The neurological history begins in detail with the presenting problem or problems (see List 31.1). The patient should be allowed to describe the symptoms in his or her own words to begin with, and then the clinician needs to ask questions to clarify information and obtain more detail. It is particularly important to ascertain the temporal course of the illness, as this may give important information about the underlying aetiology.
An acute onset of symptoms (within minutes to an hour) is suggestive of a vascular or convulsive problem (e.g. the explosive severe headache of subarachnoid haemorrhage or the rapid onset of a seizure).
These episodes of sudden onset may feature a precipitating event (e.g. exercise) or warning (aura) may be present. The aura that precedes a seizure may be localising (e.g. auditory hallucinations, an unusual smell or taste, loss of speech or motor changes) or non-localising (e.g. a feeling of apprehension). The occurrence of an aura followed by sudden unconsciousness is very suggestive of the diagnosis of a major seizure or complex partial seizure.
A stroke or cerebrovascular accident usually causes symptoms that appear over minutes or are present when the patient wakes from sleep. There is a focal problem with function of the brain. Patients may be unable to move one side of the body (hemiplegia) or have difficulty with speech or swallowing. There may have been previous episodes. When there is resolution of the symptoms within 24 hours the episode is called a transient ischaemic attack (TIA). The rapid onset of focal symptoms almost always has a vascular cause: embolism, infarction or haemorrhage. If the patient can answer questions it is important to ask about the onset of the symptoms and about risk factors for stroke (see Questions box 31.1).
The sudden onset of weakness on one side of the body followed by resolution and a severe headache is characteristic of hemiplegic migraine, but in elderly patients especially there may be no headache. This makes the distinction from a transient ischaemic episode difficult. The very gradual onset of muscle weakness suggests a muscle abnormality such as myopathy rather than a vascular event.
A subacute onset (hours to days) occurs with inflammatory disorders (e.g. meningitis, cerebral abscess or the Guillain-Barréa syndrome—acute inflammatory polyradiculoneuropathy).
A more chronic symptom course suggests that the underlying disorder may be related to either a tumour (weeks to months) or a degenerative process (months to years). Metabolic or toxic disorders may present with any of these time courses.
Based on the history (and physical examination), a judgement is made as to whether the disease process is localised or diffuse, and which levels of the nervous system are involved (the nervous system may be thought of as having four different levels: the peripheral nervous system, the spinal cord, the posterior fossa and the cerebral hemispheres). Consideration of the time course and the levels of involvement will usually lead to a logical differential diagnosis of the patient’s symptoms. After detailed questions about the presenting problem, ask about previous neurological symptoms and about previous neurological diagnoses or investigations. The patient may know the results of CT or magnetic resonance imaging brain scans performed in the past. A thorough neurological history will include routine questions about possible neurological symptoms (see Questions box 31.2). If the patient answers ‘yes’ to any of these, more-detailed questions about the nature of the problem and its time course are indicated.
Headache is a very common symptom (see Questions box 31.3). It is important, as with any type of pain, to determine the character, severity, site, duration, frequency, radiation, aggravating and relieving factors and associated symptoms.1,2 Unilateral headache that is preceded by flashing lights or zigzag lines and is associated with light hurting the eyes (photophobia) is likely to be a migraine with an aura (‘classical migraine’); common migraine has no aura. Pain over one eye (or over the temple) lasting for minutes to hours, associated with lacrimation, rhinorrhoea and flushing of the forehead, and occurring in bouts that last several weeks a few times a year or less, is suggestive of cluster headache. This occurs predominantly in males and patients cannot stay still. Headache over the occiput and associated with neck stiffness may be from cervical spondylosis. Coital headache occurs during intercourse close to orgasm. This sudden severe headache affects middle-aged men. It is of sudden onset and lasts in its severe form for about 15 minutes and can persist as a milder discomfort for a few hours. There is no nausea or neck stiffness. It is benign, the cause is unknown and it needs to be distinguished from sub-arachnoid haemorrhage, which can also occur during sexual intercourse.
A generalised headache that is worse in the morning and is associated with drowsiness or vomiting may reflect raised intracranial pressure, while generalised headache associated with photophobia and fever as well as with a stiff neck of more gradual onset may be due to meningitis. A persistent unilateral headache over the temporal area associated with tenderness over the temporal artery and blurring of vision or diplopia suggests temporal arteritis.3,4 This condition (see Good signs guide 31.1) is often associated with jaw claudication, or jaw pain during eating, which can lead to considerable loss of weight. Headache with pain or fullness behind the eyes or over the cheeks or forehead occurs in acute sinusitis. The dramatic and usually instantaneous onset of severe headache that is initially localised but becomes generalised and is associated with neck stiffness may be due to a subarachnoid haemorrhage. Morning headaches worse with coughing, especially in an obese patient, may be due to idiopathic intracranial hypertension; visual loss may occur.
