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Geniculate neuralgia | |
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Specialty | Neurology |
Geniculate ganglionitis or geniculate neuralgia (GN), also called nervus intermedius neuralgia, Ramsay Hunt syndrome, or Hunt's neuralgia, is a rare disorder characterized by severe paroxysmal neuralgic pain deep in the ear, [1] that may spread to the ear canal, outer ear, mastoid or eye regions. GN may also occur in combination with trigeminal or glossopharyngeal neuralgia. [2]
The pain of GN is sharp, shooting or burning and can last for hours. Painful attacks can be triggered by cold, noise, swallowing or touch, but triggers are usually unique to the sufferer. Other related symptoms that may be experienced include increased salivation, bitter taste, tinnitus and vertigo.
GN is rare, and only limited data is available regarding the incidence, prevalence, and risk factors associated with this condition. Middle-aged adults, however, seem to be predominantly affected, women more than men. [3]
GN may be caused by compression of somatic sensory branch of cranial nerve VII which goes through the nervus intermedius. In sufferers of GN, signals sent along these nerves are altered and interpreted by the geniculate ganglion (a structure in the brain) as GN pain. GN may also develop following herpes zoster oticus (Ramsay Hunt syndrome), where cold sores occur on the ear drum or ear. This may also be associated with facial paresis (weakness), tinnitus, vertigo and deafness. Disorders of lacrimation, salivation and/or taste sometimes accompany the pain. There is a common association with herpes zoster. [4]
Diagnostic criteria: [4]
A. Pain paroxysms of intermittent occurrence, lasting for seconds or minutes, in the depth of the ear
B. Presence of a trigger area in the posterior wall of the auditory canal
C. Not attributed to another disorder
A trial of the anticonvulsant drug carbamazepine is common for patients diagnosed with GN. [5] For patients who do not tolerate or respond to carbamazepine, alternative drugs include oxcarbazepine, gabapentin, phenytoin, lamotrigine, and baclofen. In addition, tricyclics (e.g., amitriptyline) and pregabalin are useful in other types of neuropathic pain.
A variety of surgeries have been performed including microvascular decompression (MVD) of the fifth, ninth, and tenth nerves; [2] [6] as well as partial cutting of the nervus intermedius, [7] geniculate ganglion, chorda tympani and/or the ninth and tenth cranial nerves.
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.
The facial nerve is the seventh cranial nerve, or simply CN VII. It emerges from the pons of the brainstem, controls the muscles of facial expression, and functions in the conveyance of taste sensations from the anterior two-thirds of the tongue. The nerves typically travels from the pons through the facial canal in the temporal bone and exits the skull at the stylomastoid foramen. It arises from the brainstem from an area posterior to the cranial nerve VI and anterior to cranial nerve VIII.
Trigeminal neuralgia is a long-term pain disorder that affects the trigeminal nerve, the nerve responsible for sensation in the face and motor functions such as biting and chewing. It is a form of neuropathic pain. There are two main types: typical and atypical trigeminal neuralgia. The typical form results in episodes of severe, sudden, shock-like pain in one side of the face that lasts for seconds to a few minutes. Groups of these episodes can occur over a few hours. The atypical form results in a constant burning pain that is less severe. Episodes may be triggered by any touch to the face. Both forms may occur in the same person. It is regarded to be one of the most painful disorders known to medicine, and often results in depression.
Shingles, also known as zoster or herpes zoster, is a viral disease characterized by a painful skin rash with blisters in a localized area. Typically the rash occurs in a single, wide stripe either on the left or right side of the body or face. Two to four days before the rash occurs there may be tingling or local pain in the area. Otherwise, there are typically few symptoms though some patients may have fever or headache, or feel tired. The rash usually heals within two to four weeks; however, some people develop ongoing nerve pain which can last for months or years, a condition called postherpetic neuralgia (PHN). In those with poor immune function the rash may occur widely. If the rash involves the eye, vision loss may occur.
Facial nerve paralysis is a common problem that involves the paralysis of any structures innervated by the facial nerve. The pathway of the facial nerve is long and relatively convoluted, so there are a number of causes that may result in facial nerve paralysis. The most common is Bell's palsy, a disease of unknown cause that may only be diagnosed by exclusion of identifiable serious causes.
