Dyssynergia Cerebellaris Myoclonica—primary Atrophy of the Dentate System: A Contribution to the Pathology and Symptomatology of the Cerebellum

Hunt, J. Ramsay

doi:10.1093/brain/44.4.490

Cited by 181 publications

(40 citation statements)

References 3 publications

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“…The third category is a heterogeneous group of nonspecific degenerative diseases which include dyssynergia cerebellaris myoclonica of Ramsay Hunt. This syndrome has also been called dentatorubral degeneration based on the postmortem findings of the case reported by Hunt (1921). Subsequent reports of Ramsay Hunt's dentatorubral degeneration with pathological confirmation are scarce.…”

mentioning

confidence: 99%

Progressive myoclonus and epilepsy with dentatorubral degeneration: a clinicopathological study of the Ramsay Hunt syndrome.

Bird¹,

Shaw²

1978

Journal of Neurology, Neurosurgery & Psychiatry

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SUMMARY Ramsay Hunt's progressive myoclonus and epilepsy associated with dentatorubral degeneration is a rare disorder. We report a 19 year old woman with this clinical syndrome wlho also has a more mildly affected brother. Neuropathological examination of the young woman showed spinocerebellar and cerebral cortical degeneration in addition to dentatorubral involvement. The evidence suggests that this is a distinctive hereditary disorder producing neuronal degeneration at several levels in the central nervous system.Progressive myoclonic cerebellar dyssynergia with epilepsy is an uncommon clinical syndrome with multiple causes. Harriman and Millar (1955) divide progressive familial myoclonic epilepsy into three categories. The first is a relatively uniform group associated with intracellular inclusions (Lafora bodies), and the second is associated with various cerebral lipidoses. The third category is a heterogeneous group of nonspecific degenerative diseases which include dyssynergia cerebellaris myoclonica of Ramsay Hunt. This syndrome has also been called dentatorubral degeneration based on the postmortem findings of the case reported by Hunt (1921). Subsequent reports of Ramsay Hunt's dentatorubral degeneration with pathological confirmation are scarce. We have evaluated a patient who seems to belong to this variety of progressive myoclonic epilepsy. The rarity and unusual nature of the syndrome justify documentation of the clinical and pathological details of this patient. In addition, other members of her family have possibly associated neurological findings which will also be described. Clinical summaryThe patient had a normal birth and development, and was an average student. At age 15 years she developed an awkward gait, clumsy hand coordination, and deteriorating school work. She soon developed nocturnal seizure activity, and an EEG revealed irregular runs of 4-5 Hz moderate voltage activity over the occipital areas, with bilateral spike discharges in these same regions. She was placed on anticonvulsant drugs and six months later began to complain of dull frontal headache.Neurological examination at this time showed marked horizontal nystagmus on lateral gaze to either side, dysarthria, slight dysmetria on fingerto-nose and heel-to-shin testing, poor rapid alternating movements with the left hand, ataxic gait, and a fine tremor of the head and hands. Deep tendon reflexes were normal and plantar responses were downgoing. Unusually high arched feet were noted.Cerebrospinal fluid contained 11.2 g/l protein, 3.3 mmol/l (60 mg/dl) sugar, one mononuclear cell per mm3, and negative VDRL. Skull radiographs, pneumoencephalogram, and brain scan were normal. She was treated with a combination of mephobarbitone, diphenylhydantoin, and ethosuximide.Three months later deep tendon reflexes were 140

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mentioning

confidence: 99%

Progressive myoclonus and epilepsy with dentatorubral degeneration: a clinicopathological study of the Ramsay Hunt syndrome.

Bird¹,

Shaw²

1978

Journal of Neurology, Neurosurgery & Psychiatry

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“…I n cases associated w ith dyssynergia cerebellaris m yoclonica [32], degenerative changes were found in th e d e n ta te nucleus. In p a tie n ts w ith su b acu te inclusion b ody encephalitis an d m yoclonus [15], b o th th e d e n ta te an d olivary nuclei w ere in volved.…”

Section: Discussionmentioning

confidence: 97%

“…T he ran d o m form is characterized b y asynchronous, irreg u lar jerk in g of lim b m uscles m ainly. T he la tte r is th e form occurring m ost often in various encephalitides [14][15][16][17], experim entally produced m yoclonus [10-13, 30, 31] an d in degenerative diseases of th e nervous system [32][33][34][35].…”

Section: Discussionmentioning

confidence: 99%

Transient Branchio-Respiratory and Ocular Myoclonus in Encephalitis

Carmon¹,

Behar²,

Bornstein³

1963

Stereotact Funct Neurosurg

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“…In einer zweiten, im Jahre 1921 veroffentlichten Kasuistik erfuhr das Krankheitsbild eine Ausweitung, indem epileptische Anfalle und Myoklonien als dem Syndrom zugehorig beschrieben wurden. Die Moglichkeit einer Kombination mit FRIEDREICHscher Ataxie wurde bereits damals erwahnt (HUNT 1921 Vol. 1, No.…”

Section: Syndrome De Ramsay Huntunclassified

Dyssynergia Cerebellaris Myoclonica Hunt*

Tieber¹,

Schenck²

1970

Neuropediatrics

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Dyssynergia Cerebellaris Myoclonica—primary Atrophy of the Dentate System: A Contribution to the Pathology and Symptomatology of the Cerebellum

Cited by 181 publications

References 3 publications

Progressive myoclonus and epilepsy with dentatorubral degeneration: a clinicopathological study of the Ramsay Hunt syndrome.

Progressive myoclonus and epilepsy with dentatorubral degeneration: a clinicopathological study of the Ramsay Hunt syndrome.

Transient Branchio-Respiratory and Ocular Myoclonus in Encephalitis

Dyssynergia Cerebellaris Myoclonica Hunt*

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