Accuracy in the Clinical Diagnosis of Parkinsonian Syndromes W.R.G
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Postgrad Med J: first published as 10.1136/pgmj.64.751.345 on 1 May 1988. Downloaded from Postgraduate Medical Journal (1988) 64, 345-351 Review Article Accuracy in the clinical diagnosis of parkinsonian syndromes W.R.G. Gibb National Hospitals for Nervous Diseases, Maida Vale, London W9 1TL and Neurology Department, The Middlesex Hospital, Mortimer Street, London WIN 8AA, UK. Summary: This review of Parkinson's disease and related disorders emphasizes the difficulties of distinguishing between variants of the parkinsonian syndrome. Characteristic clinical features may remain absent for many months, but accuracy of diagnosis may be improved by considering certain presenting symptoms and signs. The main characteristics of various parkinsonian syndromes are reviewed and their major distinguishing features are emphasized. Future improvement in the precision of clinical diagnosis, especially early in the course of parkinsonian syndromes, will depend on selecting out patients with Parkinson's disease using positive diagnostic criteria. Introduction Idiopathic Parkinson's disease is the foremost of a Seventy-four showed loss of pigmented cells in the variety of disorders characterized by slowness of substantia nigra with Lewy bodies in some of the copyright. movement and muscular stiffness' (Table I). Many remaining cells. Two brains without these inclusions are degenerative diseases in which loss of nigro- had the pathology of striatonigral degeneration6 striatal neurones and dopaminergic input to the and two had the pathology of post-encephalitic striatum are responsible for the parkinsonian parkinsonian syndrome.7 Lewy bodies are not disorder. Another common mechanism is associated with the pathology of these disorders so pharmacological interference by dopamine receptor that Parkinson's disease can be diagnosed and antagonists acting at the post-synaptic striatal defined according to pathological critieria as a http://pmj.bmj.com/ dopamine receptor. The variety of pathologies parkinsonian disorder with Lewy bodies in the embraced by the parkinsonian syndromes is substantia nigra. illustrated by one neuropathological study of 67 unselected parkinsonian cases.2 Only 29 (43%) had pathology consistent with Parkinson's disease, Table I Causes of parkinsonian syndromes which is cell loss in the substantia nigra with Lewy inclusion bodies in remaining cells. The rest had Idiopathic Parkinson's disease findings compatible with a number of other Drug-induced neuroleptics, reserpine on September 25, 2021 by guest. Protected parkinsonian syndromes. In most other studies Striatonigral degeneration (multiple system atrophy) restricted to patients with Parkinson's disease the Steele-Richardson-Olszewski syndrome frequency of this pathology is reported in 70%3 to Alzheimer's disease 96%,4 reflecting inaccuracy in clinical diagnosis or Atherosclerotic vascular disease Communicating hydrocephalus inconsistent pathology. Post-encephalitic parkinsonian syndrome A recent study has been done to examine whether Trauma Lewy bodies are found in the substantia nigra of all Tumours persons dying with Parkinson's disease.' A Carbon monoxide poisoning retrospective clinical diagnosis of Parkinson's Corticobasal degeneration disease was made in a selected group of 78 patients. Juvenile parkinsonian syndrome MPTP Basal ganglia calcification Wilson's disease Correspondence: W.R.G. Gibb M.D., M.R.C.P. Accepted: 3 December 1987 Refer to Fahn1 for a more comprehensive list. ©) The Fellowship of Postgraduate Medicine, 1988 Postgrad Med J: first published as 10.1136/pgmj.64.751.345 on 1 May 1988. Downloaded from 346 W.R.G. GIBB Many parkinsonian disorders have clinical appears to be abrupt and may be attributed to features which are not seen in Parkinson's disease, stressful physical or emotional life events. With but the conditions that most commonly cause relief from stress, symptoms may remit, only to confusion include striatonigral degeneration and reappear weeks or months later. A number of early drug-induced parkinsonian syndrome. The signs may be suggestive (Table III), but confident difficulties in diagnosis lie in the fact that in the diagnosis of a parkinsonian syndrome depends on early months or years of a parkinsonian disorder identifying at least two of bradykinesia, rest tremor, features diagnostic of atypical syndromes may be muscular rigidity and impaired postural reflexes. absent. First symptoms of Parkinson's disease are often Parkinson's disease is traditionally diagnosed in two stages. Firstly by identifying a parkinsonian Table II Criteria that exclude or question a diagnosis of syndrome, by recognising any two of bradykinesia, Parkinson's disease muscular rigidity, rest