Ramsay Hunt's atrophy

Also known as:

Hunt’s atrophy of the hand

Description:

An obsolete term for wasting of the small muscles of the hands without sensory loss.

Ramsay Hunt's paralysis

Also known as:

Hunt's corpus striatum-juvenile parkinsonism syndrome

Hunt’s paralysis

Hunt's syndrome II

Willige-Hunt syndrome

Synonyms:

Corpus striatum syndrome, juvenile paralysis agitans, pallidal atrophy, pallidopyramidal disease, pallidopyramidal paralysis agitans, paralysis agitans juvenilis.

Description:

A disturbance with symptoms resembling those of the adult form of parkinsonism, but less intense than in Parkinson’s disease. It is characterised by tremor, bradykinesia, dysarthria, rigidity, fixed facies and a tendency to faint. Mental functions are intact and there are no reflex changes. It belongs to the groups of extrapyramidal disturbances and is caused by degeneration of the globus pallidus. Both sexes affected, onset between the ages of 10 and 30 years. Inheritance is autosomal dominant.

Ramsay Hunt's syndrome I

Also known as:

Hunt’s ataxia

Hunt’s syndrome I

Ramsay Hunt’s syndrome II

Synonyms:

Dentate cerebellar ataxia; dentatorubral atrophy, dyssynergia cerebellaris myoclonica, dyssynergia cerebellaris progressiva, dyssynergia cerebellaris progressiva sive myoclonica, primary dentatum atrophy.

Description:

A rare form of progressive cerebellar dyssynergia mainly characterised by intention tremor and often associated with convulsions and myoclonic epileptic jerks. Tremor usually begins locally in one extremity and spreads gradually, eventually involving the entire voluntary motor system. Legs are disturbed less often than arms Other features include unsteady gait, errors in estimating the range, direction, and force of voluntary movements, muscular hypotonia, asthenia, and adiadochokinesia are associated. The cause is uncertain, it may be due to a degeneration of the olivodentatorubral system. Onset usually in early adulthood, the average is 30 years. Mental deterioration occurs but is rare. It has been classified under the spinocerebellar degenerations. Some of the cases are due to mitochondrial abnormalities. Autosomal dominant inheritance with reduced penetrance suggested.

Ramsay Hunt in 1921 described a form of progressive cerebellar dyssynergia associated with myoclonic epilepsy. Characteristic features included generalized intention tremors that began as local manifestation in the extremities, especially the arms, and gradually extended to other parts of the voluntary muscular system. In addition to the coarse ataxic tremor that was present only when the muscles were in action and ceased during rest, severe involvement of the finer coordinated movements of the extremities was also present. Associated clinical features, combined with the myoclonus, and asthenia. Hunt also observed this syndrome in association with Friedreich's ataxia. Pathologic study of one case led him to consider that the symptoms were due to primary atrophy of the efferent dentate system of the cerebellum.

Ramsay Hunt's syndrome II

Also known as:

Ramsay Hunt’s syndrome

Description:

Unilateral herpes zoster infection of the geniculate ganglion (respectively n. Intermedius) with lesions of the external ear and oral mucosa. The symptoms include facial paralysis, severe pain in the ear with a bloody serous discharge due to vesicles on the tympanic membrane. The face is paralysed on the affected side, and there is loss of sense of taste in the anterior two-thirds of the tongue on the affected side. Other features include decreased salivation, hoarseness, tinnitus, decreased lacrimation, hearing disorder, and vertigo. Rare in children but seen commonly in the elderly population, with equal distribution between men and women. It is not contagious.

It is a common complication of shingles. Shingles is an infection caused by the varicella-zoster virus, which is the virus that causes chickenpox. Shingles occurs in people who have had chickenpox and represents a reactivation of the dormant varicella-zoster virus. Ramsay Hunt syndrome II is an acquired paralysis of the face (Bells Palsy) specifically caused by a varicella-zoster virus (VSV) infection of the facial nerve.

Ramsay Hunt in 1907 first described this combination of symptoms as a syndrome. He separated between 4 forms:

1. herpes zoster without neuralgic signs
2. herpes zoster oticus with facial paresis
3. herpes zoster oticus with facial paresis and hearing symptoms
4. Ramsay Hunt syndrome - auricular herpes zoster syndrome

Ramsay Hunt's syndrome III

Also known as:

Hunt's disease

Synonyms:

Artisans palsy, metal turner's paralysis, occupational neuropathy, occupation neuritis of the deep palmar branch of the ulnar nerve, occupation neurosis, artisan’s palsy, metal turner’s paralysis, striocerebellar tremor.

Description:

Occupational compression neuritis of the deep palmar branch of the ulnar nerve (ramus profundus n. Ulnaris).

Ramsay Hunt's zone

Description:

A delimitated skin area supplied by the ganglion geniculi of the N. Intermedius. It comprises the external meatus, the eardrum, as well as parts of the ear muscle.