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The Familial Polymyoclonias
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As stated in Chap 6, the term myoclonus is applied to many conditions that are not at all alike but share a single clinical feature a multitude of exceedingly brief, random, arrhythmic twitches of parts of muscles, entire muscles, or groups of muscles Myoclonic jerks differ from chorea by virtue of their brevity (15 to 50 ms) Notably, both phenomena are considered to be symptomatic of gray matter diseases ( polioencephalopthies ) Myoclonus or polymyoclonus may, in certain conditions, stand alone as a relatively pure syndrome In other cases, it is mixed with epilepsy or athetosis and dystonia, discussed further on Most often, myoclonus is associated with cerebellar ataxia; it is therefore being considered here, with the progressive cerebellar ataxias The many acquired forms of polymyoclonus, such as subacute sclerosing panencephalitis, have been mentioned in Chap 6 In this chapter we are concerned only with those of known or presumed metabolic origin Myoclonic Encephalopathy of Infants (Infantile OpsoclonusMyoclonus Syndrome) Under this title Kinsbourne originally described a form of widespread, continuous myoclonus (except during deep sleep) affecting male and female infants whose development had been normal until the onset of the disease at the age of 9 to 20 months The myoclonus evolves over a week or less, affects all the muscles of the body, and interferes seriously with all the natural muscular activities of the child The eyes are notably affected by rapid (up to eight per second), irregular conjugate
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movements ( dancing eyes of an opsoclonic type) The child is irritable and speech may cease All laboratory tests are normal Treatment Adrenocorticotropic hormone and dexamethasone, the latter in doses of 15 to 40 mg/day, suppress the myoclonus and permit developmental progress Some patients have recovered from the myoclonus but have been left mentally slow and mildly ataxic Others have required corticosteroid therapy for 5 to 10 years, with relapse whenever it was discontinued Ordinary anticonvulsants seem to have no effect The pathology has not been determined A similar syndrome has been observed in conjunction with neuroblastoma in children and as a transient illness of unknown cause (probably viral or postinfectious) in young adults (Baringer et al; see page 641) 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 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 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 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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an alteration in muscle tone and a slight degree of cerebellar ataxia At this time, or even before the onset of myoclonus and seizures, the patient may experience visual hallucinations or exhibit irritability, odd traits of character, uninhibited or impulsive behavior, and, ultimately, progressive failure in all cognitive functions Deafness has been an early sign in a few cases Rigidity or hypotonia, impaired tendon re exes, acrocyanosis, and rarely corticospinal tract signs are late ndings Finally the patient becomes cachectic and bedfast and succumbs to intercurrent infection Most do not survive beyond their 25th birthday Nonetheless there are isolated reports of Lafora-body disease in which symptoms began as late as 40 years, with death as late as 50 years These late cases may constitute a separate genetic type No abnormalities of the blood, urine, or CSF have been detected The EEG shows diffuse slow waves and spikes as well as bursts of focal or multifocal discharges Altered hepatocytes with homogeneous PAS-positive bodies that displace the nuclei have been observed in both the presymptomatic and symptomatic stages of the disease These inclusions have been seen in skin and liver biopsies, even though liver function tests were normal Neuropathologic examinations have shown a slight loss of granule and Purkinje cells and loss of neurons in the dentate nuclei, inner segment of globus pallidus, and cerebral cortex in addition to the Lafora bodies The latter may also be seen in the retina, cerebral cortex, myocardium, and striated muscles Anticonvulsant drugs, especially methsuximide and valproic acid, help in the control of the seizures but have no effect on the basic process Polyglycosan Body Disease (See also page 914) This is another closely related disease, the features of which have been reviewed by Robitaille and coworkers, in which glycosamine bodies are found in the central and peripheral nervous system The clinical syndrome includes dementia, chorea, and amyotrophy with or without sensory loss in the limbs Similar bodies are found in the cells of the liver and heart This is a progressive disease usually beginning in adult life It has been attributed to the accumulation of polymers of glucose Diagnosis is con rmed by the nding of the polyglycosan bodies in the axons of peripheral nerves or liver cells The causative mutation in most cases affects the gene for glycogen branching enzyme Juvenile Ceroid Lipofuscinosis (Cerebroretinal Degeneration) As stated earlier, this is one of the most variable forms of the lipidoses The salient clinical features of the later-onset types are severe myoclonus, seizures, and visual loss In the juvenile type, the rst lesions are seen in the maculae; they appear as yellow-gray areas of degeneration and stand in contrast to the cherry-red spot and the encircling white ring of Tay-Sachs disease At rst, the particles of retinal pigment are ne and dust-like; later they aggregate to resemble more the bone-corpuscular shapes of retinitis pigmentosa The liver and spleen are not enlarged and there are no osseous changes The usual development of these and other manifestations of the disease was outlined by Sjogren, who studied a large number of the late infantile and juvenile types of cases in Sweden He divided the illness into ve stages: 1 2 Visual impairment, sometimes preceding retinal changes by months After approximately 2 years, the onset of generalized seizures and myoclonus, often with irritability, poor control of emotions, and stuttering, jerky speech Gradual intellectual deterioration (poor memory, reduced mental activity, inattentiveness) By this stage the move-
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