tremor and postural instability. Secondly by using a mental checklist of Criteria that exclude Parkinson's disease exclusion criteria (Table II), which may provide Oculogyric crises evidence for the alternative diagnoses (listed in Gaze palsy Table I and discussed below). Some clinicians might Several affected relatives argue that the current diagnostic success rate of 75- Poor or absent response to L-dopa 90% for Parkinson's disease is acceptable provided Cerebellar signs that surgically treatable conditions, such as com- Peripheral neuropathy municating hydrocephalus and tumours, are Autonomic neuropathy excluded. A trial of L-dopa and a treatment Complete remission of symptoms/signs* diagnosis, as a responder or non-responder, is Criteria that place the diagnosis in doubt usually considered the next step. However, an uncritical approach to is Neuroleptic medication diagnosis inappropriate, Dementia at onset because 'benign' and specifically treatable disorders Evidence of arteriosclerosis such as essential tremor or drug-induced Unexplained pyramidal signs copyright. parkinsonian syndrome can be difficult to diagnose even by experienced clinicians. Awareness of the *Minor fluctuation and apparent remission of symptoms course of a disease will readily identify remediable may occur at the onset. complications, such as those of confusion or dementia resulting from drug therapy, infection Table III Early symptoms and signs of Parkinson's or subdural haematoma. Repeated clinical disease* examinations and accurate documentation of signs may be necessary to assess response to L-dopa Early symptoms http://pmj.bmj.com/ therapy and to identify fresh clinical signs Fatigue following exertion, lethargy, depression incompatible with a diagnosis of Parkinson's Restlessness, anxiety disease alone. Diagnostic accuracy is also essential Drooling, constipation, for epidemiological work, drug trials, neuro- Low volume or hoarse voice chemical and pathological studies, for which rating Small handwriting scales for documenting stages of disease severity are Muscle cramps, pains, stiffness valuable.8 Difficulty rising from chair or turning in bed Difficulty with balance or turning, especially in the elderly on September 25, 2021 by guest. Protected Slowness over daily activities Idiopathic Parkinson's disease Early signs The age of onset can range from 20 years upwards, Expressionless face but the disease is more common in middle and late Slow movement with decrement in amplitude and age. The mean age of onset is about 60 years and disintegration of continued or repeated movement the disease duration 10 years, possibly with an Reduced movement - including blink rate, arm swing on additional 2 to 4 years with L-dopa treatment. A walking, spontaneous gestures number of vague, isolated, fluctuating or persistent Loss of automatic and emotional control of expression symptoms often Positive glabellar tap precede parkinsonian signs by Axial or proximal rigidity on synkinesis many months (Table III). These provide Pseudohemiparesis opportunities for misdiagnosis as they are not specific to Parkinson's disease or any other *These symptoms and signs occur in other parkinsonian parkinsonian syndrome. In some patients the onset syndromes. Postgrad Med J: first published as 10.1136/pgmj.64.751.345 on 1 May 1988. Downloaded from DIAGNOSIS OF PARKINSONIAN SYNDROMES 347 tremor or difficulty in walking in the elderly, and Parkinson's disease occurring in older patients tremor or muscular stiffness in younger patients. treated with small doses of less potent drugs. For Tremor at rest has been shown in pathological example, 48 of 95 patients admitted to a depart- studies to be fairly specific for Parkinson's ment of geriatric medicine with a parkinsonian disease9'10 and about 70% of patients may show syndrome had consumed neuroleptics.18 Tremor it. " Onset with unilateral symptoms is common was present in 40%, bradykinesia in 80% and falls and persistent asymmetry affecting the side of or inability to walk were frequent. At least 6 onset most is characteristic.12 L-Dopa consistently months without medication may be required to produces moderate or good improvement, and this distinguish drug-induced from idiopathic disease. may increase for some months.13 In a series of 400 patients with Parkinson's disease 15% did not show Striatonigral degeneration a beneficial therapeutic response to L-dopa, suggesting that they had other parkinsonian Striatonigral degeneration causes a parkinsonian disorders, many of which are not generally syndrome, but olivopontocerebellar atrophy and responsive to L-dopa.14 No patient with degeneration of the autonomic nervous system, uncomplicated Parkinson's disease, defined patho- which coexist, may cause additional cerebellar signs logically, has been shown to have a negative