Vascular Parkinsonism Gxv
Although the concept of vascular parkinsonism, proposed by Critchley in 1929, was almost abandoned for many years, it has once more come to the fore. The prevalence and incidence of vascular parkinsonism is variable in different series. Diagnosis of vascular parkinsonism is usually suggested by acute onset or other signs of cerebrovascular disease, but needs to be confirmed by imaging studies 72 . This is the form of parkinsonism that frequently can be diagnosed by CT, without requiring MRI to...
References 1
1. Charcot J-M. March 20, 1888 Le ons du Mardi Policlinique la Salp tri re. Paris Bureaux du Progr s M dical, 1887-1888. 2. Parkinson J. The Shaking Palsy. London Whittingham amp Rowland, 1817. 3. Charcot J-M. De la paralysie agitante, Le on 5. In Oeuvres Compl tes, vol 1. Paris Bureaux du Progr s M dical, 1892 155-189 In English On paralysis agitans. In Lectures on Diseases of the Nervous System, Sierson, G, trans. Philadelphia HC Lea, 1879 105-127. 4. Charcot J-M. Le on 21 June 12, 1888 Le...
Visuospatial Disorders In Parkinson Disease
Visuospatial abnormalities have often been reported in PD patients. Early in 1964, Proctor et al. 105 demonstrated that PD patients have difficulty in determining when a rod is vertical if they are in a darkened room. Later, this abnormality was confirmed both in patients seated in a chair that is tilted either to the right or to the left 13 , and in patients who are upright 106 . Subsequently, as we have already documented, a number of authors reported that PD is associated with...
A Conceptual Framework
Rehabilitation teams often use a model of disablement to describe the relationship from disease to disability and to clearly identify the effects of disease on an individual. A disablement model is useful in providing a conceptual framework to help focus rehabilitation efforts in an appropriate direction. A model is also helpful in establishing clear communication among the members of the rehabilitation team. The Nagi Disablement model, developed by the sociologist, Saad Nagi, is one such model...
Introduction Fmb
Parkinson's disease PD is the most common movement disorder 1 . Neuropathologically, it is defined by nerve cell loss in the substantia nigra and the presence there of Lewy bodies and Lewy neurites. Nerve cell loss and Lewy body pathology are also found in a number of other brain regions, such as the dorsal motor nucleus of the vagus, the nucleus basalis of Meynert, and some autonomic ganglia. Lewy bodies and Lewy neurites also constitute the defining neuropathological characteristics of...
References Jre
1. Litvan I, Agid Y, Calne D, et al. Clinical research criteria for the diagnosis of progressive supranuclear palsy Steele-Richardson-Olszewski syndrome Report of the NINDS-SPSP International Workshop. Neurology 1996 47 1-9. 2. Litvan I, Agid Y, Goetz C, et al. Accuracy of clinical diagnosis of corticobasal degeneration. Neurology 1997 48 119-125. 3. Hughes AJ, Daniel SE, Kilford L, Lees AJ. The accuracy of clinical diagnosis of idiopathic Parkinson's disease a clinicopathological study. J...
Corticobasal Degeneration 1
This clinicopathological entity was first described under the name corticodentonigral degeneration with neuronal achromasia 68 and early reports stressed the characteristic movement abnormalities, which often include akinetic rigidity 69 . More recently, however, a number of postmortem studies have found abnormalities of higher mental function, such as language disturbance and dementia, to be a common and sometimes predominant feature in patients with corticobasal degeneration CBD pathology...
Introduction Psv
Diseases with movement disorders may be difficult to diagnose, both at the onset when motor symptoms are mild and not sufficiently specific e.g., difficulty in manipulating objects , or at the end stage when distinctive motor signs are diluted into a severe and polymorphous clinical picture e.g., gait disorder with postural instability and cognitive decline in aged patients . Erroneous diagnoses are frequent 1,2 , even in the most common idiopathic Parkinson's disease PD 3 . Some of these...