Finally, the most frequent type of headache is episodic or chronic tension-type headache; this is commonly bilateral, occurs over the frontal, occipital or temporal areas and may be described as a sensation of tightness that lasts for hours and recurs often. There are usually no associated symptoms such as nausea, vomiting, weakness or paraesthesias (tingling in the limbs), and the headache does not usually wake the patient at night from sleep.
Remember the mnemonic POUND when trying to differentiate migraine headache from tension headache:
If there are 4–5 of these features, the LR+ for migraine is an impressive 24.5
Pain in the face can result from trigeminal neuralgia, temporomandibular arthritis, glaucoma, cluster headache, temporal arteritis, psychiatric disease, aneurysm of the internal carotid or posterior communicating artery, or the superior orbital fissure syndrome.
It is important to try to differentiate syncope (transient loss of consciousness) from epilepsy (see Questions box 31.4). However, primary syncopal events can cause a few clonic jerks in a significant number of cases.b Generalised tonic–clonic seizures (major seizures—grand mal epilepsy) cause abrupt loss of consciousness, which may be preceded by an aura. Often the patient is incontinent of urine and faeces, and the tongue may be bitten. A witness may be able to describe the type of attack that occurred. It is important to try to determine whether any seizure is generalised or localised to one side of the body: a seizure affecting part of the body may indicate a focal lesion in the central nervous system, such as a tumour or an abscess. If consciousness is impaired, these partial seizures are described as ‘complex’; if consciousness is unimpaired, they are termed ‘simple’. Idiopathic absence seizures (‘petit mal’) occur in children. These are frequent brief episodes of loss of awareness often associated with staring. Major motor movements do not occur with this type of epilepsy.
Transient ischaemic attacks affecting the brainstem can occasionally cause blackouts. Use of the term ‘drop attacks’ means the patient falls but there is no loss of consciousness. In either case the patient falls to the ground without premonition and the attacks are of brief duration. Hypoglycaemia can lead to episodes of loss of consciousness. Patients with hypoglycaemia may also report sweating, weakness and confusion before losing consciousness. They are usually diabetics who are taking insulin or an oral hypoglycaemic drug. Bizarre attacks of loss of consciousness occur with hysteriac During such attacks the patient may slump to the ground without sustaining any injury and there may be apparent fluctuations in the level of consciousness for a prolonged period.
It is important to determine what the patient means by the term dizziness.d In true vertigo, there is actually a sense of motion, usually of the surroundings but also of the head itself.6 When vertigo is severe it may not be possible for the patient to stand or walk, and associated symptoms of nausea, vomiting, pallor, sweating and headache may be present. The presence or absence of deafness and the time course of the vertigo can help with the diagnosis (see Table 31.1). Causes of vertigo include the ‘peripheral vestibular lesions’:
TABLE 31.1
The history and cause of vertigo
| Symptoms | Likely diagnosis |
| Persistent vertigo without hearing loss | Vestibular neuronitis |
| Intermittent vertigo without hearing loss | Benign positioning vertigo |
| Persistent vertigo with hearing loss | Labyrinthitis |
| Intermittent vertigo and tinnitus with hearing loss | Meniere’s disease |
• benign positioning (positional) vertigo—recurrent brief episodes of vertigo precipitated by a change of head position, due to crystals in the saccule and utricle
• vestibular neuronitis—non-positional vertigo due to inflammation of the acoustic nerve with normal hearing
Other causes of vertigo include:
• ototoxic drugs (e.g. aminoglycosides), associated with deafness or tinnitus
• Ménière’se disease, which occurs in those over 50 years of age and presents with the triad of episodic vertigo and tinnitus (ringing in the ears) with progressive deafness
• acoustic neuroma (where patients may also have deafness and tinnitus)
• central causes such as vertebrobasilar TIAs—these may be associated with diplopia (double vision; page 411), visual loss and ataxia; and multiple sclerosis and cerebellar tumours
Problems with vision can include double vision (diplopia), blurred vision (amblyopia), light intolerance (photophobia) and visual loss. The causes of deafness are summarised on page 421.
Many neurological conditions can make walking difficult. These are described on page 458. Walking may also be abnormal when orthopaedic disease affects the lower limbs or spine. A bizarrely abnormal gait can sometimes be a sign of a hysterical reaction.
