Abstract

The objectives of this paper are: (1) to present a review of the clinical features and diagnostic criteria of a number of conditions involving paroxysmal, episodic or chronic peri- orbitalpain, (2) to relate the clinical facts with neuroanatomical findings, and (3) to argue a com- mon pathophysiology. One general difficulty in this field is that much of the neuroanatomical data are obtained from animals, and we do not know how far they apply to man. Moreover, as- suming that paroxysmal pain syndromes are found only in man, animal experiments are hardly applicable for testing pathogenic models or therapeutic strategies. It is concluded that most periorbital pain syndromes can be diagnosed on the basis of clinical information alone. However, further diagnostic procedures are called for to exclude treatable life- threatening conditions. On the basis of current knowledge, periorbital pain is suggested to be of neurogenic origin, due to a disordered interganglionic 'circuitry' - interaction between sensory and sympathetidpara- sympathetic nerves at the ganglionic level. Recent evidence for the presence of this circuitry is reviewed. This pathophysiological approach may be applicable to all paroxysmal periorbital pain syndromes. However, more clinical information is needed, especially on the rarely occurring syn- dromes, for more exact determination of the basic pathogenesis. Some support for a unitary pathophysiological approach can be found in the published reports of successful treatment - at least of migraine and cluster headache - with a narrow range of medicinal compounds (in particu- lar, ergotamine and sumatriptan). Evidence of therapeutic success in other cases is still only findings in this field and to classify the disorders involved, as in the 'Classification and ~i~~~~~- tic Criteria for Headache Disorders, Cranial Neuralgias and Facial Pain' proposed by the In- ternational Headache Society (IHS) in 1988l.

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