Ramsay Hunt syndrome Trivia | 55 facts about the neurological syndrome

Ramsay Hunt syndrome is the name of three different neurological syndromes. Justin Bieber recently revealed he has the syndrome.

So let’s find out some trivia and facts about the different syndromes.

1. Three different neurological syndromes carry the name of Ramsay Hunt syndrome
2. Their only connection is that they were all first documented by the famous neurologist James Ramsay Hunt (1872–1937)
3. Ramsay Hunt syndrome type 1, also called Ramsay Hunt cerebellar syndrome, is a rare form of cerebellar degeneration which involves myoclonic epilepsy, progressive ataxia, tremor, and a dementing process
4. It has also been alternatively called dyssynergia cerebellaris myoclonica, dyssynergia cerebellaris progressiva, dentatorubral degeneration, or Ramsay Hunt cerebellar syndrome
5. Onset of symptoms usually occurs in early adulthood and is characterized by intention tremor, progressive ataxia, convulsions, and myoclonic epileptic jerks
6. Tremors usually affect one extremity, primarily the upper limb, and eventually involve the entire voluntary motor system
7. Overall, the lower extremity is usually disturbed less often than the upper extremity
8. Additional features of the syndrome include: an unsteady gait, seizures, muscular hypotonia, reduced muscular coordination, asthenia, adiadochokinesia and errors with estimating range, direction, and force of voluntary movements
9. Mental deterioration can, rarely, occur
10. Ramsay Hunt syndrome type 1 is caused by the impairment of a regulatory mechanism between cerebellar and brainstem nuclei
11. It has been associated with a wide range of diseases, including Lafora disease, dentatorubropallidoluysian atrophy, and celiac disease
12. Treatment of Ramsay Hunt Syndrome Type 1 is specific to individual symptoms
13. Myoclonus and seizures may be treated with drugs like valproate
14. Some have described this condition as difficult to characterize
15. It is named for James Ramsay Hunt who first described a form of progressive cerebellar dyssynergia associated with myoclonic epilepsy in 1921
16. Ramsay Hunt syndrome type 2 is the reactivation of herpes zoster in the geniculate ganglion
17. It is sometimes called herpes zoster oticus and has variable presentation which may include a lower motor neuron lesion of the facial nerve, deafness, vertigo, and pain
18. A triad of ipsilateral facial paralysis, ear pain, and vesicles on the face, on the ear, or in the ear is the typical presentation
19. Ramsay Hunt syndrome type 2, also known as RHS and herpes zoster oticus, is a disorder that is caused by the reactivation of varicella zoster virus in the geniculate ganglion, a nerve cell bundle of the facial nerve
20. Ramsay Hunt syndrome type 2 typically presents with inability to move many facial muscles, pain in the ear, taste loss on the front of the tongue, dry eyes and mouth, and a vesicular rash
21. Less than 1% of varicella zoster infections involve the facial nerve and result in RHS
22. Early symptoms include intense pain in one ear, the jaw on one side or the neck on one side which may precede the acute facial paralysis by a week or more
23. Ramsay Hunt Syndrome Type 2 refers to shingles of the geniculate ganglion, a portion of the facial nerves
24. After the initial infection known as chickenpox, varicella zoster virus lies dormant in nerve cells in the body, where it is kept in check by the immune system
25. If the immune system is suppressed in any way (for example during an illness, while experiencing stress or undergoing chemotherapy), the virus travels to the end of the nerve cell
26. If the nerve cells affected lie within the facial nerves, it causes the symptoms described above and is referred to as Ramsay Hunt Syndrome Type 2 or simply RHS
27. Ramsay Hunt Syndrome Type 2 is estimated to account for 12% of all facial nerve paralysis
28. It occurs in both immunocompetent and immunocompromised individuals with immunocompromised patients often having more severe disease presentation
29. RHS may occur in any age group with cases reported in patients ranging in age from 3 months to 82 years
30. The affected ganglion is responsible for the movements of facial muscles, the touch sensation of a part of ear and ear canal, the taste function of the frontal two-thirds of the tongue, and the moisturization of the eyes and the mouth
31. The syndrome specifically refers to the combination of this entity with weakness of the muscles activated by the facial nerve
32. In isolation, the latter is called Bell’s palsy
33. However, as with shingles, the lack of lesions does not definitely exclude the existence of a herpes infection
34. Even before the eruption of vesicles, varicella zoster virus can be detected from the skin of the ear
35. Ramsay Hunt Syndrome Type 2 can be diagnosed based on clinical features
36. However, in ambiguous cases,PCR or direct immunofluorescent assay of vesicular fluid can help with the diagnosis
37. Laboratory studies such as WBC count, ESR and electrolytes can distinguish infectious versus inflammatory etiologies
38. On a physical exam, look for vesicular exanthema on the external auditory canal, concha and or pinna
39. Dry eyes with possible lower cornea epithelium damage due to incomplete closure of eyelids
40. It is possible to have Ramsay Hunt Syndrome Type 2 without an external rash present
41. This is called “RHS sine herpete” and may occur in up to 30% of patients
42. Ramsay Hunt Syndrome type 2 can usually be diagnosed based on clinical features
43. However, for suspected cases with unclear presentation, varicella zoster virus can be isolated from vesicle fluid
44. Tear culture PCR can have positive varicella zoster virus
45. However, 25-35% of patients with Bell’s palsy can have false positive varicella zoster virus detected in tears
46. If central nervous system complications such as meningitis, ventriculitis or meningoencephalitis are suspected, prompt lumbar puncture with spinal fluid analysis and imaging (CT head) are recommended
47. An MRI with contrast may be ordered if the diagnosis is ambiguous so as to rule out other causes of acute facial paralysis such as a stroke, Lyme Disease, Multiple Sclerosis, cancer or tumors
48. This test is most commonly ordered if the patient presents atypically with RHS sine herpete
49. Overall between 30% and 70% of Ramsay Hunt Syndrome type 2 patients recover most functionality depending on early diagnosis and treatment
50. With chances of recovery dropping to 50% if treatment is delayed beyond 72 hours
51. Some patients report an increased sensitivity to barometric pressure with changes in weather patterns causing pain on the affected side of the face.
52. Ramsay Hunt syndrome type 3 is a less commonly referenced condition
53. It is an occupationally induced neuropathy of the deep palmar branch of the ulnar nerve
54. It is also called Hunt’s disease or artisan’s palsy
55. It is different from Ramsay Hunt syndrome type 1 and Ramsay Hunt syndrome type 2, which are completely unrelated other than having been described by the same researcher, James Ramsay Hunt