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Bell's or Friedreich's palsy
  1. J M S PEARCE
  1. 304 Beverley Road, Anlaby, Hull HU10 7BG, UK

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    Charles Bell made many important contributions to neurology,1 2 anatomy, and to the world of art. He is commonly remembered for his 1821 paper3 to the Royal Society, which provided a brief but unmistakable description of facial paralysis of lower motor neuron type. He clearly separated it from the palsy of upper motor neuron lesions, although this terminology was not then in use. His account of the upturning of the globe was graphic and important, and received more attention from Gowers and later Kinnier Wilson than his account of facial palsy. He gave his observations in a lecture for the Royal Society in 1826. He provided a more detailed description in 1829.4 His famous paper records a Mr Daniel Quick, gored by a bull, and several other, mostly traumatic causes:

    “ . . .a man shot with a pistol ball, which entered the ear and tore across the portio dura at its root. . . .The next instance was in a man wounded by the horn of an ox. The point of the horn entered under the angle of the jaw and came out before the ear, tearing across the portio dura. . . .The forehead of the corresponding side is without motion, the eyelids remaining open, the nostril has no motion in breathing, and the mouth is drawn to the opposite side. The muscles of the face by long disuse are degenerated, and the integuments of the wounded side of the face are become like a membrane stretched over the skull. . . .In this man the sensibility of the face is perfect. The same nerve (portio dura) has been divided in the extirpation of a tumour before the ear, and the immediate effect has been horrible distortion of the face by the prevalence of the muscles of the opposite side, but without loss of sensibility; and that distortion is unhappily increased when a pleasurable emotion should be reflected in the countenance.”

    The Bell's phenomenon.5 was clearly described for the first time in a case history in his 1830 monographThe nervous system of the human body.6 Moritz Romberg translated this influential volume into German a few years later.

    Bell described:

    “ . . .a very remarkable turning up of the cornea in an attempt to close the eyelids” and further on

    “.... the patient is not at all aware of the eye being turned up; although he can turn it up by a voluntary act, and be conscious of it at the same time . . .the cornea is still safe although the eye lid does not descend, yet the eye ascends to the eye lid; and it is wiped, cleaned and moistened by the partial performance of the act of winking.”

    The facial nerve he called “the respiratory nerve of the face. It ministered to the motions of the face which are connected with respiration.”

    “In all the exhilarating emotions, the eyebrows, eyelids, the nostrils and the angles of the mouth are raised. In the depressing passions it is the reverse.”9

    Bell subsequently corrected an earlier ambiguous remark, that the fifth and seventh cranial nerves innervated the muscles of the face:

    “the sensibility of the head and face depend upon the fifth pair of nerves . . . the portio dura of the seventh nerve is the principal muscular nerve of the face.”8

    Avicenna had much earlier described spastic, atonic, and convulsive types of facial palsy.7 Bell referred to the French neurologist, Roux, who described his own facial nerve paralysis with hyperacusis and altered taste in a letter to Professor Descot. Earlier still was a sketchy account of James Douglas in 1704. (cited by Bird8) However, it was Nikolaus Anton Friedreich (1761–1836), who comprehensively described three cases of peripheral facial paralysis in 1798; the English translation appeared in theAnnals of Medicine in 1800.8 9He was probably the grandfather of Nikolaus Friedreich of Heidelberg who elucidated hereditary ataxia.

    “A man of forty-six years . . .exposed the left side to a stream of cold air from a window. . . .In the morning after a very restless night a very painful swelling, of the size of a hazel nut, appeared in the neighbourhood of the left mastoid process . . .the malady was recognized to be rheumatic. . . .On the morning of the fifth day, our author found the muscles of the left side of the face paralyzed, and the mouth and the nose drawn towards the right side . . .the integrity of all the senses, and of all the other muscles of the body, he could not view the evil as apoplectic, but as being local and proceeding from the rheumatism . . .”

    Various local treatments (aconite, guiac, antimonials, and blisters etc) were applied

    “for seven weeks without any amendment. . . .”

    “He employed weak and few electric shocks, but gradually stronger and more numerous . . .directed them variously through the left side of the face from that place where the nerve comes through the stylomastoid foramen. . . .At last their voluntary action returned by degrees and after electricity had been used for a month, volition had regained its full energy, and the face its natural appearance.”

    Bell deserves full credit for his account of facial palsy, although the idiopathic condition we now call Bell's palsy was not really part of his description. The variable involvement of the nerve to stapedius causing transient hyperacusis, and the chorda tympani producing impairment of taste were elaborated at a later date, but well described, inter alia, by Todd, Gowers, and Wilson.

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