How Can We Help?
Movement disorders
Generalised stiffness and other movement disorders
- True Parkinson’s disease (see separate entry)
- Progressive cerebrovascular disease – some features like parkinsonism, but doesn’t respond to l-dopa
- Phenothiazine use
- Phenothiazine idiosyncratic reaction – neurolept-malignant syndrome. (High fever, stiffness and rigidity after use of any psychotropic – treat by stopping the drug and providing adequate hydration and nutrition. No specific agent has been demonstrated to hasten recovery. See separate entry.)
- Pellagra
- Hyperosmolar non-ketotic pre-coma
- Coning – usually either history of trauma or some lateralising signs.
- Syphilis (reputed not to occur, but a few well-documented case-reports, so worth excluding)
- Organophosphate poisoning – usually other clues!
- (Tetanus and rabies.)
Parkinsonism.
There are a number of parkinson-like conditions:
- Drug induced Parkinson’s. The history usually makes this self-evident (neuroleptic exposure), and it is by far the commonest cause for bradykinesia and rigidity in a young person.
- Multiple system atrophy Parkinsonism, cerebellar features, autonomic features (bladder and bowel problems, orthostatic hypotension) and sometimes pyramidal dysfunction. In MSA-P there is marked postural instability and little tremor, and MSA-C has the cerebellar signs. In general respond poorly to dopamine.
- Progressive supranuclear palsy In this syndrome there is a supranuclear ocular palsy with pseudobulbar palsy and axial dystonia. The first eye symptom is difficulty looking down, but later all directions of eye movement can be affected. You can tell it is supranuclear because while the eyes cannot deviate to fixate on a stimulus, if you turn the head whilst asking the patient to look ahead, you can elicit eye movements.
- Normal pressure hydrocephalus.
If Normal pressure hydrocephalus is considered, a CT is worthwhile. Considerit in elderly patients who present with the following:
- Incontinence
- Loss of memory (or other intellectual changes)
- Short-stepped rather unstable looking gait, often with hyper-reflexia.
The CT shows hydrocephalus that can be difficult to distinguish from the hydrocephalus associated with atrophy (so-called ‘hydrocephalus ex vacuo’). Sometimes the only way of being sure is to shunt such individuals, where rapid improvement in signs and symptoms supports the diagnosis.
Arteriosclerotic rigidity (‘vascular parkinsonism’).
This is well worth knowing about because you can’t treat it successfully with anti-parkinson medications, although patients are very frequently given them. All that happens then is that the person is further disabled by hallucinations and dystonias without any mobility advantage at all. Essentially you turn a stroke patient into a hallucinating dystonic stroke patient.
- Abrupt onset of symptoms, and often some mental as well as the physical changes.
- Walk with short quick jerky steps, but no difficulty getting started.
- Little, if any, tremor.
- Can be quite markedly rigid.
- Pyramidal features such as brisk reflexes, a brisk jaw jerk and extensor plantars make it clearly not Parkinson’s disease.
- There may be speech alterations, but this is more of a pseudobulbar change than the low-pitched voice of Parkinson’s. The face is often quite animated.
On occasion it can be difficult to be entirely sure, and in those (few) cases there is considerable temptation to give a trial of l-dopa. This is probably justifiable, but if done, ensure that the patient is carefully monitored, and you explain beforehand to both patient and relatives what you have in mind. When after a month or so your trial has clearly failed, remember to stop the l-dopa!
Parkinson’s disease.
Diagnosis:
- Tremor – pill rolling. It doesn’t always get better with activity, but is classically described as a slow frequency resting tremor.
- Rigidity
- Bradykinesia
A fourth feature that is less commonly elicited is loss of postural reflexes.
Many patients with absolutely classic features seem to present very late, due to a combination of family and health care workers ascribing the changes to either old age or arthritis or both.
A very characteristic feature worth asking about is difficulty turning over in bed at night. This seemingly simple task may prove virtually impossible without assistance.
Perspective – value of various features of Parkinson’s in making the diagnosis[24]:
The classical combination of tremor, rigidity and bradykinesia had a modest LR+ of 2.2 (LR- 0.5) whereas a history of a softer voice (LR+ 3.4), a glabella tap (LR+ 4.5), all performed somewhat better.