Autonomic Function Tests
Autonomic function tests are a mandatory part of the diagnostic process and clinical follow-up in patients with MSA. Findings of severe autonomic failure early in the course of the disease make the diagnosis of MSA more likely, although the specificity in comparison to other neurodegenerative disorders is unknown in a single patient. Pathological results of autonomic function tests may account for a considerable number of symptoms in MSA patients and should prompt specific therapeutic steps to...
The Synuclein Family
Synucleins are abundant brain proteins whose physiological functions are only poorly understood. The human synuclein family consists of three members a-synuclein, p-synuclein, and y-synuclein which range from 127 to 140 amino acids in length and are 55-62 identical in sequence, with a similar domain organization Fig. 1 9-11 . The amino-terminal half of each protein is taken up by imperfect 11-amino acid repeats that bear the consensus sequence KTKEGV. Individual repeats are separated by an...
Corticobasal Degeneration Rjd
Corticobasal degeneration CBD is clinically characterized by the combination of motor with cognitive disorders. Neuropathologically, it features circumscribed parietal or frontoparietal atrophy, associated with basophilic and tau-postive inclusions in the neurons of the substantia nigra and basal ganglia. Reflecting this definite pattern of neuronal damage, neuropsychological deficits of CBD patients most commonly include deficits of executive functions and of retrieval processes in relation to...
Prevalence of PSP and MSA
Measures of the prevalence of PSP vary from 0.97 to 6.54 per 100,000 eight studies and for MSA from 2.29 to 39.3 per 100,000 five studies Table 1 . A study from Sicily 8 reported one of the highest rates of 28.6 per 100,000 but this was for unspecified parkinsonism, so this category may have included cases other than MSA and PSP. The results presented in Table 1 are simple crude rates for the whole population rather than age-standardized rates. Though some studies do report such standardized...
Clinical Examination
The 4-5 Hz tremor is most apparent when the arm is fully relaxed supported and at rest, in an armchair . It is increased by mental calculation and stress and best seen during walking. It is reduced by action and intention tricks used by the patients to hide the tremor . The classical description is the pill-rolling rhythmic alternating opposition of the thumb and forefinger. Some patients have postural tremor in particular conditions holding a phone with a different frequency 6 Hz . This is the...
Reference
1. Gibb WR, Lees AJ. The significance of the Lewy body in the diagnosis of idiopathic Parkinson's disease. Neuropathol Appl Neurobiol 1989 15 1 27-44. 2. Perl DP, Olanow CW, Calne D. Alzheimer's disease and Parkinson's disease distinct entities or extremes of a spectrum of neurodegeneration Ann Neurol 1998 44 3 Suppl 1 S19-S31. 3. Hardy J, Duff K, Hardy KG, Perez-Tur J, Hutton M. Genetic dissection of Alzheimer's disease and related dementias amyloid and its relationship to tau. Nat Neurosci...
References Kdh
1. Roy EA, Square PA. Common considerations in the study of limb, verbal, and oral apraxia. In Roy EA, ed. Neuropsychological Studies of Apraxia and Related Disorders. Amsterdam North-Holland, 1985 111-161. 2. De Renzi E. Apraxia. In Boller F, Grafman J, eds. Handbook of Neuropsychology. Amsterdam Elsevier Science , 1989 245-263. 3. Rothi LJ, Heilman KM, eds. Apraxia The Neuropsychology of Action. East Sussex Psychology Press, 1997. 4. Leiguarda R, Marsden CD. Limb apraxias higher-order...
Other Genetic Diseases Occasionally Presenting With Atypical Parkinsonism
Huntington's disease HD is an autosomal-dominantly inherited disorder, usually characterized by a hyperkinetic movement disorder, personality changes, and dementia. It is caused by the pathologic expansion of a CAG-trinucleotide repeat sequence in the gene for Huntingtin on chromosome 4 94 . The fact that particularly cases of early onset frequently present with dystonia and parkin-sonism, rather than with chorea, has long been recognized 95 . In addition, the widespread use of molecular...
