Parkinsonism In Young Adults

The onset of parkinsonism under the age of 40 is usually called young onset parkinsonism. When symptoms begin under the age of 20, the term juvenile parkinsonism is sometimes used (111). Under the age of 20, parkinsonism typically occurs as a component of a more widespread degenerative disorder. However, parkin mutations have been described in some young patients (2). There is a significant overlap in young patients with dystonia and parkinsonism. Patients with young onset parkinsonism manifest...

Gender And Parkinsonism

The available evidence indicates that men have a slightly higher risk of parkinson-ism than women, with the exception of drug-induced parkinsonism (59,60). According to a meta-analysis, relative risk of parkinsonism for men compared to women is 1.5 (61). Suggested reasons for this risk included differential exposure to external risk factors, an X-linked genetic factor, and mitochondrial dysfunction. The lifetime risk of parkinsonism is greater in men than in women (4.4 vs. 3.7 ) (32). In older...

Introduction

Nearly 90 of individuals with Parkinson's disease (PD) develop voice and speech disorders during the course of their disease (1,2). These disorders are characterized by reduced voice volume (hypophonia) a breathy, hoarse, or harsh voice quality (dysphonia) imprecise consonant and vowel articulation due to reduced range of articulatory movements (hypokinetic articulation) and a tendency of these movements to decay and or accelerate toward the end of a sentence reduced voice pitch inflections...

References

Introduction to the pharmacology of CNS. In Katzung BG, ed. Basic and Clinical Pharmacology. Stanford Appleton and Lange, 2000 351-363. 2. Hare ML. Tyramine oxidase. A new enzyme system in liver. Biochem J 1928 22 968-979. 3. Pletscher A. The discovery of antidepressants A winding path. Experientia 1991 47 4-8. 4. Zeller EA, Barsky J. In vivo inhibition of liver and brain monoamine oxidase by 1-isonicotinyl-2-isopropyl hydrazine. Proc Soc Exp Biol Med 1952 81 459-461. 5. Loomer HP,...

Resonance Problems in Parkinsons Disease

Resonance problems are not common in PD, but when they are present, the voice often sounds like a foghorn. The acoustic and physiologic nature of this phenomenon is not clear, and perceptually, it is difficult to determine whether the voice is hypernasal or hyponasal. Aerodynamic and kinematic studies suggest that velopharyngeal movements may be reduced in some of these individuals (22,52,56). Abnormal tongue posture may also contribute to the resonance in parkinsonian speech. The abnormal...

Does Tolerance Develop to Levodopa

The lay literature is replete with information suggesting that levodopa loses its effect after about five years. This leads to some trepidation on the part of the patient and physician in initiating therapy. If that were the case, it would indicate that tolerance is a possible concern and would argue for delaying treatment. It is conceivable that, when all nigrostriatal cells are depleted, levodopa would lose all effectiveness since these are the cells that convert levodopa and release...

Is Levodopa Toxic

The notion that levodopa may be toxic to dopaminergic neurons leading to more rapid nigral degeneration has been a controversy for 25 years. It is based on a body of evidence that suggests that oxyradicals play an important role in the pathogenesis of cell death in PD (87). Evidence includes decreased glutathione, increased Fe2+, increased malondialdehyde, and decreased mitochondrial complex I activity in the substantia nigra (SN) (77). These changes appear to lead to apoptotic mechanisms of...

Hypertensive Crisis

Tyramine is a sympathomimetic amine normally metabolized by gut MAO-A. When tyramine rich foods such as red wines, pickled herring, or aged cheeses are ingested by someone taking an irreversible MAO-A inhibitor (clorgyline) or nonselective MAO inhibitor (e.g., phenelzine, tranylcypromine), the tyramine is absorbed rather than metabolized, causing norepinephrine release and potentially hypertensive crisis (26). This is known as the cheese effect. Levodopa is contraindicated with MAO-A or...