Eliciting a glabellar tap requires attention to detail – you need to avoid obtaining a startle reflex with your whole hand. Stand on the right side of a sitting patient and bring your left arm over the patient’s head so that your palm is almost resting on the forehead, then tap slowly, gently and rhythmically between the eyebrows using a single finger. Most people will blink in synchrony with the taps for a few times and then suppress. A positive response is present if the blink persists after more than five to ten taps.
It is important to take a history – difficulty turning in bed associated with a softer voice will give a combined LR+ of 44.2, and LR- 0.25. Eliciting a glabellar tap as well as the ‘conventional’ triad of tremor, rigidity and bradykinesia will strengthen the diagnosis considerably. Tremor alone is a weak sign (LR+ 1.3.).
Simple advice about moving around in the house, ensuring that there is someone around to give assistance, and generation of realistic expectations from therapy can be valuable[25].
Medication for Parkinson’s
Levo-dopa. Earlier recommendations that it be ‘saved’ for when it is really needed (it eventually stops working) were probably inappropriate. As a chronic ongoing degenerative disease, Parkinson’s does progress, so therapy started at any stage will eventually cease to be effective. If started early, the patient can get full advantage from the drug and may be able to lead a near-normal life. If started very late, it may still help, but often with significant residual disability which still renders the patient dependent even though a bit more mobile.
The commonly available preparation is a mixture of l-dopa either 100 mg or 250 mg, and a decarboxylase inhibitor carbidopa 25 mg, the dose of which stays constant. Start with 100 mg tablets, and give half a tablet twice a day (i.e. 50 mg 2x/d) then increase to 50 mg 3x/d, then 100 mg 3x/d, then 200 mg 3x/d and finally 250 mg 3x/d. These dose steps are a guide only, and in some patients you can push faster whereas in others you need to go slower. Allow at least a week between dose adjustments, no matter how impatient the patient is, the reason being that too rapid rises nearly always cause side-effects which may create a psychological ‘block’ to re-attempting that dose again later.
Bromocriptine. In young healthy patients there is a case for starting with a dopamine agonist first[26], but in view of the cost of these agents, their availability is somewhat limited. Motor improvement is not quite as good as with levodopa, and quality of life may be no different[27] (pramipexole). Eventual dose of bromocriptine required is often of the order of 10 – 30 mg per day in three divided doses, but one has to start with a tiny amount (1.25 mg/d) and increase quite slowly, probably at weekly intervals. In terms of equi-effective doses, pergolide is not that much more expensive than bromocriptine – also start low and work up slowly.
Amantadine. Evidence that the use of amantadine achieves much is scanty. The anticholinergics are recommended but again have rather unimpressive efficacy in practice and can cause considerable inconvenience particularly to elderly patients already experiencing problems with micturition.
Adverse effects of treatment in Parkinson’s disease
Parkinson’s disease is a prime example of a disease where the available therapies are all toxic to some extent, and these adverse effects can significantly affect ability to use the products. All can cause hypotension, but the table shows the spectrum:
Drug | Adverse event | Frequency | Comment |
|---|---|---|---|
Bromocriptine | Nausea | ||
Hallucinations | |||
Pergolide[28] | Nausea | 25% | |
Hallucinations | 10% | ||
Dyskinesias | 60% | Reduce levodopa dose if on both | |
Amantadine | Psychosis, convulsions | ||
Urinary retention | |||
L-dopa | Nausea | 80% | Take after meals |
Psychosis | |||
Involuntary movements | 50-80% | Smaller dose more often | |
Urinary retention | |||
On-off phenomenon | 20-90% | ||
Selegeline | Nausea, confusion |
Perspective – neuroprotective agents in Parkinson’s disease
Patients sometimes ask about these agents. The fall into a number of classes: antioxidants such as Vitamins E and C, monoamine oxidase type B inhibitors such as selegeline, and anti-inflammatories such as the COX-2 inhibitors. The dopamine agonist pramipexole may also have anti-oxidant effects. There is as yet no convincing evidence that any of these agents can slow or reverse disease progression, although some of them look quite promising[29].