Neurological disease

Chapter 22 Neurological disease

Common symptoms and signs

Pattern recognition in neurology – interpretation of history, symptoms and examination – is very reliable. Practical experience is vital. There are three critical questions in formulating a clinical diagnosis:

Difficulty walking and falls

Change in walking pattern is a common complaint (Box 22.1). Arthritis and muscle pain make walking painful and slow (antalgic gait). The pattern of gait is valuable diagnostically.

Spasticity and hemiparesis

Spasticity (p. 1082), more pronounced in extensor muscles, with or without weakness, causes stiff and jerky walking. Toes of shoes become scuffed, catching level ground. Pace shortens; a narrow base is maintained. Clonus – involuntary extensor rhythmic leg jerking – may occur.

In a hemiparesis when spasticity is unilateral and weakness marked, the stiff, weak leg is circumducted and drags.

Parkinson’s disease: shuffling gait

There is muscular rigidity (p. 1118) throughout extensors and flexors. Power is preserved; the pace shortens, and slows to a shuffle; its base remains narrow. A stoop and diminished arm swinging become apparent. Gait becomes festinant (hurried) with short rapid steps. There is difficulty turning quickly and initiating movement, sometimes with falls. Retropulsion means small backward steps, taken involuntarily when a patient is halted.

Cerebellar ataxia: broad-based gait

In lateral cerebellar lobe disease (p. 1083) stance becomes broad-based, unstable and tremulous. Ataxia describes this incoordination. When walking, the person tends to veer to the side of the affected cerebellar lobe.

In disease of midline structures (cerebellar vermis), the trunk becomes unsteady without limb ataxia, with a tendency to fall backwards or sideways – truncal ataxia.

Sensory ataxia: stamping gait

Peripheral sensory lesions (e.g. polyneuropathy, p. 1145) cause ataxia because of loss of proprioception (position sense). Broad-based, high-stepping, stamping gait develops. This form of ataxia is exacerbated by removal of sensory input (e.g. vision) and worse in the dark. Romberg’s test, first described in sensory ataxia of tabes dorsalis (p. 1129), becomes positive.

Examination and formulation

Following a short or detailed examination, relevant findings are summarized in a brief formulation – the basis for investigation, transfer of information and management (Practical Boxes 22.1, 22.2 and Table 22.2).

Table 22.2 Six grades of muscle power

Grade Definition


Normal power


Active movement against gravity and resistance


Active movement against gravity


Active movement with gravity eliminated


Flicker of contraction


No contraction

Functional neuroanatomy

Localization within the cerebral cortex

This subject causes unnecessary difficulty. Work on neuronal networks, functional imaging and plasticity questions the traditional views of highly specific localization of cortical function. The following paragraphs summarize areas of clinical relevance.


Aphasia is loss of or defective language from damage to the speech centres within the left hemisphere. Numerous varieties have been described.

Cranial nerves (Table 22.3)

I: Olfactory nerve

This sensory nerve arises from olfactory (smell) receptors within nasal mucosa. Branches pierce the cribriform plate and synapse in the olfactory bulb. The olfactory tract passes to the olfactory cortex.

Table 22.3 Cranial nerves

Number Name Main clinical action






Vision, fields, afferent light reflex



Eyelid elevation, eye elevation, ADduction, depression in ABduction, efferent (pupil)



Eye intorsion, depression in ADduction



Facial (and corneal) sensation, mastication muscles



Eye ABduction



Facial movement, taste fibres



Balance and hearing






Sensation – soft palate, taste fibres



Cough, palatal and vocal cord movements



Head turning, shoulder shrugging



Tongue movement

Anosmia (loss of sense of smell) is caused by head injury (shearing of olfactory neurones as they pass through the cribriform plate at the skull base) or tumours of the olfactory groove (e.g. meningioma). Olfaction is temporarily (occasionally permanently) lost or diminished after upper respiratory infections and with local disorders of the nose. Many patients with gradual onset anosmia over many years may be unaware of the deficit, e.g. in Parkinson’s disease where anosmia precedes motor symptoms by many years but is often not noticed by the patient.

Detailed smell testing is difficult in routine clinical practice and rarely performed. Adequate testing requires use of commercially available kits such as scratch and sniff cards or odour filled pens with forced multiple choice identification.

II: Optic nerve and visual system (Fig. 22.4)

Light regulated by the pupillary aperture is converted into action potentials by retinal rod, cone and ganglion cells (see page 1055). The lens, under control of the ciliary muscle, produces the image (inverted) on the retina. Axons in the optic nerve (1) decussate at the optic chiasm (2), fibres from the nasal retina cross and join with uncrossed fibres originating in the temporal retina to form the optic tract (3). Each optic tract thus carries information from the contralateral visual hemifield.

