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It is mentioned here because a similar condition is also known in adults, as a paraneoplastic disease with ovarian, breast, gastric, and bronchogenic carcinomas and with other occult tumors In a broader survey of the pediatric opsoclonus-myoclonus syndrome, Pranzatelli and associates reported their experience with 27 cases, some with neural crest tumors, others with viral infections or hypoxic injury (intention myoclonus) In nearly all of their patients there was cerebellar ataxia and mental disorder, and 10 percent had seizures The CSF was normal The investigators have emphasized the pathogenetic heterogeneity and de ned a rare serotoninergic type (low levels of 5-hydroxytryptophan and homovanillic acid in the CSF) that responds to 5-hydroxyindole acetic acid.

Next to hypertension, anticoagulant therapy is currently the most common cause of cerebral hemorrhage. The hemorrhages that develop, though sometimes situated in the sites of predilection of

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Familial Progressive Myoclonus Five major categories of familial polymyoclonus of late childhood and adolescence have been delineated: (1) Lafora- or amyloid-body type, (2) juvenile cerebroretinal degeneration, (3) cherry-red spot myoclonus (sialidosis or neuraminidosis), (4) mitochondrial encephalopathy, and (5) a more benign degenerative disease (dyssynergia cerebellaris myoclonica of Hunt) Familial myoclonus may also be a prominent feature of two other diseases GM2 gangliosidosis and Gaucher disease which occasionally have their onset in this age period Lafora-Body Polymyoclonus with Epilepsy This disease, which is inherited as an autosomal recessive trait, was rst identi ed by Lafora in 1911 on the basis of the large basophilic cytoplasmic bodies that were found in the dentate, brainstem, and thalamic neurons These inclusions have been shown by Yokoi and colleagues to be composed of a glucose polymer (polyglycosan) that is chemically but not structurally related to glycogen.

In pure dysarthria or anarthria, there is no abnormality of the cortical language mechanisms The dysarthric patient is able to understand perfectly what is heard and, if literate, has no dif culty in reading and writing, although he may be unable to utter a single intelligible word This is the strict meaning of being inarticulate Defects in articulation may be subdivided into several types: lower motor neuron (neuromuscular); spastic (pseudobulbar); rigid (extrapyramidal); cerebellar-ataxic; and hypo- and hyperkinetic dysarthrias Lower Motor Neuron (Neuromuscular) Dysarthria, Atrophic Bulbar Paralysis This is due to weakness or paralysis of the articulatory muscles, the result of disease of the motor nuclei of the medulla and lower pons or their intramedullary or peripheral extensions (lower motor neuron paralysis) In advanced forms of this disorder, the shriveled tongue lies inert and fasciculating on the oor of the mouth, and the lips are lax and tremulous.

Possibly some of the cases of familial myoclonus epilepsy reported by Unverricht and by Lundborg were of this type, but since these authors provided no pathologic data, one cannot be sure Beginning in late childhood and adolescence (11 to 18 years) in a previously normal individual, the disease announces itself by a seizure, a burst of myoclonic jerks, or both In about half the cases there are focal (often occipital) seizures The illness may at rst be mistaken for ordinary epilepsy, but within a few months it becomes evident that something far more serious is occurring The myoclonus becomes widespread and can be evoked as a startle by noise, an unexpected tactile stimulus (even the tap of a re ex hammer), and also by excitement, or certain sustained motor activities An evoked train of myoclonic jerks may progress to a generalized seizure with loss of consciousness.

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Saliva constantly collects in the mouth because of dysphagia, and drooling is troublesome Dysphonia alteration of the voice to a rasping monotone due to vocal cord paralysis is often added As this condition evolves, speech becomes slurred and progressively less distinct There is special dif culty in the enunciation of vibratives, such as r, and as the paralysis becomes more complete, lingual and labial consonants are nally not pronounced at all In the past, bilateral paralysis of the palate, causing nasality of speech, often occurred with diphtheria and poliomyelitis, but now it occurs most often with progressive bulbar palsy, a form of motor neuron disease (page 940), and with certain other neuromuscular disorders, particularly myasthenia gravis.

As the disease advances, the myoclonus interferes increasingly with the patient s motor activities until voluntary function is seriously impaired Speech may be marred, much as it is in chorea Close examination may also reveal.

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