James Ramsay Hunt

Biography

• Born on February 1, 1874 in Philadelphia
• Quaker School at the Friends’ Central School of Philadelphia
• 1893 – MD, University of Pennsylvania School of Medicine
• 1893-1895 Resident physician Pennsylvania University Hospital
• 1896 Personal physician for a wealthy patient of Silas Weir Mitchell, whilst he travelled to Japan, China and India.
• 1897-1900 Studied medicine and pathology in Europe, working with Heinrich Obersteiner (Vienna), Hermann Oppenheim (Berlin), as well as Pierre Marie, Joseph Babinski and Jules-Joseph Déjerine (Paris).
• 1900 – Clinical Assistant and then Instructor at Cornell University, New York
• Died on July 22, 1937

Medical Eponyms

Ramsay Hunt syndrome I (1907)

Ramsey Hunt syndrome 1 is also called Ramsay Hunt cerebellar syndrome; Herpes zoster oticus; Herpetic inflammation of the Gasserian ganglion, a rare condition secondary to cerebellar degeneration with causes myoclonic epilepsy, progressive ataxia and tremor.

This affection was characterised by generalised 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.

James Ramsey Hunt

Ramsay Hunt syndrome type 2

Herpes zoster infection of the geniculate ganglion. A triad of ipsilateral facial paralysis, ear pain and vesicles on the face or around the ear is classic. Patients can also complain of deafness and vertigo, and just like normal shingles the pain and paralysis can occur before the vesicles making the diagnosis difficult on first presentation.

Ramsay Hunt syndrome type 3

Occupational induced neuropathy. It is caused by damage to the deep palmar branch of the ulnar nerve (in Guyon’s canal) causing weakness and wasting of the small muscles in the hand. Motor function loss includes:

• Only the intrinsic muscles of the hand are affected.
• Abduction and adduction of the fingers cannot occur (due to paralysis of the interossei).
• Movement of the 4th and 5th digits is impaired (due to paralysis of the medial two lumbricals and hypothenar muscles).
• Adduction of the thumb is impaired, and the patient will have a positive Froment’s sign (due to paralysis of adductor pollicis).

Also called metal turner paralysis due to the tools grasped in the palm. For those in a wide life crisis, long days on a motorbike can also induce this syndrome.

Ramsay Hunt paralysis (1917)

Juvenile paralysis agitans of Ramsay Hunt

Major Publications

• Hunt JR. On herpetic inflammations of the geniculate ganglion. A new syndrome and its complications. The Journal of Nervous and Mental Disease 1907;34:73–96 [Ramsay Hunt syndrome I]
• Hunt JR. Occupational neurosis of the deep palmar branch of the ulnar nerve.. A well-defined clinical type of professional palsy of the hand. The Journal of Nervous and Mental Disease 1908;35:673–89. [Ramsay Hunt syndrome III]
• Hunt JR. Dyssynergia cerebellaris progressiva: a chronic progressive form of cerebellar tremor. Brain 1914;37:247. [Ramsay Hunt syndrome II]
• Hunt JR. Hunt JR. The role of the carotid arteries in the causation of vascular lesions of the brain, with remarks on certain special features of the symptomatology. American Journal of the Medical Sciences 1914;147:704–13 [Ramsay Hunt syndrome II]
• Hunt JR. Progressive atrophy of the globus pallidus (primary atrophy of the pallidal system). A system disease of the paralysis agitans type, characterized by atrophy of the motor cells of the corpus striatum. A contribution to the functions of the corpus striatum. Brain 1917;40:58–148 [Ramsay Hunt paralysis]
• Hunt JR. Dyssynergia cerebellaris myoclonica- primary atrophy of the dentate system. A contribution to the pathology and symptomatology of the cerebellum. Brain 1922; 44(4): 490–538 [presented in abstract at the annual meeting of the American Neurological Association, June 13, 1921] [Ramsay Hunt syndrome II]

References

Biography

• Louis ED, Williams M. A biography of James Ramsay Hunt (1874-1937). J Hist Neurosci. 2003 Sep;12(3):266-75
• Louis ED. James Ramsay Hunt (1874-1937). J Neurol. 2004 Feb;251(2):240-1.

Eponymous terms

• Pearce JMS. Some syndromes of James Ramsay Hunt. Practical Neurology 2007; 7 139-139
• Compston A. Progressive atrophy of the globus pallidus (primary atrophy of the pallidal system). A system disease of the paralysis agitans type, characterized by atrophy of the motor cells of the corpus striatum. A contribution to the function of the corpus striatum. By J. Ramsay Hunt MD, New York. Brain 1917; 40: 58-148. Brain. 2017 Jun 1;140(6):1808-1813.