Pins and needles in the hands or feet may indicate nerve entrapment or a peripheral neuropathy (page 445) but can result from sensory pathway involvement at any level. The carpal tunnel syndrome is common: here there is median nerve entrapment, and patients experience pain and paraesthesias in the hand and wrist. Sometimes pain may extend to the arm and even to the shoulder, but paraesthesias are felt only in the fingers. These symptoms are usually worse at night and may be relieved by dangling the arm over the side of the bed or shaking the hand (the flick sign).
Nerve root, spinal cord and cerebral abnormalities can all cause disturbance of sensation and weakness.
Limb weakness can be caused by lesions at different levels in the motor system. There are a number of patterns of limb and muscle weakness:
• Upper motor neurone (UMN) weakness (page 436) is due to interruption of a neural pathway at a level above the anterior horn cell. The result is an increase in tone and peripheral reflexes. Interruption of this pathway has the greatest effect on the antigravity muscles and is called pyramidal weakness. There is little or no muscle wasting.
• Lower motor neurone (LMN) weakness (page 437) is due to a lesion that interrupts the reflex arc between the anterior horn cell and the muscle. There is a reduction in tone and reflexes, fasciculation (irregular contractions of small areas of muscle) may be seen and muscle wasting is prominent.
• Muscle disease causes weakness in a particular muscle or group of muscles. There is wasting and decreased tone, and the reflexes are reduced or absent.
• Disease at the neuromuscular junction (e.g. myasthenia gravis, page 471) causes generalised weakness, which worsens with repetition. The reflexes and tone are often normal.
• Non-organic weakness (e.g. due to hysteria) causes a non-anatomical pattern of weakness in association with normal tone and power and, unless there has been prolonged disuse, normal muscle bulk.
Tremor is a rhythmical movement (see Table 31.2). A slow tremor has, by definition, a rate between 3 Hz and 5 Hz. Rapid tremors are faster than 10 Hz. Resting tremors are present mostly during relaxation of the muscles, while intention tremors occur with deliberate movement and become more pronounced towards the end of the action. Tremors become worse with fatigue or anxiety. Shivering is a type of tremor brought on by cold. It is normal for there to be a fine tremor associated with holding a posture or performing a movement slowly. This is called a physiological tremor. It becomes more obvious with fright and fatigue. It is often increased by the beta-agonist drugs used to treat asthma or by caffeine. Thyrotoxicosis is a cause of exaggeration of physiological tremor. These movements are very fine and may be difficult to see unless looked for specifically. Benign essential (familial) tremor is an inherited disorder that causes tremor, but no other signs. The tremor is most easily seen when the patient’s arms are stretched out; it can become worse during voluntary movements. It usually disappears when the muscles are at complete rest. Parkinson’sf disease may present with a resting tremor (page 475). Intention (or target-seeking) tremor is due to cerebellar disease (page 453). Chorea involves involuntary jerky movements (page 478). Definitions of the terms used to describe movement disorders are shown inTable 31.3.
TABLE 31.2
| Parkinson’s disease | 3 to 5 Hz |
| Essential/familial | 4 to 7 Hz |
| Physiological | 8 to 13 Hz |
TABLE 31.3
Definitions of terms used to describe movement disorders
| Akithesia | Motor restlessness; constant semi-purposeful movements of the arms and legs |
| Asterixis | Sudden loss of muscle tone during sustained contraction of an outstretched limb |
| Athetosis | Writhing, slow sinuous movements, especially of the hands and wrists |
| Chorea | Jerky small rapid movements, often disguised by the patient with a purposeful final movement (e.g. the jerky upward arm movement is transformed into a voluntary movement to scratch the head) |
| Dyskinesia | Purposeless and continuous movements, often of the face and mouth; frequently a result of treatment with major tranquillisers for psychotic illness |
| Dystonia | Sustained contractions of groups of agonist and antagonist muscles, usually in flexion or extremes of extension; it results in bizarre postures |
| Hemiballismus | An exaggerated form of chorea involving one side of the body: there are wild flinging movements that can injure the patient (or bystanders) |
| Myoclonic jerk | A brief muscle contraction that causes a sudden purposeless jerking of a limb |
| Myokymia | A repeated contraction of a small muscle group; often involves the orbicularis oculi muscles |
| Tic | A repetitive irresistible movement that is purposeful or semi-purposeful |
| Tremor | A rhythmical alternating movement |
Speech may be disturbed by many different neurological diseases and is discussed on page 430. A number of different diseases can also result in delirium or dementia (see Chapter 37).
Enquire about a past history of meningitis or encephalitis, head or spinal injuries, a history of epilepsy or convulsions and any previous operations. Any past history of sexually transmitted infection (e.g. risk factors for HIV infection or syphilis) should be obtained. Ask about risk factors that may predispose to the development of cerebrovascular disease (see List 31.1). A previous diagnosis of peripheral vascular disease or of coronary artery disease indicates an increased risk of cerebrovascular disease. Chronic or paroxysmal atrial fibrillation is associated with a greatly increased risk of embolic stroke, especially for people over the age of 70.
