Trickier NeurologyClinical Syndromes for the AMK

By Olivia JaggerAcademic Education F2

Examination findings and causes

Upper Motor NeuroneLower Motor

Neurone

Tone Spastic Normal

Reflexes Increased Decreased or absent

Clonus ?+ve (ankles) -ve

WastingAbsent (disuse

wasting)Present

Fasiculatio

nAbsent ?Present (esp MND)

Plantars Extensor Flexor or absent

Causes Stroke, MS, MND Guillain Barre, MND

A 34-year old woman presents to A+E with a two day history of increasing headache and confusion. She has a temperature of 38.2 degrees. Whilst in the emergency department she has a tonic-clonic seizure. MRI shows increased signal in the temporal lobes on T2 imaging. What is the most likely cause?

A. Alcohol intoxication

B. Severe pneumonia

C. Viral encephalitis

D. Subarachnoid haemorrhage

E. Hepatic encephalopathy

Herpes Simplex Encephalitis

• History

Malaise, fever, headache, nausea and vomiting.

• Followed by acute or subacute onset of:

Altered consciousness

Focal and generalised seizures

Features of raised intracranial pressure

Psychiatric symptoms, behavioural abnormalities, confusion and delirium

Focal neurological signs, including hemiparesis and cranial nerve lesions

• Investigations

MRI head - commonly affects the temporal lobes.

LP. Culture of organisms is usually unsuccessful. PCR of the CSF is test of choice. Diagnosis can also be made by the detection of HSV-specific antibodies in the CSF.

• Management

IV acyclovir.

a notifiable disease!

A 53-year-old woman has a one-week history of sudden onset of weakness of the left side of her face. On examination she has a left-sided facial droop with difficulty on the left side in raising her eyebrow, closing her eye and blowing out her cheek. What is the most likely diagnosis?

A. Myasthenia gravis

B. Lacunar infarct

C. Multiple sclerosis

D. Bell’s palsy

E. Middle cerebral artery infarct

A 51 year old gentleman is referred by his GP for worsening back pain. He works as an electrician and is concerned that he is unable to work due to the pain. On questioning, he has had 'niggling pain' in his lower back for a few months but this morning, 4 hours prior to presentation he notes that all of a sudden the pain became far worse, very sharp in nature with no radiation. He notes that he wants to pass urine but feels unable to and is complaining of saddle anaesthesia.

• Which is the most appropriate diagnosis?

sciatica

mechanical back pain

cauda equina syndrome

syringomyelia

prolapsed disc

A 51 year old gentleman is referred by his GP for worsening back pain. He works as an electrician and is concerned that he is unable to work due to the pain. On questioning, he has had 'niggling pain' in his lower back for a few months but this morning, 4 hours prior to presentation he notes that all of a sudden the pain became far worse, very sharp in nature with no radiation. He notes that he wants to pass urine but feels unable to and is complaining of saddle anaesthesia.

• Which is the most appropriate diagnosis?

Thoraco-lumbar spine Xrays

MRI spine

Bloods including erythrocyte sedimentation rate and C-reactive protein

CT head

Nerve conduction studies

Cauda Equina - neurological emergency!

Commonest cause is central disc prolapse

Compression of central sacral or lumbar roots

Sciatica

Loss of bladder / bowel control / sexual dysfunction

Saddle sensory loss

Perianal sensory loss and sphincter disturbance

Lower limb weakness (decreased/lost reflexes, paraesthesia)

Urgent lumbar MRI and surgical referral for decompression

• A 25-year-old man develops sudden weakness of the legs followed a few days later by weakness in the arms associated with ‘pins and needles’. He is normally well apart from 3 days of diarrhoea about 2 weeks before. On examination he has weakness in all four limbs and his reflexes are absent.

• What is the most appropriate diagnosis?

Peripheral neuropathy

Brainstem lesion

Spinal Cord Compression

Guillain-Barre syndrome

Brown-Sequard syndrome

• A 25-year-old man develops sudden weakness of the legs followed a few days later by weakness in the arms associated with ‘pins and needles’. He is normally well apart from 3 days of diarrhoea about 2 weeks before. On examination he has weakness in all four limbs and his reflexes are absent.

• What is the most appropriate investigation?

Vital Capacity

CSF

Viral blood screen

Nerve conduction tests

Chest Xray

Guillain-Barre Syndrome - Presentation

Autoimmue attack v myelin antigens

60% have antecedent infection (EBV, CMV, campylobacter)

Syndrome about 2 weeks later

Sensory symptoms in toes (paraesthesia), followed by weakness in legs (areflexia and later wasting)

Weakness ascends over 10-14 days (can involve face and respiratory muscles)

can be associated with back pain

GBS - Investigations and Management

CSF: raised protein, absence of WBCs

Nerve conduction studies normal in early stages

? recent infection (viral screen)

Monitor Vital Capacity (+ / - ventilation)

Best nursed on HDU / ITU

ECG (occ heart block)

IVIg if evolving significant weakness

Analgesia

5% mortality; 10% permanent disability

A 67-year-old woman had a 3 month history of weakness of his left leg and a 1 month history of weakness in both arms. He felt things were getting progressively worse. On examination he had wasting and fasciculations in all four arms. He was weak in all four limbs with brisk reflexes throughout with upgoing plantars. He had a normal sensory examination. What is the most likely diagnosis?

