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Neurology – miscellaneous (MND, multiple sclerosis, hydrocephalus, idiopathic intracranial hypertension)
Motor neurone disease
This disease is generally recognised by a characteristic combination of upper and lower motor neurone signs without sensory signs. It has traditionally been divided into four subtypes. There is often considerable overlap, particularly with time, but the initial presentations are sufficiently different to arouse confusion if not considered:
- Amyotrophic lateral sclerosis
- Progressive spinal muscular atrophy
- Progressive bulbar palsy
- Primary lateral sclerosis
Amyotrophic lateral sclerosis
This is the commonest, and presents with hand clumsiness progressing to the characteristic tetrad of:
weakness and wasting of the hands and forearms.
spastic weakness of the legs.
generally increased reflexes.
no sensory changes.
Fasciculation of involved muscles may be seen. Variants include those involving the shoulder girdle and pelvic muscles (so-called Kugelberg-Welander disease), those involving the legs first (present with foot drop – so-called crural amyotrophy) and very occasionally a hemiplegic (Mills) type.
Progressive spinal muscular atrophy
Weakness and atrophy of hand and arm muscles without hyper-reflexa and without sensory changes. More slowly progressive than ALS.
Progressive bulbar palsy
Progressive difficulty with articulation, and then with chewing and swallowing. Weak facial muscles but markedly hyperactive jaw jerk. (Put your finger across the chin, get the patient to relax, and then tap your finger with the patella hammer.)
Primary lateral sclerosis (rare!)
Spastic paraplegias, sometimes associated with cerebellar ataxia, are sometimes hereditary, so it is important to ask if anyone else in the family has a similar problem. When this occurs in infancy, it may be labelled the Werdnig-Hoffmann type, but it can occur at all ages and there are numerous variants.
Management
Once the condition is recognised, management is primarily about counselling the patient and family about the progressive nature of the illness and the lack of any clearly effective medication. Assistance with activities of daily living involves imaginative discussion of what constitute real problems for that particular individual, and early and regular involvement of speech therapist, physiotherapist and occupational therapist if any of these are available. In their absence, don’t do nothing – a little common sense can find workable solutions for many common problems, and there is no law preventing you from phoning up individuals in one of these departments in a teaching hospital and asking for advice. Allowing the family and patient access to you in outpatients may prevent unnecessary admissions and, like palliative care for cancer patients, can be particularly rewarding.
Multiple sclerosis.
This is not common in warmer countries. It is mentioned mainly because of the propensity to dump other illnesses into a MS basket. It presents with weakness and numbness or paraesthesiae in one limb, often of abrupt onset. About a quarter of patients develop optic neuritis at some stage (blindness in one eye which recovers partially or completely within weeks). Practically any neurological sign can occur, and in some patients symptoms are short-lived (seconds to minutes) and recurrent.
Diagnosis is predominantly based on the characteristic history and signs, and the tempo of their progression. Looking for oligoclonal bands on CSF, and white matter lesions on MRI, may be helpful, but in view of the implications of the diagnosis, it is preferable that a neurologist sees all such patients. SLE and ALS as well as cervical spondylosis may cause confusion.
Perspective – medication in multiple sclerosis.
Just as in other diseases with a remitting and relapsing course (e.g. rheumatoid arthritis) trials of therapy are sometimes difficult to interpret. The use of corticosteroids (either pulse methylprednisolone or oral prednisone) may benefit some patients, and interferon is popularly prescribed although there is still some controversy about the magnitude of the treatment benefit. All patients should be managed by or in consultation with a neurologist, and attention should be given to ‘fixable’ but somewhat mundane aspects of care such as bladder function, spasticity and mood.
Chronic fatigue syndrome.
Clinicians’ ‘belief’ in the condition ranges from the unquestioning adherents to the defiant sceptics. There probably are some individuals with a constellation of features suggestive of this syndrome, and on occasion it might be reasonable to consider it.
Major features[89] proposed (these are research rather than clinical definitions) include sudden onset without a previous history of a debilitating illness resulting in more than 50% activity reduction, not relieved by bed rest and lasting for more than 6 months. Consider organic and psychiatric disorders before contemplating the diagnosis. Myalgias, headaches, minor adenopathy and sleep disturbances are ‘minor’ features.
Management is elusive and purportedly best results are achieved by cognitive behavioural therapy.
Hydrocephalus
Types:
Hydrocephalus ex vacuo – cerebral atrophy leads to passive ventricular enlargement
Obstructive. Label according to the site (dilatation proximal to obstruction!) – one lateral ventricle, both lateral ventricles, both lateral ventricles and the third ventricle, or all four ventricles.
Symptoms and signs can be confusing because they are rather insidious:
- Mild headache
- Inattention, mental slowing, perseveration, and other frontal lobe signs.
- Clumsy gait, deteriorating to one that looks parkinsonian, or even cerebellar.
- Sometimes papilloedema
- In normal pressure hydrocephalus, get all of the above except papilloedema, and also get incontinence.
Treatment is to some extent related to the cause, but in general shunting is the therapy of choice.
Complications of shunts for hydrocephalus.
Shunts can be life-saving, but subsequently do give problems. A CT is warranted in any patient with a shunt who is drowsy, vomiting, or complaining of severe headache. Meningitis is also common, so if the CT does not show that shunt revision is needed, then think about how to get CSF for MC&S.
