Neurology: Spine and Sleep Disorders

• Spinal cord: Degenerative Cervical Myelopathy | Other Spinal Cord Disorders
• Dizziness and sleep: Chronic and Recurrent Dizziness | Narcolepsy and Sleep Disorders
• Other neurology: Neurocutaneous Syndromes | Chronic Neurological Infections | Neurology of Systemic Disease | Functional Neurological Disorder | Driving and Neurological Conditions
• Revision: Drug Reference Table | Spot the Diagnosis | Exam-style Scenarios | Key Points | Guidelines and Further Reading

🧭 This chapter brings together spinal cord disorders, dizziness, sleep disorders, neurocutaneous syndromes, chronic CNS infections, the neurology of systemic disease, functional neurological disorder and driving rules. Acute spinal cord compression, meningitis and encephalitis are covered in the Neurological Emergencies chapter.
Your four questions for every patient: Is there a structural cause that needs imaging? Is this a treatable complication of a systemic disease, drug or infection? Is the problem functional - and have I made a positive diagnosis rather than just excluding things? What does the patient need to know about driving and safety?

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Degenerative Cervical Myelopathy

What it is: Compression of the cervical spinal cord by age-related degeneration - disc bulges, osteophytes, thickened ligaments, or ossification of the posterior longitudinal ligament. It is the commonest cause of spinal cord dysfunction in adults, and is often diagnosed late.

Symptoms (gradual, often stepwise):

• Clumsy, numb hands - difficulty with buttons, writing, dropping things.
• Unsteady gait; falls.
• Neck pain and stiffness (not always).
• Lhermitte's sign.
• Urinary urgency (late).

Signs:

• UMN signs in the legs - spasticity, brisk reflexes, extensor plantars.
• In the arms:
  • Wasting of the hand muscles.
  • Hoffmann's sign.
  • Inverted supinator reflex (C5–6).
• Patchy sensory loss in the hands.

Investigation: MRI cervical spine (cord compression and signal change). Exclude B12 and copper deficiency and MND.

Management:

• Moderate or severe, or progressive - surgical decompression (anterior discectomy and fusion, or posterior laminoplasty or laminectomy). The aim is to stop progression; recovery of existing deficits is partial.
• Mild - may be monitored or offered surgery.
• Avoid neck manipulation. A minor fall can cause acute cord injury (central cord syndrome).
• Refer early - delay worsens outcomes.

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Other Spinal Cord Disorders

• Syringomyelia and Chiari malformation:
  • A syrinx is a fluid-filled cavity in the cord, often with a Chiari I malformation (cerebellar tonsils pushed down through the foramen magnum).
  • Features:
    • "Cape-like" loss of pain and temperature across the shoulders and arms (crossing fibres damaged), with light touch and vibration preserved.
    • Wasting of the hands, loss of arm reflexes.
    • Painless burns.
    • Scoliosis.
    • Later UMN signs in the legs.
    • Horner's syndrome.
  • Chiari symptoms: Occipital headache on coughing or straining; downbeat nystagmus; ataxia.
  • Treatment: Neurosurgical decompression of the foramen magnum.
• Subacute combined degeneration of the cord (B12 deficiency):
  • Dorsal columns (loss of vibration and joint position, sensory ataxia, positive Romberg's) plus corticospinal tracts (extensor plantars), often with neuropathy (absent ankle jerks).
  • Anaemia may be absent.
  • Nitrous oxide ("laughing gas") misuse causes it in young people - even with a normal B12 level (check homocysteine and methylmalonic acid).
  • Treat urgently with IM hydroxocobalamin (1 mg on alternate days until no further improvement, then every 2 months).
• Copper deficiency myelopathy: A similar picture, with anaemia and neutropenia; after bariatric surgery or excess zinc.
• Spinal dural arteriovenous fistula:
  • Usually older men.
  • Slowly progressive paraparesis, sensory loss and bladder problems, often worse with exercise or standing.
  • MRI: cord swelling with dilated vessels ("flow voids") on the cord surface; spinal angiography.
  • Treatable by embolisation or surgery - don't miss it.
• Spinal cord infarction: Sudden onset - anterior spinal artery syndrome (weakness and loss of pain sensation, with joint position preserved), after aortic surgery or dissection.
• Hereditary spastic paraplegia: Slowly progressive spastic legs with a family history.
• HTLV-1-associated myelopathy (tropical spastic paraparesis) - people from the Caribbean, Japan, West Africa and South America.
• Radiation myelopathy; cord tumours (ependymoma, astrocytoma, meningioma, schwannoma).
• Transverse myelitis and MS - see the MS chapter.

