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degeneration The pathologic changes were found by chance in four middle-aged patients, none with a family history of similar disease, in three of whom a parkinsonian syndrome had been described clinically In one of the three, who had been examined carefully, the typical rigidity, stiffness, and akinesia had begun on one side of the body, then spread to the other, and progressed over a 5-year period, but with little or none of the characteristic tremor of idiopathic Parkinson disease A exed posture of the trunk and limbs, slowness of all movements, poor balance, mumbling speech, and a tendency to faint when standing were other elements in the clinical picture Mental function was intact, and there were no re ex changes, no suck and grasp re exes, and no cerebellar signs or involuntary movements The other two patients had been seen by competent neurologists who had made a diagnosis of Parkinson disease Some of the symptoms had been partially relieved by anticholinergic drugs There was an early-onset cerebellar ataxia in the fourth patient, later obscured by a Parkinson syndrome In each case the postmortem examination disclosed extensive loss of neurons in the zona compacta of the substantia nigra, but notably there were no Lewy bodies or neuro brillary tangles in the remaining cells Even more striking were the degenerative changes in the putamina and to a lesser extent in the caudate nuclei These structures were greatly reduced in size and had lost most of their neurons more of the small than the large ones and more on the side opposite the rst clinical symptoms The ndings were suggestive of the striatal lesions of Huntington chorea except that the cell loss was greater in the putamen than in the caudatum Secondary pallidal atrophy (mainly a loss of striatopallidal bers) was present In the fourth patient there was, in addition, an advanced olivopontocerebellar degeneration Following the original report, in 1964, many patients were recognized in whom the changes of striatonigral and olivopontocerebellar degeneration were combined and in some of whom the symptoms and signs of cerebellar ataxia actually preceded the parkinsonian manifestations Equally frequent was a predominantly extrapyramidal syndrome accompanied by autonomic insuf ciency, as described below Shy-Drager Syndrome Nearly half of the patients with striatonigral degeneration are handicapped by orthostatic hypotension, which proves at autopsy to be associated with loss of intermediolateral horn cells and of pigmented nuclei of the brainstem This combined parkinsonian and autonomic disorder, still referred to as the Shy-Drager syndrome, has been mentioned in the chapters on fainting and the autonomic nervous system (pages 326 and 463) In addition to orthostatic hypotension, symptoms of failure of autonomic control include impotence, loss of sweating, dry mouth, miosis, and urinary retention or incontinence Vocal cord palsy is an important and sometimes initial manifestation of the autonomic disorder It causes dysphonia or stridor and airway obstruction requiring tracheostomy (see page 463) A dusky discoloration of the hands that has been ascribed to poor control of cutaneous blood ow has been emphasized by Klein and colleagues, but we have not seen it in our patients It should be noted that orthostatic hypotension is also observed in up to 15 percent of patients with idiopathic Parkinson disease, sometimes exaggerated by medications, but the degree of drop in blood pressure is far greater and more consistent in patients with this form of mulitple system atrophy Multiple System Atrophy Because of the recognition that the clinical and pathologic features of sporadic striatonigral degeneration, with or without autonomic failure, and in some instances.

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Many persons throughout their lives are given to habitual movements These range from simple, highly personalized, idiosyncratic mannerisms (eg, of the lips and tongue) to repetitive actions such as snif ng, clearing the throat, protruding the chin, or blinking whenever these individuals become tense Stereotypy and irresistibility are the main identifying features of these phenomena The patient admits to making the movements and feels compelled to do so in order to relieve perceived tension Such movements can be suppressed for a short time by an effort of will, but they reappear as soon as the subject s attention is diverted In certain cases the tics become so ingrained that the person is unaware of them and seems unable to control them An interesting feature of many tics is that they correspond to coordinated acts that normally serve some purpose to the organism It is only their incessant repetition when uncalled for that marks them as habit spasms or tics The condition varies widely in its expression from a single isolated movement (eg, blinking, snif ng, throat clearing, or stretching the neck) to a complex of movements Children between 5 and 10 years of age are especially likely to develop habit spasms These consist of blinking, hitching up one shoulder, snif ng, throat clearing, jerking the head or eyes to one side, grimacing, etc If ignored, such spasms seldom persist for longer than a few weeks or months and tend to diminish if the child is provided rest and a calmer environment In adults, relief of nervous tension by sedative or tranquilizing drugs may be helpful, but the disposition to tics persists A putative relationship to streptococcal infection is discussed below When idle, adults often display a wide variety of dgeting types of movement, gestures, and mannerisms that vary in degree from one person to another They are slower and more complex than tics and spasms Laypersons refer to them as bad habits Special types of rocking, head bobbing, hand waving (in autism) or hand wringing (typical of Rett syndrome), and other movements, particularly self-stimulating movements, are disorders of motility unique to the mentally retarded These rhythmias have no known pathologic anatomy in the basal ganglia or elsewhere in the brain Apparently they represent a persistence of some of the rhythmic, repetitive movements (head banging, etc) of normal infants In some cases of impaired vision and photic epilepsy, eye rubbing or moving the ngers rhythmically across the eld of vision is observed, especially in mentally retarded children.

Gilles de la Tourette Syndrome (Tourette Syndrome)

Multiple tics snif ng, snorting, involuntary vocalization, and troublesome compulsive and aggressive impulses constitute the rarest and most severe tic syndrome, Tourette syndrome The problem begins in childhood, in boys three times more often than girls, usually as a simple tic; as the condition progresses, new tics are added to the repertoire It is the multiplicity of tics and the combination of motor and vocal tics that distinguish the Tourette syndrome from the more benign, restricted tic disorders Vocal tics, usually loud and irritating in pitch, are characteristic Some patients display repetitive and annoying motor behavior, such as jumping, squatting, or turning in a circle Other common types of repetitive behavior include the touching of other persons and repeating one s own words (palilalia) and the words or movements of others Explosive and involuntary cursing and the

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