Corticobasal Degeneration

Corticobasal degeneration (CBD) is only rarely mistaken for PD due to characteristic focal cortical signs that are the clinical hallmark of this disorder. Common clinical presentations include progressive asymmetrical rigidity and apraxia, progressive aphasia, and progressive frontal lobe dementia (43). Most cases also have some degree of parkinsonism, with bradykinesia, rigidity, and dystonia more common than tremor. Given the prominent cortical findings on clinical evaluations, it is not...

Therapy Evaluation

Evaluation of the PD patient is focused on determining the patient's functional limitations. Range of motion, strength and balance deficits, postural deviations, general mobility, and level of conditioning are all assessed to determine their contribution to the patient's overall activity level. The evaluation consists of three parts the history, physical examination, and the functional assessment. This information is then utilized to determine a plan of care (11). The first component of both...

Info

International Standard Book Number-10 0-8493-7621-1 (Hardcover) International Standard Book Number-13 978-0-8493-7621-4 (Hardcover) This book contains information obtained from authentic and highly regarded sources. Reprinted material is quoted with permission, and sources are indicated. A wide variety of references are listed. Reasonable efforts have been made to publish reliable data and information, but the author and the publisher cannot assume responsibility for the validity of all...

Multiple System Atrophy

Multiple System Atrophy

MSA refers to a neurodegenerative disease characterized by parkinsonism, cerebellar ataxia, and orthostatic hypotension 31 . There is no known genetic risk factor or genetic locus in MSA. The MSA brain shows varying degrees of atrophy of the cerebellum, cerebellar peduncles, pons and medulla, as well as atrophy and discoloration of the posterolateral putamen and pigment loss in the substantia nigra. The histopathological findings include neuronal loss, gliosis, and microvacuolation, involving...

Levodopa Challenge Test

It can be difficult to accurately differentiate PD from other forms of parkinsonism, especially during the early stages of disease. Levodopa administration can be used for diagnostic purposes, as PD patients respond more frequently and robustly to levodopa compared with other forms of parkinsonism. Clarke and Davies 145 reviewed 13 studies that examined whether an acute levodopa or apomorphine challenge test could aid in the diagnosis of PD. Four studies examined de novo patients and nine...

Globus Pallidus Internal Segment Suppression Theory

Parkinsons Globus Pallidus

This theory posits that the loss of SNpc dopamine neurons causes decreased activity in the striatal neurons of the direct pathway. This results in a reduction of inhibition of GPi neurons, which in turn results in increased inhibition of the VL thalamus and a reduction of excitation of the MC and SMA, thus providing an explanation of loss and slowing of movements Fig. 1 . Loss of SNpc dopaminergic drive to striatal neurons of the indirect pathway results in decreased inhibition of these...

Sweating Dysfunction

Hyperhidrosis is a problem in some patients with PD, and when severe, this symptom can be severely disabling. Little is known about the problem due to a paucity of careful studies. A small study 71 demonstrated that PD patients generate more sweat when exposed to heat than control patients and that excessive sweating increases with disease severity. Swinn et al. 72 recruited 77 consecutive PD patients and 40 controls for a study of sweating. The authors designed their own questionnaire to...

Ronald F Pfeiffer

Department of Neurology, University of Tennessee Health Science Center, Memphis, Tennessee, U.S.A. The introduction of levodopa therapy for Parkinson's disease PD , initially by Birkmayer et al. 1 in 1961, by Barbeau et al. 2 in 1962, and in its ultimately successful form by Cotzias et al. 3 in 1967, still represents the defining landmark in the treatment of PD. This dramatic advance was preceded by methodical basic laboratory research in the late 1950s and early 1960s, which formed a...

Historical And Literary Precedents

Boissier Sauvages

FIGURE 1 Franciscus de le B e 1614-1672 . Also known as Sylvius de le B e, and Francis-cus Sylvius, this early physician was a Professor of Leiden and a celebrated anatomist. In his medical writings, he also described tremors and he may be among the very earliest writers on involuntary movement disorders 2 . FIGURE 1 Franciscus de le B e 1614-1672 . Also known as Sylvius de le B e, and Francis-cus Sylvius, this early physician was a Professor of Leiden and a celebrated anatomist. In his medical...

