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Some syndromes of James Ramsay Hunt
  1. J M S Pearce
  1. Consultant Neurologist, Emeritus Department of Neurology, Hull Royal Infirmary, Hull, UK
  1. Correspondence to:
 Dr J M S Pearce
 304 Beverley Road, Anlaby, East Yorkshire HU10 7BG, UK; jmsp{at}freenet.co.uk

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James Ramsay Hunt (1872–1937)

James Ramsay Hunt qualified MD at the University of Pennsylvania in 1893. He was appointed Instructor at Cornell University School of Medicine from 1900–10 and worked with C L Dana, mainly on the anatomy and clinical features of the corpus striatum and cerebellum. In 1924, he became a full professor at Columbia University School of Medicine. In the First World War he rose to the rank of Lieutenant Colonel in the Army Medical Corps, serving in France as director of neuropsychiatry. He was on the faculty of the College of Physicians & Surgeons of Columbia University in 1911–13 and again from 1924 until his death. He was Consulting Neurologist and Senior Attending Neurologist at the Neurological Institute of New York from c.1914 to 1937. Despite his high ranking in Neurology, few biographical details have been recorded about his life.1 His 29 casebooks are housed in the Columbia University Health Sciences library archives and contain about 5019 cases dating from 1903 to 1937. Without doubt, he was one of the foremost neurologists of his time. His researches and writings were diverse and show originality and fine attention to detail with shrewd deductions about pathogenesis. He died on 22 July 1937 in Katonah, New York leaving two children, Alice Hunt Sokoloff, an accomplished musician and biographer, and James Ramsay Hunt Jr, a CIA intelligence officer.

Ramsay Hunt had the distinction of having at least three syndromes named after him. The best known is herpes zoster oticus. Dyssynergia cerebellaris myoclonica is the second. Third, is progressive atrophy of the globus pallidus (primary atrophy of the pallidal system), the basis of juvenile paralysis agitans of Hunt. Less well known, but of clinical importance, is his 1914 account of the clinical features produced by carotid artery occlusion, and he also provided an early description of deep palmar ulnar nerve entrapment.

RAMSAY HUNT SYNDROMES

Herpetic inflammation of the Gasserian ganglion

This Ramsay Hunt syndrome1,2 is caused by varicella-zoster virus (VZV). It is characterised by zoster around the outer ear or oropharynx, acute peripheral facial palsy, and frequently by a disorder of the auditory nerve as well.3 Facial palsy and zoster do not always appear simultaneously, and some patients develop facial palsy days before or after the eruption. VZV can even cause peripheral facial palsy without skin lesions—zoster sine herpete—diagnosed by serological assay or polymerase chain reaction in the cerebrospinal fluid. The virus can be detected even before the eruption of vesicles, from the skin of the ear.4 Ramsay Hunt reported:

Clinically the cases of geniculate herpes resolve themselves into three groups. The simplest expression of the disease is a herpes of the auricle and external auditory canal. Within this skin area is to be found the zoster zone for the geniculate ganglion. In another group of cases there is added to the aural herpes a paralysis of the facial nerve. This I explain by pressure of the inflamed ganglion or in some cases by a direct extension of the inflammation to the nerve. The most interesting, as well as the most severe, type of the disease occurs when the acoustic nerve is involved. In this form there are with herpes auricularis and facial palsy, various auditory symptoms, ranging in severity from tinnitus aurium and diminution of hearing to … acoustic involvement as seen in Ménières [sic] syndrome … the inflammation has extended to the auditory nerve …

A large study showed that complete recovery of facial palsy, but not of acoustic function, can be achieved in 75% of patients if prednisone and acyclovir are started within the first 3 days of onset of symptoms.5 Half of all patients whose treatment was delayed had complete loss of response to facial nerve stimulation.