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Chronic and Recurrent Dizziness

First, what does "dizzy" mean?

• Vertigo - a false sense of movement (spinning) - vestibular.
• Presyncope - lightheaded, about to faint - cardiovascular.
• Imbalance - unsteady on the feet - neurological or multifactorial.
• Vague dizziness - anxiety, drugs, PPPD.

Acute severe vertigo (stroke vs vestibular neuritis - HINTS) is covered in the Stroke chapter.

• Benign paroxysmal positional vertigo (BPPV):
  • The commonest cause of vertigo.
  • Brief spells (under a minute) of vertigo triggered by head movements - rolling over in bed, looking up.
  • Dix–Hallpike test - rotatory (torsional) nystagmus after a short delay, which fades.
  • Treatment: Epley manoeuvre (cures most). Vestibular sedatives don't help.
  • Common after head injury and in older people.
• Vestibular neuritis / labyrinthitis:
  • Sudden prolonged vertigo for days, with gradual recovery. With hearing loss = labyrinthitis.
  • Short course of prochlorperazine or cyclizine (no more than 3 days).
  • Early vestibular rehabilitation exercises.
• Ménière's disease:
  • Attacks of vertigo lasting 20 minutes to 12 hours, with fluctuating low-frequency hearing loss, tinnitus and ear fullness.
  • Progressive hearing loss over years.
  • Management:
    • ENT referral; audiometry.
    • Acute attacks: prochlorperazine.
    • Prevention: betahistine (limited evidence).
    • Intratympanic treatments.
    • Tell the DVLA.
• Vestibular migraine:
  • Recurrent vertigo lasting minutes to days, with migraine features (headache, light sensitivity) in at least some attacks.
  • Very common.
  • Treat as migraine (lifestyle, preventives).
• Persistent postural-perceptual dizziness (PPPD):
  • Chronic dizziness and unsteadiness for over 3 months, worse when upright, with motion, and in busy visual environments (supermarkets).
  • Often follows an acute vestibular event.
  • Vestibular rehabilitation, CBT, SSRIs.
• Vestibular schwannoma: One-sided progressive hearing loss and tinnitus (rarely vertigo) - MRI internal auditory meatus.
• Bilateral vestibular failure: Imbalance worse in the dark, and oscillopsia when walking. Often from gentamicin toxicity.
• Older people: Dizziness is often multifactorial:
  • Postural hypotension (drugs).
  • Neuropathy.
  • Poor vision.
  • Vestibular loss.
  • Cervical myelopathy.
  • Fear of falling.
  • A falls assessment and drug review help most.

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Narcolepsy and Sleep Disorders