Druginduced Parkinsonism And Oculogyric Crisis
Drug intoxications, especially with phenothiazines such as the butyrophenones, may produce a parkinsonian picture with slowing of saccades and an akinetic mutism picture. A distinct syndrome is oculogyric crisis, which was once a common feature of postencephalitic parkinsonism, but is now a side effect of drugs, especially neuroleptic agents 87 . Oculogyric crises may also rarely be a feature of Wilson's disease 88 , and disorders of amino acid metabolism aromatic L-amino acid decarboxylase...
Vascular Parkinsonism 1
VP, also known as arteriosclerotic parkinsonism, multi-infarct parkinsonism, and lower body parkinsonism, can result from lacunar infarctions, leukoaraiosis, and Binswanger's disease. In 1929, Critchley 2 described arteriosclerotic parkinsonism characterized by rigidity, pseudobulbar affect, dementia, incontinence, and short stepped gait in an elderly hypertensive individual with multiple ischemic insults in the basal ganglia. Zijlman et al. 3 in their report of clinico-pathological findings of...
Further Research In Neurophysiology Of Apd
There is a large amount of possibilities for research in neurophysiology of parkinsonism and of movement disorders in general. Neurophysiology offers the advantage of a good temporal resolution of events and should bring further understanding of what is wrong in the CNS that leads to motor dsyfunction. The research in clinical neurophysiology will probably continue until the pathophysi-ological mechanisms of the diseases are well understood. However, meaningful information can only be obtained...
References Xmw
1. Rebeiz J, Kolodny E, Richardson E. Corticodentatonigral degeneration with neuronal achromasia a progressive disorder of late adult life. Trans Am Neurol Assoc 1967 92 23-26. 2. Rebeiz J, Kolodny E, Richardson E. Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol 1968 18 20-33. 3. Boeve B, Lang A, Litvan I. Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia. Ann Neurol 2003 54 S15-S19. 4. Lippa C, Smith T, Fontneau...
Multiple System Atrophy Ubu
MSA has been separated into MSA-P and MSA-C according to the predominant parkinsonian or cerebellar features. The former corresponds to the old term striatonigral degeneration SND , the latter to sporadic olivopontocerebellar atrophy OPCA . The term Shy-Drager syndrome is no longer been considered useful by the Consensus Conference on MSA 11 , but a proposal to introduce the term MSA-A autonomic features-predominant MSA was recently made by Horimoto et al. 12 to indicate those MSA patients in...
Msa
Speech Oral motor programming praxis Presence of nonverbal orofacial apraxia or apraxia of speech is commonly reported. Apraxia of speech often presents concurrently with orofacial apraxia, whereas orofacial apraxia may occur singly. Dysarthria is commonly reported with primarily hypokinetic and spastic features. Dysarthria severity has not been found to correlate with disease duration. Aphasia present in approximately one third to one half of cases, of mild to moderate severity, and...
Laboratory
There are no laboratory markers for the diagnosis of PSP but ocular motor studies, electrophysi-ological studies, MRI, magnetic resonance spectroscopy, and positron emission tomography PET scans may be helpful to support the diagnosis or exclude other disorders. In general the sensitivity and specificity of these techniques in distinguishing patients with clinically equivocal or early PSP from other conditions has not been assessed. For this reason this techniques are not yet considered...
Clinical Features And Differential Diagnosis
The most important features that characterize and differentiate PSP from other disorders are presented in Table 1 and described in more detail in the subheading that follows. Postural instability manifested as nonexplained and unexpected falls or tendency to falls is the most frequent symptom presentation in PSP 7-14 . In the National Institutes of Neurological Disorders and Stroke NINDS study 10 , 96 of 24 PSP patients had gait disorder and postural instability 83 history of falls at the first...
Pet
18FDG glucose metabolism H215O blood flow 18F dopa marker of the ability of the putamen and caudate to decarboxylate exogenous levodopa and store the resultant dopamine 11C SCH23390 D1-type receptor binding 11C raclopride and 11C methylspiperone D2-type receptor binding 11C diprenorphine nonselective opioid binding 11C PK11195 marker of microglial activation 11C NM4PA and 11C-physostigmine acetylcholinesterase levels SPECT 123I P-CIT and 123I FP-CIT dopamine transporter density 123I IBZM and...