Dyssynergia cerebellaris myoclonica

This Ramsay Hunt syndrome is a rare and nosologically uncertain disorder that he called dyssynergia cerebellaris myoclonica.6,7 It was a “well defined … progressive cerebellar disorder in association with myoclonus-epilepsy”. He noted the myoclonus-epilepsy was similar to the familial form described by Unverricht and Rabot. Symptoms included seizures, tremor and reduced muscle coordination. Myoclonus and seizures may be treated with drugs like valproate. This syndrome is caused by the impairment of a regulatory mechanism between cerebellar and brainstem nuclei and has been associated with a wide range of diseases, including Lafora disease and rarely with coeliac disease and antigliadin antibodies.7 Ramsay Hunt’s 1921 paper presents a wonderful clinical picture as well as a critical appraisal of clinical cerebellar physiology:


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This affection was characterized by generalized intention tremors, which began as a local manifestation and gradually extended to other parts of the voluntary muscular system. The extremities, and more especially the arms, showed the greatest involvement. The coarse ataxic-tremor … was only present when the muscles were in action, and ceased entirely during relaxation and rest. … associated with it a disorder of muscle tone and of the ability to measure direct and associated muscular movements, the clinical manifestations of which were dyssynergia, dysmetria, adiadokokinesis, hypotonia and asthenia. All of these symptoms … showed the existence of a fundamental disorder of cerebellar function.


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He attributed the signs to atrophy of the cerebellar efferent dentate system. Anita Harding’s view was that “the concept of the Ramsay Hunt syndrome is clinically useful as long as those using it remember that it is a syndrome, not a disease”.8

Juvenile paralysis agitans of Ramsay Hunt

Ramsay Hunt described four patients9 with progressive atrophy of the globus pallidus, two of whom were Jewish. He mentioned several earlier reports, but furnished the first detailed neuropathological study in one patient. Siehr had reported two earlier cases in 1899.10 The clinical features were similar to adult cases of Parkinson’s disease.11 Ramsay Hunt distinguished the syndrome pathologically from later onset Parkinsonism, and summarized the features:

A pure system disease characterized by progressive atrophy of the efferent motor neurons of the globus pallidus mechanism … beginning in earlier decades of life, often familial, and is characterized by progressive rigidity and tremor of the Paralysis Agitans. type and paralysis of certain automatic and associated movements … The pathological changes in progressive atrophy of the globus pallidus consist of atrophy and diminution in number of the motor cells … i.e. of the globus pallidus proper, the ganglion basale of Meynert and especially the cells of similar type scattered through the caudate nucleus and putamen. … The cells of the nucleus ruber, corpus Luysii and substantia nigra are normal, so that the pathological changes represent a primary system degeneration of the efferent motor neurons of the globus pallidus mechanism.”

Subsequently, autosomal recessive12 (including those with mutations in the Parkin gene locus 6q25.2–q27) and dominant familial parkinsonism cases have been reported. DOPA-responsive dystonia13 with diurnal variation is similarly an inherited dystonia with mild parkinsonian features, linked to chromosome 14q.

CAROTID STENOSIS

Thomas Willis was possibly the first physician to describe carotid stenosis; he observed pathologically a right carotid occlusion in an asymptomatic patient.14 Van Swieten15 in 1754 postulated embolism from the heart and great vessels, naming the carotid artery as a cause of apoplexy (that is, stroke), far ahead of his time. Interestingly, in 1905, Chiari had shown that by occluding the carotid in the neck manually, he could temporarily cause hemiplegia, aphasia or loss of consciousness.16 At the time of Ramsay Hunt’s account however, carotid stenosis was not generally recognised and certainly not clinically diagnosed. In 1914 he referred to intermittent brain claudication in patients with carotid artery stenosis long in advance of angiography,17 and prefaced his paper with the following observations:

The object of the present study is to emphasise the importance of obstructive lesions of the main arteries of the neck, in the causation of softening of the brain, and more especially to urge the routine examination of these vessels in all cases presenting cerebral symptoms of vascular origin. In other words, the writer would advocate the same attitude of mind towards this group of cases as towards intermittent claudication, gangrene, and other vascular symptoms of the extremities, and never omit a detailed examination of the main arterial stem.

Miller Fisher18 and many others later elucidated the pathogenesis and clinical features. The advent of Moniz’s arteriography, and then carotid endarterectomy, paved the way for modern surgical treatments,19,20 pioneered in 1954 by Eastcott, Pickering and Rob21 at St Mary’s Hospital, London.

PALSY OF THE DEEP BRANCH OF THE ULNAR NERVE

Finally, Ramsay Hunt provided an early description of weakness and wasting of the small muscles of the hand caused by deep palmar ulnar nerve entrapment between the abductor and short flexor muscles of the hypothenar eminence (in Guyon’s canal).22 The common causes are synovial cysts, local trauma, rheumatoid arthritis and occupational repetitive injury—so-called metal turner’s paralysis.

REFERENCES

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