In the main discussion in chapter 7, the neurophysiological findings in the patients with the Miller Fisher syndrome are critically assessed and compared with those from the patients with the Guillain-Barre syndrome in the present study and in reports from the literature. This discussion is set in the context of concepts of peripheral and of central nervous system involvement. Clinical similarities between the Miller Fisher and the Guillain-Barre syndromes are outlined and 20 patients who appear to have overlapping features which suggest a link or continuum between the two syndromes, are reviewed from the literature. (Abstract shortened by ProQuest.).

Item Type: Thesis (MD)
Qualification Level: Doctoral
Keywords: Medicine, Pathology
Date of Award: 1987
Depositing User: Enlighten Team
Unique ID: glathesis:1987-77843
Copyright: Copyright of this thesis is held by the author.
Date Deposited: 14 Jan 2020 11:53
Last Modified: 14 Jan 2020 11:53

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