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More recently, Rifal and associates have reviewed the ndings in 25 cases of this disease one observed personally and 24 reported previously The dementia appears to be relatively mild, consisting of impairment of retentive memory, dysnomia, dyscalculia, and sometimes expressive dysphasia and de cits of visual integration ; this was overshadowed by the rigidity and spasticity of the limbs and the peripheral nerve disorder Nerve conduction velocities were diminished and the leg muscles were denervated Moderate degrees of generalized cerebral atrophy, multifocal areas of white matter rarefaction, and increased iron deposition in the putamina was disclosed by MRI The nding of polyglycosan axon inclusion in biopsied nerve con rms the diagnosis The disease has sometimes been misdiagnosed as adrenoleukodystrophy Its status is unclear to the authors, and there is no treatment

DISEASES CHARACTERIZED BY ABNORMALITIES OF POSTURE AND MOVEMENT Parkinson Disease (Paralysis Agitans)

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This common disease, known since ancient times, was rst cogently described by James Parkinson in 1817 In his words, it is characterized by involuntary tremulous motion, with lessened muscular power, in parts not in action and even when supported; with a propensity to bend the trunk forward, and to pass from a walking to a running pace, the senses and intellect being uninjured Strangely, his essay contains no reference to rigidity or to slowness of movement, and it stresses unduly the reduction in muscular power The same criticism can be leveled against the term paralysis agitans, which appeared for the rst time in 1841 in Marshall Hall s textbook Diseases and Derangements of the Nervous System The natural history of the disease is of interest As a rule, it begins between 40 and 70 years of age, with the peak age of onset in the sixth decade It is infrequent before 30 years of age, and most series contain a somewhat larger proportion of men Trauma, emotional upset, overwork, exposure to cold, rigid personality, and so on, are among many factors that have been suggested over the years as predisposing to the disease, but there is no convincing evidence to support any such claims A possible relationship to repeated cerebral trauma and to the punch-drunk syndrome (dementia pugilistica, page 863) has been particularly problematic and is unresolved despite several celebrated cases (Lees) Idiopathic Parkinson disease is observed in all countries, all ethnic groups, and all socioeconomic classes, although the incidence in African Americans is only one-quarter that in whites In Asians, the incidence is one-third to one-half that in whites The disease is frequent in North America, where there are approximately 1 million patients, constituting about 1 percent of the population over the age of 65 years The incidence in all European countries where vital statistics are kept is similar Genetic Aspects Considering its frequency, coincidence in a family on the basis of chance occurrence might be as high as 5 percent A lack of concordance of Parkinson disease in twins was at rst thought to negate the role of genetic factors, but a study of dopamine metabolism utilizing PET scanning has shown that 75 percent of asymptomatic twins of Parkinson patients had evidence of striatal dysfunction and only a small portion of dizygotic twins showed these changes (Piccini et al) These data suggest a more substantial role for an inherited trait in cases of ostensibly sporadic disease.

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