From the lateral geniculate body, fibres pass in the optic radiation through the parietal and temporal lobes (4 and 5) to reach the visual cortex of the occipital lobe (6 and 7), which is somatotopically organized with macular vision located at the occipital pole (see Fig. 22.4).

Beyond the visual cortex visual information is further processed by neighbouring visual association areas to detect lines, orientation, shapes, movement, colour and depth; there is even a distinct area responsible for face recognition.

Visual field defects

Visual fields are assessed at the bedside by confrontation – comparing the examiner’s and patient’s fields, one eye at a time and quadrant by quadrant. Patience and good technique are required to get reliable results. White and red targets (traditionally hatpins) are used to assess peripheral and central fields respectively although in practice a fingertip is often substituted as a cruder screening test. More detailed quantification of fields may be obtained using Goldmann (manual) or Humphrey (automated) perimetry testing.

Field defects are described as hemianopic when half the field is affected and quadrantanopic when a quadrant is affected. Lesions posterior to the optic chiasm produce homonymous field defects, indicating involvement of the same part of the visual field in both eyes as information from the two visual hemifields is separated beyond this point. Lesions damaging decussating nasal fibres at the optic chiasm cause bitemporal defects.

Optic nerve lesions

Unilateral visual loss, commencing with a central or paracentral (off-centre) scotoma, is the hallmark of an optic nerve lesion. Because most fibres in the optic nerve subserve macular vision, lesions within the nerve disproportionately affect central vision and colour vision. A total optic nerve lesion causes unilateral blindness with loss of pupillary light reflex. Examination findings in optic neuropathy:

Causes are listed in Box 22.3.


Papilloedema means swelling of the optic disc. Causes are shown in Box 22.4. The earliest signs of swelling are disc pinkness, with blurring and heaping up of disc margins, nasal first. There is loss of spontaneous pulsation of retinal veins within the disc. The physiological cup becomes obliterated, the disc engorged with dilated vessels. Small haemorrhages often surround the disc.

Various conditions simulate true disc swelling. Marked hypermetropic (long-sighted) refractive errors make a disc appear pink, distant and ill-defined. Myelinated nerve fibres at disc margins and hyaline bodies (drusen, p. 1064) can be mistaken for disc swelling.

Disc infiltration also causes a swollen disc with raised margins (e.g. in leukaemia).

When there is doubt about disc oedema, i.v. fluorescein angiography is diagnostic; retinal leakage is seen with papilloedema.

Papilloedema produces few if any visual symptoms other than momentary visual obscurations with changes in posture. The underlying disease is the source of the patient’s symptoms. The blind spot is enlarged but this is not noticed by the patient. However, over time progressive and permanent constriction of visual fields occurs, ultimately culminating in optic atrophy.

The pupils

A slight difference between the size of each pupil (up to 1 mm) is common (physiological anisocoria) and does not vary with differing light levels. The pupil tends to become smaller and irregular in old age (senile miosis); anisocoria is more pronounced. Convergence becomes sluggish with ageing.

Pupillary reactions to light and accommodation may be tested (Fig. 22.5). A bright torch (not an ophthalmoscope light!) should be used to test the pupillary light reaction.

Afferent pupillary defect. A complete optic nerve lesion causes a dilated pupil and an afferent pupillary defect (APD). For a left APD:

Relative afferent pupillary defect (RAPD). This occurs with incomplete damage to one optic nerve relative to the other. An RAPD is a sensitive sign of optic nerve pathology and can provide evidence of an optic nerve lesion even after recovery of vision. For a left RAPD:

III, IV, VI: Oculomotor, trochlear and abducens nerves

These cranial nerves supply the extraocular muscles and disorders commonly result in abnormal eye movements and diplopia (double vision) due to breakdown of conjugate (yoked) eye movements. Diplopia may also occur with local orbital lesions or myasthenia gravis.

Abnormalities of conjugate lateral gaze

A destructive lesion on one side allows the eyes to be driven by the intact opposite pathway. A left frontal destructive lesion (e.g. an infarct) leads to failure of conjugate lateral gaze to the right. In an acute lesion the eyes are often deviated to the side of the lesion, past the midline and therefore look towards the left (normal) limbs; there is usually a contralateral (i.e. right) hemiparesis.

In the brainstem a unilateral destructive lesion involving the PPRF leads to failure of conjugate lateral gaze towards that side. There is usually a contralateral hemiparesis and lateral gaze is deviated towards the side of the paralysed limbs.


Nystagmus is rhythmic oscillation of eye movement, and a sign of disease of the retina, cerebellum and/or vestibular systems and their connections. Nystagmus is either jerk or pendular. Nystagmus must be sustained within binocular gaze to be of diagnostic value – a few beats at the extremes of gaze are normal.

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Apr 1, 2017 | Posted by in GENERAL & FAMILY MEDICINE | Comments Off on Neurological disease

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