Motor Disorder
Because the results of drug treatment for the motor disorder of MSA are generally poor, other therapies are all the more important. Physiotherapy helps maintain mobility and prevent contractures, and speech therapy can improve speech and swallowing and provide communication aids. Dysphagia may require feeding via a nasogastric tube or even percutaneous endoscopic gastrostomy PEG . Occupational therapy helps to limit the handicap resulting from the patient's disabilities and should include a...
Info Vmg
Modified from Jardim et al. 26 . By permission of the American Medical Association. average age at onset of parkinsonian symptoms in SCA3 kindreds is 42 yr range, 31-57 yr 31 . In general, the average disease duration spans decades. However, as in SCA2 families, the length of survival is unknown because many affected individuals are still living 31 . Clinically, dystonia is defined as a syndrome of sustained muscle contractions causing twisting and repetitive movement or abnormal postures 33 ....
Irene Litvan md
Atypical Parkinsonian Disorders Clinical and Research Aspects, edited by Irene Litvan, 2005 Psychiatry for Neurologists, edited by Dilip V. Jeste and Joseph H. Friedman, 2005 Status Epilepticus A Clinical Perspective, edited by Frank W. Drislane, 2005 Thrombolytic Therapy for Acute Stroke, Second Edition, edited by Patrick D. Lyden, 2005 Parkinson's Disease and Nonmotor Dysfunction, edited by Ronald F. Pfeiffer and Ivan Bodis-Wollner, 2005 Movement Disorder Emergencies Diagnosis and Treatment,...
Legends For The Video Segments
Video segment 1. Two patients with PSP featuring slow and limited voluntary eye movements. The second patient shows responses of the lower facial muscles, but not of the periocular muscles, to a scratch stimulus to the thenar eminence. Video segment 2. Two patients with MSA-P showing minipolymyoclonus. Note the irregular movements, sometimes limited to one finger Video segment 3. Two patients with corticobasal degeneration. The first patient shows some features of alien limb. The second patient...
References Stc
1. Hughes AJ, Daniel SE, Blankson S, Lees AJ. A clinicopathologic study of 100 cases of Parkinson's disease. Arch Neurol 1993 50 2 140-148. 2. Wenning GK, Quinn N, Magalhaes M, Mathias C, Daniel SE. Minimal change multiple system atrophy. Mov Disord 1994 9 2 161-166. 3. Stadlan EM, Yahr MD. The pathology of parkinsonism. Proceedings of the 5th International Congress of Neuropathology 1965 100 569-571. 4. Winogrodzka A, Wagenaar RC, Bergmans P, et al. Rigidity decreases resting tremor intensity...
Marie Vidailhet Frdric Bourdain and JeanMarc Trocello
The current chapter was prepared to help clinicians examine patients with parkinsonian syndromes and to detect clinical signs and clues that should alert to the appropriate diagnosis. For the main clinical diagnosis criteria of various parkinsonian syndromes Parkinson's disease PD , multiple system atrophy MSA , progressive supranuclear palsy PSP , corticobasal degeneration CBD , and dementia with Lewy bodies DLB , , the semiology will be detailed. The validity and reliability of these criteria...
Abc 1
Fig. 8. Serial axial FLAIR MR images in a patient with features of frontotemporal dementia at age 79 A , which then evolves to include nonfluent aphasia at age 81 B and corticobasal syndrome findings at age 82 C . matter, most often in superior frontal gyrus, superior parietal gyrus, pre- and postcentral gyri, and striatum Fig. 17 . Tau oligodendroglial coiled bodies are also common Fig. 18 . While achromatic, ballooned neurons that are immunoreactive to phosphorylated neurofilament or...
