Dyssynergia Cerebellaris Progressiva—a Chronic Progressive Form of Cerebellar Tremor

Hunt, J. Ramsay

doi:10.1093/brain/37.2.247

Cited by 39 publications

(4 citation statements)

References 2 publications

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“…The eponym RHS, under which many of these patients have been reported earlier by our group, is inadequate and has generated much confusion. The cases reported by Ramsay Hunt as dyssynergia cerebellaris progressiva in 1914 (28) and as DCM in 1921 (1) are heterogeneous. Three cases have myoclonus and dyssynergia without epilepsy.…”

Section: Conclusion and Nosologymentioning

confidence: 99%

The Ramsay Hunt syndrome revisited: Mediterranean myoclonus versus mitochondrial encephalomyopathy with ragged-red fibers and Baltic myoclonus

Genton¹,

Michelucci

Tassinari

et al. 2009

Acta Neurologica Scandinavica

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Among progressive myoclonus epilepsies (PME), the nosography of the Ramsay Hunt syndrome (RHS) has been much debated. The authors report on a homogeneous group of 43 patients originating from around the western Mediterranean, with a large number of northern African subjects, who were followed up for a mean period of 11.6 years. Onset is between 6 and 17 years (mean: 11.2) and the transmission appears to be recessive. The clinical features include: action myoclonus, generalized epileptic seizures, mild cerebellar signs and lack of dementia. EEG features include normal background activity, spontaneous fast generalized spike‐wave discharges, photosensitivity, lack of activation during nREM sleep and vertex/rolandic spikes during REM sleep. The prognosis is variable, even within families, but the progression seems to be slow in a majority of patients. This condition can be distinguished from mitochondrial encephalomyopathy and is less severe than Baltic myoclonus. The authors propose that this form of PME, formerly reported as RHS, be more properly described as Mediterranean myoclonus.

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Section: Conclusion and Nosologymentioning

confidence: 99%

The Ramsay Hunt syndrome revisited: Mediterranean myoclonus versus mitochondrial encephalomyopathy with ragged-red fibers and Baltic myoclonus

Genton¹,

Michelucci

Tassinari

et al. 2009

Acta Neurologica Scandinavica

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“…The second Ramsay Hunt syndrome encompasses the clinical features produced by carotid artery occlusion 2. A third Ramsay Hunt syndrome is dyssynergia cerebellaris progressiva,3 but a lack of pathological material has not allowed its adequate classification among the degenerative spinocerebellar disorders. The wide scope of Hunt's works included the first full description of deep palmar neuropathy attributed to median nerve compression between the abductor and short flexor muscles of the hypothenar eminence 4.…”

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confidence: 99%

NOSOLOGICAL ENTITIES?: Ramsay Hunt syndrome

Sweeney

2001

Journal of Neurology, Neurosurgery &Amp; Psychiatry

364

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The strict definition of the Ramsay Hunt syndrome is peripheral facial nerve palsy accompanied by an erythematous vesicular rash on the ear (zoster oticus) or in the mouth. J Ramsay Hunt, who described various clinical presentations of facial paralysis and rash, also recognised other frequent symptoms and signs such as tinnitus, hearing loss, nausea, vomiting, vertigo, and nystagmus. He explained these eighth nerve features by the close proximity of the geniculate ganglion to the vestibulocochlear nerve within the bony facial canal. Hunt's analysis of clinical variations of the syndrome now bearing his name led to his recognition of the general somatic sensory function of the facial nerve and his defining of the geniculate zone of the ear. It is now known that varicella zoster virus (VZV) causes Ramsay Hunt syndrome.Compared with Bell's palsy (facial paralysis without rash), patients with Ramsay Hunt syndrome often have more severe paralysis at onset and are less likely to recover completely. Studies suggest that treatment with prednisone and acyclovir may improve outcome, although a prospective randomised treatment trial remains to be undertaken. In the only prospective study of patients with Ramsay Hunt syndrome, 14% developed vesicles after the onset of facial weakness. Thus, Ramsay Hunt syndrome may initially be indistinguishable from Bell's palsy. Further, Bell's palsy is significantly associated with herpes simplex virus (HSV) infection. In the light of the known safety and eVectiveness of antiviral drugs against VZV or HSV, consideration should be given to early treatment of all patients with Ramsay Hunt syndrome or Bell's palsy with a 7-10 day course of famciclovir (500 mg, three times daily) or acyclovir (800 mg, five times daily), as well as oral prednisone (60 mg daily for 3-5 days).Finally, some patients develop peripheral facial paralysis without ear or mouth rash, associated with either a fourfold rise in antibody to VZV or the presence of VZV DNA in auricular skin, blood mononuclear cells, middle ear fluid, or saliva. This indicates that a proportion of patients with "Bell's palsy" have Ramsay Hunt syndrome zoster sine herpete. Treatment of these patients with acyclovir and prednisone within 7 days of onset has been shown to improve the outcome of recovery from facial palsy. (J Neurol Neurosurg Psychiatry 2001;71:149-154)

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“…o.). Zudem beschrieb er die so genannte "dyssynergia cerebellaris progressiva", eine Kombination aus Myoclunusepilepsie, Intentionstremor, progressiver Ataxie und seltener einer kognitiven Beeinträchtigung, die allerdings aktuell keinen Platz in der Klassifikation von degenerativen spinozerebellären Syndromen findet [10]. Und zuletzt war Ramsay Hunt einer der Erstbeschreiber der Kompression der motorischen Partie des N. ulnaris in der Guyon'schen Loge [11].…”

Section: Therapieunclassified