Sunday, October 9, 2022

 The history 120

Common presenting symptoms 122

Past medical history 123

Drug history 124

Family history 124

Social history 124

The physical examination 124

Assessment of conscious level 124

Meningeal irritation 124

Speech 125

Dysphasias 125

Cortical function 126

Cranial nerves 127

Olfactory (I) nerve 127

Optic (II), oculomotor (III), trochlear (IV) and abducens (VI) nerves 128

Trigeminal (V) nerve 128

Facial (VII) nerve 130

Vestibulocochlear (VIII) nerve 131

Glossopharyngeal (IX) and vagus (X) nerves 131

Accessory (XI) nerve 133

Hypoglossal (XII) nerve 133

Motor system 133

Anatomy 133

Stance and gait 134

Inspection and palpation of the muscles 136

Tone 137

Power 138

Deep tendon reflexes 139

Primitive reflexes 141

Coordination 141

Sensory system 142

Anatomy 142

Common presenting symptoms 143

Sensory modalities 144

Peripheral nerves 146

Median nerve 146

Radial nerve 146

Ulnar nerve 146

Common peroneal nerve 147

Lateral cutaneous nerve of the thigh 147

Interpretation of the findings 147

Investigations 147

Initial investigations 147

Specific investigations 149

OSCE example 1: Headache history 149

OSCE Example 2: Tremor 149

Integrated examination sequence for the nervous system 150

120 • The nervous system

The history

For many common neurological symptoms such as headache,

numbness, disturbance/loss of consciousness and memory loss,

the history is the key to diagnosis, as the examination may be

either normal or unhelpful. Some symptoms, including loss of

consciousness or amnesia, require an additional witness history;

make every effort to contact such witnesses.

Remember the two key questions: where (in the nervous

system) is the lesion and what is the lesion?

Neurological symptoms may be difficult for patients to describe,

so clarify exactly what they tell you. Words such as ‘blackout’,

‘dizziness’, ‘weakness’ and ‘numbness’ may have different

meanings for different patients, so ensure you understand what

the person is describing.

Ask patients what they think or fear might be wrong with

them, as neurological symptoms cause much anxiety. Patients

commonly research their symptoms on the internet; searches

on common benign neurological symptoms, like numbness or

weakness, usually list the most alarming (and unlikely) diagnoses

such multiple sclerosis, motor neurone disease or brain tumours

first, and almost never mention more common conditions such

as carpal tunnel syndrome or functional disorders.

Time relationships

The onset, duration and pattern of symptoms over time often

provide diagnostic clues: for example, in assessing headache

(Box 7.1) or vertigo (see Box 9.3).

Ask:

When did the symptoms start (or when was the patient

last well)?

Are they persistent or intermittent?

If persistent, are they getting better, getting worse or

staying the same?

Anatomy and physiology

The nervous system consists of the brain and spinal cord (central

nervous system, CNS) and the peripheral nerves (peripheral

nervous system, PNS). The PNS includes the autonomic nervous

system, responsible for control of involuntary functions.

The neurone is the functional unit of the nervous system. Each

neurone has a cell body and axon terminating at a synapse,

supported by astrocytes and microglial cells. Astrocytes provide

the structural framework for the neurones, control their biochemical

environment and form the blood–brain barrier. Microglial cells

are blood-derived mononuclear macrophages with immune and

scavenging functions. In the CNS, oligodendrocytes produce

and maintain a myelin sheath around the axons. In the PNS,

myelin is produced by Schwann cells.

The brain consists of two cerebral hemispheres, each with

four lobes (frontal, parietal, temporal and occipital), the brainstem

and the cerebellum. The brainstem comprises the midbrain, pons

and medulla. The cerebellum lies in the posterior fossa, with two

hemispheres and a central vermis attached to the brainstem by

three pairs of cerebellar peduncles. Between the brain and the

skull are three membranous layers called the meninges: dura mater

next to the bone, arachnoid and pia mater next to the nervous

tissue. The subarachnoid space between the arachnoid and pia

is filled with cerebrospinal fluid (CSF) produced by the choroid

plexuses. The total volume of CSF is between 140 and 270 mL

and there is a turnover of the entire volume 3–4 times a day;

thus CSF is produced at a rate of approximately 700 mL per day.