• Narcolepsy:
  • Onset usually in teens or twenties.
  • Excessive daytime sleepiness with irresistible sleep attacks.
  • Cataplexy (type 1) - sudden loss of muscle tone triggered by emotion (laughter, surprise) with consciousness preserved - knees buckle, face sags.
  • Sleep paralysis and hypnagogic hallucinations (on falling asleep).
  • Disturbed night sleep.
  • Type 1 - low CSF orexin (hypocretin); linked to HLA-DQB1*06:02.
  • Diagnosis: Overnight sleep study followed by a multiple sleep latency test (falls asleep quickly, with early REM sleep).
  • Treatment:
    • Scheduled naps; sleep hygiene.
    • Modafinil, pitolisant, solriamfetol, methylphenidate.
    • Sodium oxybate (cataplexy and sleepiness).
    • Antidepressants (venlafaxine, clomipramine) for cataplexy.
  • Driving: Tell the DVLA; stop driving until symptoms are controlled.
• Other causes of daytime sleepiness:
  • Obstructive sleep apnoea (see the Pulmonary Vascular and Sleep chapter).
  • Insufficient sleep.
  • Drugs (sedatives, dopamine agonists).
  • Depression.
  • Hypothyroidism.
  • Idiopathic hypersomnia.
• REM sleep behaviour disorder:
  • Acting out dreams - punching, kicking, shouting (normal REM paralysis is lost).
  • Often men over 50.
  • Strongly predicts Parkinson's disease, DLB or MSA (over 70% within 10–15 years).
  • Bed safety; melatonin or clonazepam.
  • Can be triggered by antidepressants.
• Non-REM parasomnias (sleepwalking, night terrors, confusional arousals): Common in children; occur early in the night; little memory afterwards.
• Restless legs syndrome - see the Movement Disorders chapter.
• Insomnia: CBT for insomnia is first line; avoid long-term hypnotics.

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Neurocutaneous Syndromes

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Chronic Neurological Infections

• Neurosyphilis:
  • Can occur at any stage.
  • Forms:
    • Meningitis.
    • Strokes (meningovascular).
    • General paresis - dementia, personality change, psychosis.
    • Tabes dorsalis - dorsal column loss, lightning pains, Charcot joints.
    • Argyll Robertson pupils.
    • Ocular and auditory syphilis.
  • Tests: Serum treponemal tests; CSF examination.
  • Treatment: IV benzylpenicillin for 14 days (or procaine penicillin with probenecid); test for HIV.
• HIV-related neurology (especially with a low CD4 count):
  • Cerebral toxoplasmosis - multiple ring-enhancing lesions (basal ganglia), CD4 under 100. Pyrimethamine with sulfadiazine; response within 2 weeks supports the diagnosis.
  • Primary CNS lymphoma - EBV-related; single or few lesions.
  • Cryptococcal meningitis - headache, raised pressure; CSF antigen; amphotericin with flucytosine; repeated LPs for pressure.
  • PML - JC virus; non-enhancing white matter lesions.
  • HIV-associated neurocognitive disorder; distal sensory neuropathy; myelopathy.
  • Offer HIV testing in any unexplained neurological illness.
• TB meningitis:
  • Subacute (weeks) headache, fever, confusion, cranial nerve palsies, strokes, hydrocephalus.
  • CSF: Lymphocytes, very high protein, low glucose.
  • Treat early on suspicion - RHZE for 12 months plus dexamethasone.
• Lyme neuroborreliosis (tick bites - New Forest, Scottish Highlands):
  • Facial palsy (can be bilateral).
  • Painful radiculitis.
  • Lymphocytic meningitis.
  • Heart block.
  • Treatment: Doxycycline (or IV ceftriaxone).
• Neurocysticercosis:
  • Pork tapeworm larvae in the brain - the commonest cause of acquired epilepsy worldwide (travellers and migrants from Latin America, Asia and Africa).
  • Cysts and calcifications on imaging.
  • Albendazole with steroids; antiseizure medicines.
• Brain abscess:
  • From the sinuses, teeth or ears, the blood (endocarditis, lung AVMs in HHT, congenital heart disease) or after surgery.
  • Streptococcus anginosus group, anaerobes, staphylococci.
  • Headache, fever (often absent), focal signs, seizures.
  • Ring-enhancing lesion with restricted diffusion on MRI.
  • Neurosurgical aspiration plus prolonged IV antibiotics (e.g. ceftriaxone with metronidazole).
• Varicella zoster: Post-herpetic neuralgia; Ramsay Hunt syndrome; VZV vasculopathy (strokes weeks to months after shingles, especially ophthalmic zoster).
• Botulism:
  • Descending flaccid paralysis - eye muscles, bulbar, then limbs.
  • Dilated pupils; normal sensation.
  • Wound botulism in people who inject drugs.
  • Antitoxin urgently (via UKHSA); ventilatory support.
• Tetanus:
  • Muscle spasms; lockjaw (trismus); risus sardonicus.
  • Dirty wounds; injecting drug use.
  • Immunoglobulin, metronidazole, wound care, ITU.