Idiopathic intracranial hypertension.
(Other names: benign intracranial hypertension, pseudotumour cerebri) May have:
- Headache
- Blurred vision
- Papilloedema
- Visual field testing showing peripheral loss.
- Normal or small ventricles on CT
- Raised CSF pressure at LP, and normal CSF cell count and chemistry.
Therapy Deal with associated ‘causes’: e.g. oral contraceptives, doxycycline, and obesity. Try a simple thiazide diuretic first. Alternatives are regular CSF drainage or prednisone 40 mg per day or all three. If visual deterioration progresses despite treatment, patients should be considered for optic nerve fenestration procedures or lumboperitoneal shunts. Good evidence of efficacy of most therapies is lacking.
Nerve and nerve root syndromes
Radial nerve palsy.
This is usually a reversible apraxia related to sleeping in an unusual position often after an alcoholic binge, hence the term Saturday night palsy. Patients present with a wrist drop and minor or no sensory signs. It usually responds to reassurance, time, and dynamic splinting by the physiotherapists.
Carpal tunnel syndrome
Pain, discomfort, and tingling in the lateral half (thumb side) of the palm of the hand, usually worst on getting up in the morning and improving during the day. Exclude hypothyroidism and then refer for carpal tunnel release by an orthopaedic surgeon.
Lateral cutaneous nerve of thigh
Hypoaesthesia or dysaesthesia in a relatively circumscribed area on the lateral aspect of the upper part of the thigh. Associations are with obesity and diabetes, sometimes after starting on insulin. Reassurance, weight loss and good sugar control.
Sciatica
Pain down the back of the thigh. Loss of ankle jerk and reduced power in toe (plantar flexion) are concerning signs. Bedrest probably doesn’t help. Surgical referral is appropriate, particularly if there is a clear motor deficit.
Cranial neuropathies
Pseudo tumour of the orbit and Tolosa-Hunt syndrome
The lateral wall of the cavernous sinus contains cranial nerves III, IV, V and VI, and a painful ophthalmopathy involving one eye and various combinations of these nerves, or sudden onset is thought to be due to a granulomatous inflammatory response. The point of recognising this (and differentiating it from all the other causes such as aneurysms and cavernous sinus thromboses) is that it seems to be exquisitely steroid sensitive, with symptoms settling promptly within days of starting treatment.
Other causes of proptosis
Besides the obvious, such as thyrotoxicosis, (which is not always bilateral) think of:
- mucormycosis (fungal infection of sinuses, usually in diabetics, usually with some degree of inflammation of the overlying skin)
- cavernous sinus thrombosis (also often associated with sinusitis, but can occur in young individuals with normal immunity)
- retro-orbital tumours – e.g. lymphomas
- granulomatous diseases (see pseudotumour above) including sarcoidosis
VII nerve palsy
There is often some confusion about differentiating upper and lower motor neurone lesions of the VII th nerve. It is perhaps easier to think of the VIIth nerve nuclei as consisting of two parts, with supply to the upper parts being bilateral.
Bell’s palsy:
Relatively sudden onset.
Inability to wrinkle the forehead.
The eye rolls up when the patient tries to close the lid. (Bell’s phenomenon.)
Occasionally there are mild sensory changes (V) on the face as well.
Testing tearing, hearing and taste seldom contributes to management, and findings can be variable owing to patchy involvement.
Management
Management of an isolated LMN VII is expectant as more than 85% can be expected to recover completely within nine months. Re-assure the patient that it isn’t a stroke, explain the generally excellent prognosis, and ensure that the cornea is protected at night – apply lubricating ointment (artificial tears) or tape the eye closed on going to bed.
Perspective – therapy of idiopathic facial paralysis.
A Cochrane review[90] failed to demonstrate benefit from corticosteroids, although the reviewers made the point that more evidence was required. The same applied to the use of acyclovir[91]. A subsequent large trial from Scotland[92] has demonstrated that acyclovir is of no benefit, but high dose prednisolone (25 mg 2x/d for 10 days) given within 72 hours of onset is helpful, although the clinical relevance of this is unclear in the absence of any difference in quality of life between the two groups at nine months.
Control | Prednisolone | ARR | NNT | |
Complete recovery | 0.816 | 0.944 | 0.128 (0.081 – 0.176) | 8 (6 – 13) |
Quality of life score | 0.88 SD 0.16 | 0.84 SD 0.26 | NS |
UMN VIIth:
Often sudden onset (stroke); can wrinkle forehead. ‘Emotional’ movement of corner of mouth possible (e.g. laughing.) Look for other stroke signs (e.g. clumsy hand.)
Isolated third or sixth nerve palsies
If a single cranial nerve is responsible then VIth paralysis will stop the eye looking straight laterally, IIIrd will prevent upward and inward gaze, and paralysis of the IVth downward and inward. You may need to tilt the head to bring out the signs of a IVth.
Lateral (ear) | SR | IO (III) | Medial (nose) | |
LR (VI) | Right Eye | MR | ||
IR | SO (IV) |
Third: diabetes (spares pupil) and posterior communicating artery aneurysm.
Sixth: raised intracranial pressure is usually the first thought, but don’t forget about thiamine deficiency which can progressive catastrophically if overlooked.