The spinal cord contains afferent and efferent fibres arranged

in discrete bundles (pathways running to and from the brain),

which are responsible for the transmission of motor and sensory

information. Peripheral nerves have myelinated and unmyelinated

axons. The sensory cell bodies of peripheral nerves are situated in

the dorsal root ganglia. The motor cell bodies are in the anterior

horns of the spinal cord (Fig. 7.1).

7.1 Clinical characteristics of headache syndromes

Onset Duration/periodicity Pain location Associated features

Primary syndromes

Migraine Evolves over

30–120 mins

Usually last <24 h, recurrent

with weeks/months

symptom-free

Classically unilateral but

may be anywhere

including face/neck

Aura (usually visual), nausea/vomiting,

photophobia and phonophobia

Cluster headache Rapid onset, often

waking patient from

sleep

30–120 mins, 1–4 attacks

within 24 h, clusters usually

last weeks to months, with

months to years of remission

Orbital/retro-orbital;

always same side during

cluster, may switch

sides between clusters

Autonomic features, including

conjunctival injection, tearing, nasal

stuffiness, ptosis, miosis, agitation

Stabbing headache Abrupt, rarely from sleep Very brief, seconds or less Anywhere over head Common in migraineurs

Secondary syndromes

Meningitis Usually evolves over a

day or two, can be

abrupt

Depends on cause and

treatment, usually days to

weeks

Global, including neck

stiffness

Fever, meningism, rash, false localising

signs, signs of raised intracranial

pressure

Subarachnoid

haemorrhage

Abrupt, immediately

maximal, rare from sleep

May be fatal at onset,

usually days to weeks

Anywhere, poor

localising value

20% isolated headache only; nausea/

vomiting, reduced consciousness, false

localising signs, III nerve palsies

Temporal arteritis Gradual onset of temple

pain and scalp

tenderness

Continuous Temple and scalp Usually in those >55 years; unwell, jaw

pain on chewing, visual symptoms, tender

temporal arteries, elevated erythrocyte

sedimentation rate and C-reactive protein

The history • 121

7

Pons

Medulla oblongata

Cerebellum

A

Postcentral gyrus

(sensory area)

Precentral gyrus

(motor area)

Sensory speech area

 (Wernicke's area)

Central sulcus

Occipital lobe

Parietal lobe

Motor speech area

 (Broca's area)

Lateral sulcus (fissure)

Frontal lobe

Temporal lobe

Posterior median septum

Dorsal column

Dorsal root entry zone

Central canal

Ventral grey horn

Rootlets

Mixed spinal nerve

Anterior median

fissure

B

Dorsal

Ventral

Spinal ganglion

Ventral column

Lateral column

Dorsal grey horn

Dorsal intermediate septum

Pia mater

Arachnoid mater

Dura mater

Dural root

sleeve

Position sense

Vibration sense

1/2 touch

Voluntary

movement

Voluntary

movement

Anterior (direct)

corticospinal tract C

Lateral (indirect)

corticospinal tract

Fasciculus gracilis

Fasciculus cuneatus

Anterior spinothalamic tract

Lateral spinothalamic tract

Anterior spinocerebellar tract

Posterior spinocerebellar tract

Areas of

extrapyramidal

tracts

Equilibrium

Pain

Temperature

1/2 touch

Presynaptic

nerve fibres

Nucleus

Dendrites

Cell body

Axon

Myelin

Node of

Ranvier

Spinal

motor neurone

Nerve

terminals

D

Fig. 7.1 Anatomy of the central nervous system. A Lateral surface of the brain. B Spinal cord, nerve roots and meninges. C Cross-section of the

spinal cord. D Spinal motor neurone. The terminals of presynaptic neurones form synapses with the cell body and dendrites of the motor neurones.

