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TREMOR, MYOCLONUS, FOCAL DYSTONIAS, AND TICS
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and ataxia have been combined (eg, dyssynergia cerebellaris myoclonica of Ramsay Hunt; see page 937) As mentioned above, diffuse myoclonus is a prominent and often an early feature of a unique transmissible subacute illness in adults commonly referred to as Creutzfeldt-Jakob disease and characterized by dementia, disturbances of gait and coordination and all manner of mental and visual aberrations Originally, the jerks are random in character, but late in the disease they may attain a certain rhythmicity and symmetry In addition, there is an exaggerated startle response, and violent myoclonus may be elicited by tactile, auditory, or visual stimuli in advanced stages of the disease In this condition too, there is a progressive destruction of nerve cells, mainly but not exclusively of the cerebral and cerebellar cortices, and a striking degree of gliosis In addition to parenchymal destruction, the cortical tissue shows a ne-meshed vacuolation; hence the preferable designation subacute spongiform encephalopathy Both the sporadic and rare familial forms of this disease are due to an unconventional transmissible agent, or prion (see also page 653) In yet another group of myoclonic dementias, the most prominent associated abnormality is a progressive deterioration of intellect Like the myoclonic epilepsies, the myoclonic dementias may be sporadic or familial and may affect both children and adults A rare childhood type is subacute sclerosing panencephalitis (SSPE), which is an acquired subacute or chronic (occasionally remitting) disease related in some way to previous infection with the measles virus (page 650) Another type, familial in nature and affecting infants and young children, has been described by Ford, who called it progressive poliodystrophy On a background of normal birth and development over the rst few months or years of life, there occurs a progressive psychomotor deterioration, with spastic quadriplegia, seizures, myoclonus, and blindness The fundamental pathologic alteration is a destruction of nerve cells in the cerebral and cerebellar cortices with replacement gliosis Intention or Action (Postanoxic) Myoclonus This type of myoclonus was described by Lance and Adams in 1963 in a group of patients who were recovering from hypoxic encephalopathy When the patient is relaxed, the limb and other skeletal muscles are quiet (except in the most severe cases); only seldom does the myoclonus appear during slow, smooth (ramp) movements Fast (ballistic) movements, however, especially when directed to a target, as in touching the examiner s nger, elicit a series of irregular myoclonic jerks that differ in speed and rhythmicity from intention tremor For this reason it was called intention or action myoclonus Only the limb that is moving is involved; hence it is a localized, stimulusevoked myoclonus Speech may be affected; it is fragmented by the myoclonic jerks, and a syllable or word may be almost compulsively repeated, as in palilalia Action myoclonus is almost always associated with cerebellar ataxia The pathologic anatomy has not been ascertained Lance and Adams found the irregular discharges to be transmitted via the corticospinal tracts, preceded in some cases by a discharge from the motor cortex Chadwick and coworkers postulated a reticular loop re ex mechanism Hallett and colleagues found that a cortical re ex mechanism was operative in some cases and a reticular re ex mechanism in others Whether these are two aspects of one mechanism could not be decided Barbiturates and valproic acid have been helpful in some cases Clonazepam in combination with valproate is our preference Several clinical trials and case reports have suggested that the ex-
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perimental drug piracetam, and, more persuasively, the anticonvulsant levetiracetam may be useful (Krauss et al) The use of 5hydroxytryptophan alone or in combination with tryptophan or other drugs has been recommended in the past (van Woert et al) Usually, a combination of several of these medications is required to make the patient functional
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