Mptp Intoxication
Some intravenous drug addicts who use a synthetic heroin-like drug meperidine develop chronic parkinsonism as a result of contamination by MPTP 193 . MPTP is metabolized in the brain to 1-methyl-4-phenylpyridinium MPP 194 and is selectively transported into dopaminergic cells, which it kills by inhibiting mitochondrial function 195 . Human postmortem studies reveal severe depletion of pigmented neurons in the substantia nigra without LBs 196 . The presence of active gliosis and microglial...
Major Points
Parkinsonism may be associated with a variety of underlying pathologies. Most of the conditions that cause parkinsonism have damage of the striatonigral system as the anatomicopathological substrate. The most common causes of parkinsonism are neurodegenerative diseases, each of which has a defining pattern of neuropathology, often characterized by the abnormal accumulation of protein in the form of a cellular inclusion. For many of these conditions, recent molecular genetic findings have...
Andrew Lees
Diagnosis is a system of more or less accurate guessing in which the end point achieved is a name. These names applied to disease come to assume the importance of specific entities, whereas they are for the most part no more than insecure and temporary conceptions. Despite its imprecision, atypical parkinsonism is a useful clinical term. It unites a group of disorders linked by the predominant presence of bradykinesia, but where additional clinical features exclude Parkinson's disease PD hence...
Introduction Hri
Approaches to the pharmacotherapy of the atypical parkinsonian or Parkinson plus disorders have, as yet, met with little success. Drugs attempting to replace or mimic specific neurotransmitters are relatively ineffective, as evidenced, for example, by the inconsistent response rates to dopamine DA replacement by the administration of levodopa. This variable and modest efficacy contrasts with the predictable and significant benefit obtained with dopaminomimetics in Parkinson's disease PD in this...
Test Stimuli For Audiotaped Samples
You wish to know all about your grandfather. Well, he is nearly 93 years old, yet he still thinks as swiftly as ever. He dresses himself in an old black frock coat, usually several buttons missing. A long beard clings to his chin, giving those who observe him a pronounced feeling of the utmost respect. Twice each day he plays skillfully and with zest upon a small organ. Except in the winter when the snow or ice prevents, he slowly takes a short walk in the open air each day. We have often urged...
Progressive Supranuclear Palsy 1
In the original description of this disease entity, patients with supranuclear ophthalmoplegia, pseudobulbar palsy, dysarthria, rigidity, and mild cognitive deficits were found to have abundant NFTs in subcortical nuclei 91 . The clinical phenotype is now recognized to be much more variable and includes both pure parkinsonism and frontotemporal dementia with no movement abnormality 1,2,4,5,71 . The macroscopic changes are quite variable. The cerebral cortex often appears normal but may show...
Onset And Progression
MSA usually manifests in middle age the median age of onset is 53 , affects both sexes, and progresses relentlessly with a mean survival of 6-9 yr 13,19,20 . MSA patients may present with akinetic-rigid parkinsonism that usually responds poorly to levodopa. This has been identified as the most important early clinical discriminator of MSA and PD 11,21-23 , although a subgroup of MSA patients may show a good or, rarely, excellent, but usually short-lived, response to levodopa 24-26 . Progressive...
Postanoxic Parkinsonism
Parkinsonism can rarely result from hypoxic ischemic injury. Different movement disorders including chorea, tics, athetosis, dystonia, and myoclonus have been reported. Patients can develop parkinsonism with or without dystonia weeks to months after the ischemic event 74 . MRI findings include T1 hyper intensities in the basal ganglia bilaterally, indicative of ischemia or gliosis. In the case described by Li et al. 74 , the clinical findings included mainly an akinetic rigid syndrome with...
References Iiv
1. McKeith IG, Galasko D, Kosaka K, et al. Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies DLB report of the consortium on DLB international workshop. Neurology 1996 47 1113-1124. 2. Bhatia KP, Marsden CD. The behavioural and motor consequences of focal lesions of the basal ganglia in man. Brain 1994 117 Pt 4 859-876. 3. Burns A, Jacoby R, Levy R. Psychiatric phenomena in Alzheimer's disease. I Disorders of thought content. Br J Psychiatry 1990 157...