122 • The nervous system

Seizure

An epileptic seizure is caused by paroxysmal electrical discharges

from either the whole brain (generalised seizure) or part of the

brain (focal seizure). A tonic–clonic seizure (convulsion) is the

most common form of generalised seizure, and typically follows a

stereotyped pattern with early loss of consciousness associated

with body stiffening (tonic phase) succeeded by rhythmical jerking

crescendoing and subsiding over 30–120 seconds (clonic phase);

this is followed by a period of unresponsiveness (often with

heavy breathing, the patient appearing to be deeply asleep) and

finally confusion as the patient reorientates (postictal phase).

The history from the patient and witnesses can help distinguish

syncope from epilepsy (Box 7.2). Focal seizures may or may not

involve loss of awareness (complete loss of consciousness is less

typical) and are characterised by whichever part of the brain is

involved: for example, a focal motor seizure arising from the motor

cortex, or temporal lobe seizures characterised by autonomic

and/or psychic symptoms, often associated with automatisms

such as lip smacking or swallowing. Functional dissociative

attacks (also known as non-epileptic or psychogenic attacks, or

pseudoseizures) are common, and may be difficult to distinguish

from epileptic seizures. These attacks are often more frequent

than epilepsy, sometimes occurring multiple times in a day, and

may last considerably longer, with symptoms waxing and waning.

Other features may include asynchronous movements, pelvic

thrusts, side-to-side rather than flexion/extension movements

and absence of postictal confusion. The widespread availability

of videophones allows witnesses to capture such events and

may prove invaluable.

If intermittent, how long do they last, and how long does

the patient remain symptom-free in between episodes?

Was the onset sudden or gradual/evolving?

Precipitating, exacerbating or

relieving factors

What was the patient doing when the symptoms

occurred?

Does anything make the symptoms better or worse,

such as time of day, menstrual cycle, posture or

medication?

Associated symptoms

Associated symptoms can aid diagnosis. For example, headache

may be associated with nausea, vomiting, photophobia (aversion

to light) and/or phonophobia (aversion to sound) in migraine;

headache with neck stiffness, fever and rash may be associated

with meningitis (Box 7.1).

Common presenting symptoms

Headache

Headache is the most common neurological symptom and

may be either primary or secondary to other pathology. Primary

(idiopathic) causes include:

migraine

tension-type headache

trigeminal autonomic cephalalgias (including cluster

headache)

primary stabbing, cough, exertional or sex headache

primary thunderclap headache

new daily persistent headache.

Secondary (or symptomatic) headaches are less common,

but include potentially life-threatening or disabling causes such

as subarachnoid haemorrhage or temporal arteritis. One of the

key history aspects is rapidity of onset; isolated headache with

a truly abrupt onset may represent a potentially serious cause

such as subarachnoid haemorrhage or cerebral vein thrombosis,

whereas recurrent headache is much more likely to be migraine,

particularly if associated with other migrainous features like aura,

nausea and/or vomiting, photophobia and phonophobia (Box

7.1). Asking patents what they do when they have a headache

can be instructive. For example, abandoning normal tasks and

seeking a bed in a dark, quiet room suggest migraine, whereas

pacing around the room in an agitated state, or even head

banging, suggests cluster headache.

Transient loss of consciousness

Syncope is loss of consciousness due to inadequate cerebral

perfusion and is the most common cause of transient loss of

consciousness (TLOC). Vasovagal (or reflex) syncope (fainting)

is the most common type and precipitated by stimulation of the

parasympathetic nervous system, as with pain or intercurrent

illness. Exercise-related syncope, or syncope with no warning or

trigger, suggests a possible cardiac cause. TLOC on standing

is suggestive of orthostatic (postural) hypotension and may be

caused by drugs (antihypertensives or levodopa) or associated

with autonomic neuropathies, which may complicate conditions

such as diabetes.