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Neurology of Systemic Disease

• Alcohol:
  • Wernicke's encephalopathy (confusion, ataxia, eye signs - thiamine).
  • Korsakoff syndrome.
  • Cerebellar degeneration (gait ataxia).
  • Peripheral neuropathy; myopathy.
  • Withdrawal seizures.
  • Osmotic demyelination syndrome.
  • Subdural haematoma.
  • Marchiafava–Bignami disease (rare).
• Osmotic demyelination syndrome (central pontine myelinolysis):
  • After correcting chronic hyponatraemia too quickly.
  • Days later: quadriparesis, dysarthria, dysphagia, "locked-in" syndrome.
  • Risk factors: alcohol, malnutrition, low potassium, liver disease.
  • Prevention: limit the sodium rise (no more than 8–10 mmol/L in 24 hours; lower in high-risk patients).
• Posterior reversible encephalopathy syndrome (PRES):
  • Headache, seizures, visual loss, confusion.
  • Triggers: severe hypertension, eclampsia, ciclosporin and tacrolimus, chemotherapy, kidney failure, sepsis.
  • MRI: Posterior (parieto-occipital) white matter oedema.
  • Treatment: Control BP; stop the trigger drug; treat seizures. Usually reversible.
• Metabolic encephalopathy:
  • Uraemia; liver failure (asterixis).
  • Hypercapnia.
  • Low or high glucose, sodium or calcium.
  • Thyroid disease (myxoedema coma; Hashimoto's encephalopathy).
• Endocrine:
  • Hypothyroidism - carpal tunnel, myopathy, ataxia, slowed reflexes.
  • Thyrotoxicosis - tremor, myopathy, periodic paralysis.
  • Cushing's - myopathy.
  • Acromegaly - carpal tunnel.
  • Hypercalcaemia - confusion.
• Connective tissue disease:
  • SLE - seizures, psychosis, strokes (antiphospholipid), chorea, myelitis, neuropathy.
  • Sjögren's - sensory neuronopathy, small fibre neuropathy.
  • Rheumatoid arthritis - atlantoaxial subluxation (cord compression), carpal tunnel, vasculitic neuropathy.
• Cancer treatment:
  • Chemotherapy neuropathy.
  • Radiation necrosis and late cognitive decline.
  • Immune checkpoint inhibitor toxicity (myasthenia, myositis, encephalitis, GBS).
  • CAR-T cell neurotoxicity (ICANS).
• Coeliac disease - ataxia, neuropathy, epilepsy with occipital calcification.
• Pregnancy - eclampsia, PRES, venous sinus thrombosis, pituitary apoplexy, carpal tunnel, meralgia paraesthetica, Bell's palsy, worsening of myasthenia.

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Functional Neurological Disorder

What it is: Genuine neurological symptoms caused by a problem with how the nervous system functions, rather than structural damage. It is one of the commonest reasons for neurology referral, and can be as disabling as MS or Parkinson's.

Forms:

• Functional limb weakness.
• Dissociative (non-epileptic) seizures - see the Epilepsy chapter.
• Functional movement disorders - see the Movement Disorders chapter.
• Functional sensory symptoms.
• Functional speech and swallowing problems.
• Functional cognitive disorder.
• Persistent postural-perceptual dizziness (PPPD).
• Often with fatigue, pain and poor sleep.