Manganese

Manganese Mn was first recognized as a neurotoxin in the 19th century with the report of four manganese ore crushers, developing a syndrome of a lower extremity predominant muscular weakness, festination, postural instability, facial masking, hypophonia, and sialorrhea 40 . The syndrome was more clearly delineated by Rodier 35 in 1955 when he described a group of Moroccan manganese miners with a neurologic illness, characterized by parkinsonism, gait disorder, dystonia, psychosis, and emotional...

Familial Parkinsonism

While most parkinsonian disorders are sporadic, rare familial forms have been described and mutations have been found or genetic linkage analyses have suggested a strong genetic factor in their etiology 58 . Perhaps the most common cause of early onset familial PD is autosomal recessive juvenile PD ARJP . The clinical features are somewhat atypical in that dystonia is common in ARJP 59 . The pathology of ARJP is based upon only a few autopsy reports. Initial studies emphasized severe neuronal...

Dementia Pugilistica

An akinetic-rigid syndrome with dysarthria and dementia is sometimes a long-term outcome of repeated closed-head trauma, as seen in professional boxers. The pathology on gross examination, other than lesions that can be attributed to trauma, for example, subdural membranes and cortical contusions, is nonspecific. The substantia nigra may also show pigment loss. Microscopically, there are NFTs similar to those in AD in the brainstem monoaminergic nuclei, cortex and hippocampus and some cases...

Mentation Behavior And Mood History

Consistent forgetfulness with partial recollection of events and no other 2 Moderate memory loss, with disorientation and moderate difficulty handling complex problems. Mild but definite impairment of function at home with need of occasional prompting. 3 Severe memory loss with disorientation for time and often to place. Severe 4 Severe memory loss with orientation preserved to person only. Unable to make judgments or solve problems. Requires much help with personal care. Cannot be left...

Freezing And Other Gait Abnormalities

A slow, shuffling, narrow-based gait is one of the most characteristic features of PD 83 . The parkinsonian gait reveals certain features that overlap with the gait disturbance associated with normal pressure hydrocephalus 84,85 . In a study of 50 subjects older than 70 years, Sudarsky and Ronthal 86 established a principal cause of the gait disorder in all but seven subjects essential gait disorder . They, but not others 87 , suggested that this senile gait is related to normal pressure...

Neuropsychology Behavioral Neurology And Neuropsychiatry

Sir William Osler first used the term neuropsychology in 1913 however, neuropsychology, at least as a clinical endeavor, did not emerge as a subdiscipline of psychology until the 1940s, largely in response to demands for the assessment and rehabilitation of brain-injured soldiers in World War II 1 . The likely first published use of a clinical neuropsychological test with persons having parkinsonian syndrome is Shaskin et al.'s 2 administration of the Wechsler Bellevue Scale, an intelligence...

The Parasomnias

Rapid Eye Movement Sleep Behavior Disorder REM sleep behavior disorder RBD was first reported by Schenck et al. in 1986 and is a parasomnia, which is typically characterized by vivid and usually frightening dreams or nightmares associated with a paradoxical simple or complex movement during REM sleep when muscles are usually atonic 13,23 . RBD is thought to have a population prevalence of 0.5 . During REM sleep, patients enact their dreams, which can be vivid or unpleasant, and partners report...

Jeanmartin Charcot And The Salpetriere School

Belladonna Parkinson

Working in Paris, in the second half of the 19th century, Charcot knew of Parkinson's description and studied the disorder in the large Salpetriere hospital that housed elderly and destitute women. He identified the cardinal features of Parkinson's disease and specifically separated bradykinesia from rigidity 5,16 FIGURE 12 Jean-Martin Charcot. Working in Paris, in the second half of the 19th century, Charcot knew of Parkinson's description and studied the...