• What is the most appropriate diagnosis?

Parkinson’s disease

Multiple sclerosis

Motor neurone disease

Myasthenia gravis

Huntington’s Chorea

Motor Neurone Disease - Signs

Combination of UMN and LMN signs (selective of MOTOR neurones)

Wasting / weakness small muscles hands (LMN)

Fasiculations upper limbs / shoulders (LMN)

leg weakness / brisk reflexes / extensor plantars (spastic) (UMN)

Bulbar / pseudobulbar palsy - affects speech / swallow (LMN/UMN)

Motor Neurone Disease - Diagnosis and Management

Diagnosis mainly clinical (Absence of sensory symptoms / signs, CK moderately increased, NCS show denervation)

Aetiology unknown, 10% dominantly inherited

Life expectancy on average 4 years

Management - supportive (SALT, OT, PEG, +/- Riluzole)

A 25-year-old woman develops a painful visual disturbance in her left eye. She had previously had an episode of ataxia a year earlier lasting a few months.

What is the most appropriate diagnosis?

Parkinson’s disease

Multiple sclerosis

Motor neurone disease

Myasthenia gravis

Huntington’s Chorea

Multiple SclerosisInflammatory demyelinating CNS disorder - Disease of the white matter

relapsing/remitting, secondary progressive, primary progressive

Acute attack:

- Optic neuritis (optic nerve)

- Transverse myelitis (spinal cord)

- Diplopia (brainstem)

Late disease: spastic paraplegia, incontinance, dementia, cerebella ataxia, bilateral intranuclear opthalmoplegia

Multiple Sclerosis

Investigations: Brain MRI / LP (oligoclonal banding)

Acute Management: steroids

Disease modifying agents: Betainterferons, Natalizumab

Other treatments: PT, OT, antispasmotic agents (baclofen), ??Vit D

A 50-year-old woman presents with difficulty keeping her head up. She has proximal weakness in the arms which is fatigable. She comments that he speech becomes very slurred towards the end of the day and she is finding it difficult to swallow.

• What is the most appropriate diagnosis?

Stroke

Parkinson’s disease

Multiple Sclerosis

Myasthenia Gravis

Huntington’s disease

Myasthenia Gravis - presentation

• Aetiology

✴ Autoimmune condition. Antibodies attack the postsynaptic acetylcholine receptors at the neuromuscular junction, leading to decreased conduction at NMJ and muscle fatigue/ weakness.

✴ Disease has a fluctuating pattern of ‘crises’ and remittances.

✴ Very strong association with disorders of the THYMUS. In 75% of patients with Myasthenia Gravis there is hyperplasia of the thymus, and in 10% this becomes a thymoma.

• Symptoms

✴ Weakness in muscles, particularly the ocular, bulbar, proximal skeletal and axial. (Respiratory muscles can be involved).

✴ Fatigability is a key symptom! Symptoms get worse at the end of the day.

✴ The fatigability of muscles can be demonstrated by getting the patient to do a repetitive movement (e.g flap their arm’s) for 30-60 seconds.

MG - Investigation / Treatment

•Investigations

✴Blood tests for serum acetylcholine receptor antibodies

✴Nerve conduction studies

✴CT/ MRI scans - to image the thymus

✴Vital Capacity (Spirometry / peak flow) may need respiratory support

✴Ice cube test - place over ptosis for 2mins, eye opens. Though that low temperatures improve neuromuscular transmission.

• Treatment

✴ Acetylcholinesterase inhibitors (neostigime) increase the amount of Acetylcholinesterase available to functioning receptors.

✴ Immunosupression (steroids .i.e. prednisolone)

✴ In crisis - IV Immunoglobulins or plasmaphoresis may be indicated.

✴ Thymectomy

A 66-year-old man is finding that he has more difficulty getting up and moving about for the past year. He is annoyed by a tremor in his hands, but the tremor goes away when he performs routine tasks using his hands. His friends remark that he seems more sullen and doesn't smile at them, but only stares with a fixed expression on his face. He has not suffered any loss of mental ability.

• Which of the following conditions is he most likely to have?

Parkinson’s disease

Recurrent TIA’s

Huntington’s disease

Myasthenia Gravis

Motor Neurone Disease

Parkinsons - clinical features

Pathology: loss of dopaminergic neurons from the substania nigra. Surviving neurons often contain Lewy bodies (collections of proteins - α-synuclein).