Neuralgia is pain in the distribution of a nerve or nerves, as in intercostal neuralgia, trigeminal neuralgia, and glossopharyngeal neuralgia.
Ear pain, also known as earache, is pain in the ear. Primary ear pain is pain that originates from the ear. Secondary ear pain is a type of referred pain, meaning that the source of the pain differs from the location where the pain is felt.
Occipital neuralgia (ON) is a painful condition affecting the posterior head in the distributions of the greater occipital nerve (GON), lesser occipital nerve (LON), third occipital nerve (TON), or a combination of the three. It is paroxysmal, lasting from seconds to minutes, and often consists of lancinating pain that directly results from the pathology of one of these nerves. It is paramount that physicians understand the differential diagnosis for this condition and specific diagnostic criteria. There are multiple treatment modalities, several of which have well-established efficacy in treating this condition.
Microvascular decompression (MVD), also known as the Jannetta procedure, is a neurosurgical procedure used to treat trigeminal neuralgia a pain syndrome characterized by severe episodes of intense facial pain, and hemifacial spasm. The procedure is also used experimentally to treat tinnitus and vertigo caused by vascular compression on the vestibulocochlear nerve.
The geniculate ganglion is a collection of pseudounipolar sensory neurons of the facial nerve located in the facial canal of the head. It receives fibers from the facial nerve. It sends fibers that supply the lacrimal glands, submandibular glands, sublingual glands, tongue, palate, pharynx, external auditory meatus, stapedius muscle, posterior belly of the digastric muscle, stylohyoid muscle, and muscles of facial expression.
James Ramsay Hunt was an American neurologist.
A neurectomy is a type of nerve block involving the severing or removal of a nerve. This surgery is performed in rare cases of severe chronic pain where no other treatments have been successful, and for other conditions such as vertigo, involuntary twitching and excessive blushing or sweating.
The intermediate nerve, nervus intermedius, nerve of Wrisberg or Glossopalatine nerve, is the part of the facial nerve located between the motor component of the facial nerve and the vestibulocochlear nerve. It contains the sensory and parasympathetic fibers of the facial nerve. Upon reaching the facial canal, it joins with the motor root of the facial nerve at the geniculate ganglion. Alex Alfieri postulates, that the intermediate nerve should be considered as a separate cranial nerve and not a part of the facial nerve.
Three different neurological syndromes carry the name of Ramsay Hunt syndrome. Their only connection is that they were all first described by the famous neurologist James Ramsay Hunt (1872–1937).
Atypical trigeminal neuralgia (ATN), or type 2 trigeminal neuralgia, is a form of trigeminal neuralgia, a disorder of the fifth cranial nerve. This form of nerve pain is difficult to diagnose, as it is rare and the symptoms overlap with several other disorders. The symptoms can occur in addition to having migraine headache, or can be mistaken for migraine alone, or dental problems such as temporomandibular joint disorder or musculoskeletal issues. ATN can have a wide range of symptoms and the pain can fluctuate in intensity from mild aching to a crushing or burning sensation, and also to the extreme pain experienced with the more common trigeminal neuralgia.
The International Classification of Headache Disorders (ICHD) is a detailed hierarchical classification of all headache-related disorders published by the International Headache Society. It is considered the official classification of headaches by the World Health Organization, and, in 1992, was incorporated into the 10th edition of their International Classification of Diseases (ICD-10). Each class of headache contains explicit diagnostic criteria—meaning that the criteria include quantities rather than vague terms like several or usually—that are based on clinical and laboratory observations.
Herpes zoster ophthalmicus (HZO), also known as ophthalmic zoster, is shingles involving the eye or the surrounding area. Common signs include a rash of the forehead with swelling of the eyelid. There may also be eye pain and redness, inflammation of the conjunctiva, cornea or uvea, and sensitivity to light. Fever and tingling of the skin and allodynia near the eye may precede the rash. Complications may include visual impairment, increased pressure within the eye, chronic pain, and stroke.
Facial nerve decompression is a type of nerve decompression surgery where abnormal compression on the facial nerve is relieved.
Laryngotracheal reconstruction is a surgical procedure that involves expanding or removing parts of the airway to widen a narrowing within it, called laryngotracheal stenosis or subglottic stenosis.
Use of these new techniques, sometimes in combination with selective section of the Vth cranial nerve, has been successful in relieving the pain of geniculate neuralgia.
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