Positive signs - the diagnosis is made on these, not just on normal tests:

• Hoover's sign - weak hip extension that returns to normal when the patient flexes the opposite hip against resistance.
• Hip abductor sign.
• Give-way weakness (collapsing weakness with normal power for a moment).
• Drift without pronation.
• Dragging monoplegic gait.
• Inconsistency between examination and observed function.

Triggers and risk factors: Physical injury, illness, migraine, a panic attack or medical procedure often precede onset. Anxiety, depression and previous adversity are common, but not universal. It is not "made up" or "all in the mind".

Management:

• Explain clearly and positively - name the diagnosis, show the positive signs, emphasise that it is common, genuine and potentially reversible. Point to reliable information (neurosymptoms.org).
• Specialist physiotherapy (movement retraining), occupational therapy and speech therapy.
• Psychological therapy (CBT) where relevant.
• Treat pain, fatigue, sleep, mood and other conditions.
• Avoid unnecessary repeat tests, procedures and medicines.
• About 1 in 10 also has another neurological condition - keep an open mind if things change.

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Driving and Neurological Conditions

Always check the current DVLA "Assessing fitness to drive" guidance. These are common Group 1 (car) rules; Group 2 (lorry and bus) rules are much stricter.

💡 Clinical Pearl: It is the driver's legal duty to tell the DVLA, but doctors must explain this clearly and document it. If a patient continues to drive against advice and is a danger, GMC guidance allows doctors to inform the DVLA after telling the patient.

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Drug Reference Table

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Spot the Diagnosis

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Exam-style Scenarios

1. A 22-year-old student has numb feet, unsteady walking and extensor plantars. He inhales nitrous oxide most weekends.
Answer: Subacute combined degeneration (and neuropathy) from nitrous oxide.

• B12, homocysteine and methylmalonic acid; MRI spine (dorsal column signal).
• IM hydroxocobalamin urgently.
• Stop nitrous oxide; harm reduction support.

2. A 68-year-old has brief spinning vertigo whenever she lies down or rolls over. Dix–Hallpike is positive on the right.
Answer: Right posterior canal BPPV.

• Epley manoeuvre.
• Avoid vestibular sedatives.
• Repeat if needed; teach home exercises.

3. A 30-year-old has sudden right leg weakness after a minor fall. Tone and reflexes are normal, and Hoover's sign is positive.
Answer: Functional limb weakness.

• Explain the diagnosis positively, showing the Hoover's sign.
• Specialist physiotherapy; address pain, mood and sleep.
• Avoid unnecessary tests.

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Key Points

• Degenerative cervical myelopathy is common and under-diagnosed: clumsy hands, Hoffmann's sign, spastic gait - MRI and early surgical referral.
• Syrinx: cape-like loss of pain and temperature with wasted hands. Nitrous oxide causes B12-related cord damage.
• BPPV: brief positional vertigo - Dix–Hallpike and Epley. Ménière's: hours of vertigo with hearing loss and fullness.
• Narcolepsy: sleep attacks with cataplexy. RBD predicts synucleinopathy.
• NF1: café-au-lait spots, freckling, neurofibromas, phaeochromocytoma. TSC: ash-leaf macules, epilepsy, angiomyolipomas.
• Offer HIV testing in unexplained neurological illness; ring-enhancing lesions in low CD4 suggest toxoplasmosis.
• PRES: hypertension, eclampsia or calcineurin inhibitors with posterior oedema. Correct chronic hyponatraemia slowly.
• Functional neurological disorder is diagnosed on positive signs and treated with explanation and physiotherapy.
• Stroke or single TIA: no driving for 1 month (Group 1).

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Guidelines and Further Reading

• Myelopathy.org and AO Spine guidance on degenerative cervical myelopathy
• NICE CKS: Benign paroxysmal positional vertigo; Ménière's disease; Vertigo
• British Sleep Society and NICE guidance on narcolepsy treatments
• British HIV Association guidelines on opportunistic infection
• DVLA: Assessing fitness to drive - neurological disorders
• neurosymptoms.org - functional neurological disorder information
• BNF - for current doses

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