Madhavi Thomas and Joseph Jankovic
Idiopathic Parkinson's disease PD is the most common cause of parkinsonism, accounting for about 75 of all cases. Other causes of parkinsonism include multiple system atrophy MSA , progressive supranuclear palsy PSP , corticobasal degeneration CBD , and a variety of other neurodegenerative disorders in which rest tremor, bradykinesia, rigidity, and other parkinsonian features are present. Since these disorders are often associated with other neurological deficits, such as dysautonomia in MSA ,...
Other Neurodegenerative Conditions
Many other common idiopathic neurodegenerative conditions have parkinsonism as an inconsistent or minor clinical feature. Extrapyramidal symptoms are very common in AD and may take the form of true parkinsonism 135-137 . Many patients with a clinical diagnosis of AD and parkin-sonism are found to have coexisting LB pathology at autopsy, either restricted to subcortical structures or also involving the cerebral cortex 35,136,138 . The correct terminology for these cases is uncertain, because of...
References Ptf
1. Hughes AJ, Daniel SE, Ben-Shlomo Y, et al. The accuracy of diagnosis of parkinsonism syndromes in a specialist movement disorder service. Brain 2002 125 861-870. 2. Hughes AJ, Daniel SE, Kilford L, Lees AG. Accuracy of clinical diagnosis of idiopathic Parkinson's disease a clinico-pathological study of 100 cases. J Neurol Neurosurg Psych 1992 55 181-184. 3. Rajput AH, Rozdilsky B, Rajput A. Accuracy of clinical diagnosis of parkinsonism a prospective study. Can J Neurol Sci 1991 18 275-278....
Progressive Supranuclear Palsy Efi
With regard to MRI diagnosis of PSP, all the attention has been given to atrophy of the midbrain where pathologic changes are known to occur 43 . Signal changes in this region have also been noted. Less attention has been paid to basal ganglia abnormalities and cerebral atrophy, which are more variable and less useful for the diagnosis. MRI simply confirmed what pneumoencephalography first, and later CT 44,45 have shown demonstration of midbrain atrophy is the cardinal neuroradiologic feature...
Hemiplegic Parkinsons Disease
Early neurologists considered Parkinson's disease to be a bilateral condition, but often commented on the mild asymmetry of tremor, especially in the early years of disease. Within the context of this asymmetric but bilateral archetype, they distinguished another form of Parkinson's disease that was highly asymmetric with prominent disability in the involved upper extremity beyond that expected with bradykinesia alone 3 . Collectively termed hemiplegic Parkinson's disease, these cases form a...
Info Ffg
Fig. 1. Plots summarizing important dynamic properties of saccades. A Plot of peak velocity vs amplitude of vertical saccades. Data points are saccades from 10 normal subjects. The data are fit with an exponential equation also plotted are the 5 and 95 prediction intervals. The indicate vertical saccades from a patient with PSP, which lie outside the prediction intervals for normals. B Plot of duration vs amplitude. The data from 10 normal subjects are fit with a power equation. The indicate...
Contributors
Dag Aarsland, PhD Centre for Neuro and Geriatric Psychiatric Research, Rogaland Central Hospital, Stavanger, Norway Yves Agid, md, PhD INSERM U-289, H pital de la Salp triere, Paris, France Clive Ballard, PhD Wolfson Centre for Age-Related Diseases, Kings College London, London, UK Yoav Ben-Shlomo, mrcp, FFPHM Department of Social Medicine, University of Bristol, Bristol, UK Bradley F. Boeve, md Division of Behavioral Neurology, Department of Neurology, Mayo Clinic College of Medicine,...
Progressive Supranuclear Palsy
In 1963, Steele, Richardson, and Olszewski presented a report at the American Neurological Association of a new syndrome typified by parkinsonism, marked vertical gaze paresis, dementia, and axial rigidity 10,11 . Though they felt they were describing a new syndrome, they referred colleagues to similar cases from the recent past 12-14 Fig. 4 . H. Houston Merritt opened the discussion, commenting that he had not seen similar cases, that the involved areas all related to cell populations of...