Previous and current medications may be the cause of certain neurological or apparently neurological syndromes (see List 31.2).
Ask about treatment for neurological disorders with anticonvulsants, anti-Parkinsonian drugs, steroids, immunosuppressants, biological agents, anticoagulants, antiplatelet agents and for other drug treatment that may be associated with neurological problems; use of the contraceptive pill; and use of antihypertensive agents.
Since smoking predisposes to cerebrovascular disease, the smoking history is relevant. It is useful to ask about occupation and exposure to toxins (e.g. heavy metals). Alcohol can also result in a number of neurological diseases (see List 1.3, page 12). Many neurological diseases affect people’s ability to work and look after themselves. In these cases, questions about financial security, living conditions and availability of help at home become very important.
Any history of neurological or mental disease should be documented. A number of important neurological conditions are inherited (see Table 31.4).
TABLE 31.4
Examples of inherited neurological conditions
| X-linked | Colour blindness, Duchenne’s and Becker’s muscular dystrophy, Leber’s* optic atrophy |
| Autosomal dominant | Huntington’s chorea, tuberose sclerosis, dystrophia myotonica |
| Autosomal recessive | Wilson’s disease, Refsum’s† disease, Freiderich’s ataxia, Tay-Sachs’‡ disease |
| Increased incidence in families | Alzheimer’s§ disease (autosomal dominant, increased risk with ApoE epsilon 4) |
*Theodor Karl von Leber (1840-1917), German ophthalmologist, professor of ophthalmology at Heidelberg. He began studying chemistry but was advised by Bunsen that there were too many chemists and so changed to studying medicine.
†Siguald Refsum (1907-91), Norwegian neurologist. He described this in 1945, calling it heredotaxia hemerlopica polyneuritiformis.
‡Warren Tay (1843-1927), English ophthalmologist, described the ophthalmological abnormalities of the condition. Bernard Sachs (1858-1944), American neurologist and psychiatrist, described the neurological features in 1887. He studied in Germany and was a pupil of von Recklinghausen. The condition was originally called amaurotic family idiocy. This may be another reason to use eponymous names.
§Alois Alzheimer (1864-1915), Bavarian neuropathologist, described the condition in 1906. His doctoral thesis was on the wax-producing glands of the ear.
1. Sturm, JW, Donnan, GA. Diagnosis and investigation of headache. Aust Fam Phys. 1998; 27:587–589. [An accurate clinical history is the key to diagnosing the cause of headache.].
2. Marks, DR, Rapoport, AM. Practical evaluation and diagnosis of headache. Seminars in Neurology. 1997; 17:307–312. [The history should include information about the onset, intensity, associated autonomic symptoms and trigger factors of headache. Special attention must be paid to the frequency of analgesic use, both prescription and over-the-counter, to identify analgesic rebound headache.].
3. Hellmann, D. Temporal arteritis: a cough, headache and tongue infarction. JAMA. 2002; 287:2996–3000.
4. Smetana, GW, Shmerling, RH. Does this patient have temporal arteritis. JAMA. 2002; 287:92–101.
5. Detsky, ME, McDonald, DR, Baerlocher, MR. Does this patient with a headache have a migraine or need neuroimaging. JAMA. 2006; 296:1274–1283.
6. Froehling, DA, Silverstein, MD, Mohr, DN, Beatty, CW. Does this dizzy patient have a serious form of vertigo. JAMA. 1994; 271:385–388. [Helps the clinician distinguish vertigo from other causes of dizziness.].
aGeorges Guillain (1876–1961), Jean Alexandre Barré (1880–1967) and A Strohl described the syndrome in 1916; Strohl’s name was dropped because of anti-German feeling during World War I.
bThe diagnosis of the cause of transient loss of consciousness can be difficult. Patients who have seen a cardiologist first are often told the cause is neurological, while those who have seen a neurologist first are often told that it is cardiac in origin.
cHysteria is an old but still popular term that refers to a presumed psychogenic condition.
dThe word comes from the Old English word dysig, meaning ‘stupid’.
eProsper Ménière (1799–1862), director of the Paris Institution for Deaf-Mutes, characterised this condition just before he died of post-influenzal pneumonia. He was an expert on orchids and a friend of Victor Hugo and Honoré de Balzac. He added the grave accent on the second ‘e’ in his name; the acute accent on the first ‘e’ was added by his son.
fJames Parkinson (1755–1824), English general practitioner, published an essay on ‘The Shaking Palsy’ in 1817. He was nearly transported to Australia for reformist activities.