Aetiology: most commonly idiopathic (can be drug induced and several genetic ‘parkinsons plus’ syndromes)

Clinical features: TRIAD 1) resting tremor 2) rigidity 3) bradykinesia

Other typical signs/symptoms: Festinating gait - stooped, shuffling, narrow based, slow to start then accelerates, with swinging arms, Hypomimia (and reduced blinking rate - glabellar tap), Hypographia, Depression, Hallucinations, Lewy body dementia, Anosmia.

Power and reflexes are normal (extra-pyramidal)

No sensory abnormalities.

Parkinsons - treatment• L-DOPA (most common, 1st line)

Dopamine cannot cross the BBB, but its precursor L-DOPA can, and once it has, it is converted to dopamine by DOPA decarboxylase. Acts by increasing levels of free dopamine in the brain.

Short half-life. Therefore, L-Dopa is given with a decarboxylase inhibitor (carbidopa, entacapone or benserazide) to prevent peripheral breakdown and prolong the half-life of L-dopa, reducing the dose needed by about 90%!

• Side Effects

• Efficacy decreases over time

• ‘On-off effect’ at the end of the dose

• Nausea / GI Upset

• Dyskinesias

• Psychosis

• Compulsive behaviours – often related to gambling, money or sexual behaviour.

Other neuro conditions you should be familiar with ...

Migraine - Classical Features• Classical Features

✴ Unilateral

✴ Visual disturbance – zigzag lines

✴ Changes in sensation of face

✴ Vomiting/nausea/abdominal pain

✴ Aura/halo

✴ Daytime onset

✴ Relieved by sleep

✴ Have to lie in a quiet, dark room (photophobia and noise adversion)

✴ FH migraine

✴ Are they on COCP? (CI in migraines)

✴ Can have focal signs (i.e. arm/leg weakness)

• Treatment

✴ Acute attacks: Triptans (sumatriptan), hydration, analgesia, anti-emetic

✴ Prophylaxis: Propranolol

Meningitis• Signs and Symptoms

Photophobia

Stiff neck

Rash (non-blanching)

Systemically unwell

Contact with meningitis

• Investigations

CT head prior to LP to ensure no raised intracranial pressure

LP - increased protein (inflammation) and WCC (infection) and low glucose (gobbled up by bacteria). May culture an organism (commonly Neisseria meningitidis)

Treatment

•Start antibiotics immediately if suspected•At GP/in ambulance - IM Benzylpenicillin•At Hospital - IV Ceftriaxone

a notifiable disease!

Subarachnoid Haematoma• History:

✴ Sudden onset thunder clap headache

✴ Occipital

✴ SEVERE - peak intensity within seconds

✴ Associated vomiting

✴ Occasionally seizures

✴ FH of berry aneurysms (related to Polycystic Kidneys)

• Investigation

✴ CT angiogram

✴ LP (if normal CT) looking for xanthochromia broken down RBC in CSF

• Treatment:

✴ Ca2+ channel anatagonist (nimodipine) to reduce spasm and cerebral ischaemia

✴ Phenytoin if seizing

✴ Neurosurgery (coil / clip aneurysm)

✴ or best supportive care (if surgery CI)

Space-occupying lesion• Symptoms of raised intracranial pressure

✴ Worse in the mornings

✴ Night time wakening

✴ Worse when lying flat / coughing / bending down

✴ Associated with vomiting

✴ Visual changes

✴ Facial nerve palsy

✴ Seizures

✴ Papilloedema

• Tumours

✴ Abnormal movements – abnormal gait/loss of coordination

✴ Behavioural changes – change in mood/personality

✴ History of Ca

✴ Symptoms of Ca (weight loss)

Bells Palsy• Aetiology

Thought to be virally induced

Most start to recover within 3 weeks and resolve within 2 - 3 months (75%)

• Signs and Symptoms

Rapid onset unilateral facial nerve weakness.

Patient unable to show teeth, crew up eye and raise eyebrows on affected side

Severe cases may also present with notable loss of taste sensation (anterior 2/3 of tongue), intolerance of high-pitched noises, mild dysarthria.

Lower Motor Neuron Signs (LMN)

Can be distinguished from an UMN lesion (e.g. a stroke) by testing if the forehead is affected

◦Forehead normal – UMN lesion – due to bilateral cortical representation and innervation of the forehead

◦Forehead affected – LMN lesion

• Treatment

Steroids – Prednisolone (no evidence effective after 72 hours of onset)

Anti-virals – Aciclovir often given as it is thought many cases of Bell’s Palsy are due to herpes simplex or zoster infection. No evidence if more useful than steroids alone.

Good Luck!

Remember, some of the possible answers you will know and be able to exclude / rule in

Think what signs the question scenario is describing UMN or LMN

Some extra reading, look up Stroke and Epilepsy.

Any questions to olivia.jagger@nhs.net