7.2 Features that help discriminate vasovagal syncope

from epileptic seizure

Feature Vasovagal syncope Seizure

Triggers Typically pain, illness,

emotion

Often none (sleep

deprivation, alcohol,

drugs)

Prodrome Feeling faint/

lightheaded, nausea,

tinnitus, vision

dimming

Focal onset (not always

present)

Duration of

unconsciousness

<60 s 1–2 mins

Convulsion May occur but usually

brief myoclonic jerks

Usual, tonic–clonic

1–2 mins

Colour Pale/grey Flushed/cyanosed, may

be pale

Injuries Uncommon,

sometimes biting of

tip of tongue

Lateral tongue biting,

headache, generalised

myalgia, back pain

(sometimes vertebral

compression fractures),

shoulder fracture/

dislocation (rare)

Recovery Rapid, no confusion Gradual, over 30 mins;

patient is often confused,

sometimes agitated/

aggressive, amnesic

The history • 123

7

than ischaemia include use of anticoagulation, headache, vomiting,

seizures and early reduced consciousness. Haemorrhagic stroke

is much more frequent in Asian populations. Spinal strokes are

very rare; patients typically present with abrupt bilateral paralysis,

depending on the level of spinal cord affected. The anterior

spinal artery syndrome is most common and causes loss of

motor function and pain/temperature sensation, with relative

sparing of joint position and vibration sensation below the level

of the lesion.

Dizziness and vertigo

Patients use ‘dizziness’ to describe many sensations. Recurrent

‘dizzy spells’ affect approximately 30% of those over 65 years

and can be due to postural hypotension, cerebrovascular disease,

cardiac arrhythmia or hyperventilation induced by anxiety and

panic. Vertigo (the illusion of movement) specifically indicates a

problem in the vestibular apparatus (peripheral) or, much less

commonly, the brain (central) (see Box 9.3 and p. 174). TIAs

do not cause isolated vertigo. Identifying a specific cause of

dizziness is often challenging but may be rewarding in some

cases, including benign paroxysmal positional vertigo (BPPV),

which is eminently treatable. As a guide, recurrent episodes of

vertigo lasting a few seconds are most likely to be due to BPPV;

vertigo lasting minutes or hours may be caused by Ménière’s

disease (with associated symptoms including hearing loss, tinnitus,

nausea and vomiting) or migrainous vertigo (with or without

headache).

Functional neurological symptoms

Many neurological symptoms are not due to disease. These

symptoms are often called ‘functional’ but other (less useful

and more pejorative) terms include psychogenic, hysterical,

somatisation or conversion disorders. Presentations include

blindness, tremor, weakness and collapsing attacks, and patients

will often describe numerous other symptoms, with fatigue,

lethargy, pain, anxiety and other mood disorders commonly

associated. Diagnosing functional symptoms requires experience

and patience (p. 363). Clues include symptoms not compatible

with disease (such as retained awareness of convulsing during

non-epileptic attacks, or being able to walk normally backwards

but not forwards), considerable variability in symptoms (such

as intermittent recovery of a hemiparesis), multiple symptoms

(often with numerous previous assessments by other specialties,

particularly gynaecology, gastroenterology, ear, nose and throat

and cardiorespiratory) and multiple unremarkable investigations,

leading to numerous different diagnoses. The size of a patient’s

case notes can sometimes be a clue in itself! Beware of labelling

symptoms as functional simply because they appear odd or

inexplicable. Like disease, most functional neurological disorders

follow recognisable patterns, so be cautious when the pattern

is atypical.

Past medical history

Symptoms that the patient has forgotten about or overlooked may

be important; for example, a history of previous visual loss (optic

neuritis) in someone presenting with numbness suggests multiple

sclerosis. Birth history and development may be significant, as

in epilepsy. Contact parents or family doctors to obtain such

information. If considering a vascular cause of neurological

symptoms, ask about important risk factors, such as other

vascular disease, hypertension, family history and smoking.

Stroke and transient ischaemic attack

A stroke is a focal neurological deficit of rapid onset that is

due to a vascular cause. A transient ischaemic attack (TIA) is

the same but symptoms resolve within 24 hours. TIAs are an

important risk factor for impending stroke and demand urgent

assessment and treatment. Hemiplegia following middle cerebral

artery occlusion is a typical example but symptoms are dictated by

the vascular territory involved. Much of the cerebral hemispheres

are supplied by the anterior circulation (the anterior and middle

cerebral arteries are derived from the internal carotid artery), while

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