Neurology Flashcards

1
Q

What is the definition and epidemiology of Bell’s Palsy?

A

Bell’s Palsy is an idiopathic lower motor neurone facial (VII) nerve palsy. Annual incidence is around 15-40 per 100 000. Although idiopathic, it is preceded by an upper airway infection in 60%, suggesting a viral or post-viral aetiology.

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2
Q

What are the clinical features of Bell’s Palsy?

A

Patients have a prodrome of pre-auricular pain in some cases, followed by acute onset (hours/days) unilateral facial weakness and droop.

Fifty percent of patients experience pain or numbness in the face, neck or ear. Patients also experience hypersensitivity to sound (due to stapedius muscle paralysis), and tearing or drying of exposed eye. A loss of taste sense is uncommon.

On Examination:
There is lower motor neurone weakness of facial muscles (affects all the ipsilateral muscles of facial expression, and does not spare muscles of the upper part of the face as seen in UMN facial nerve palsy).

May elicit Bell’s phenomenon where the eyeball rolls up but eye remains open when trying to close eyes.

The ear should be examined for other causes (e.g, otitis media, herpes zoster infection).

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3
Q

What are the potential complications of Bell’s Palsy?

A

Corneal ulcers and eye infections are likely complications.

Aberrant reinnervation may also occur, e.g. blinking may cause contraction of the angle of the mouth due to simultaneous innervation of obicularis oculi and ori. Parasympathetic fibres may also aberrantly reinnervate causing ‘crocodile tears’ when salivating.

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4
Q

How can Bell’s Palsy be investigated?

A

Usually unnecessary, except to exclude Lyme serology and herpes zoster serology.

An EMG may show local axonal conduction block in facial canal. However, only useful >1 week after osnet.

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5
Q

How is Bell’s Palsy managed?

A

Protection of cornea with protective glasses/patches and artificial tears.

High-dose corticosteroids is beneficial if given within 72h of onset. It helps shorten time of recovery and improve long-term outcomes.

Antiviral agents such as acyclovir, have shown to improve long-term sequelae.

Surgical decompression can be done in patients that follow the following criteria

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6
Q

What is the definition and epidemiology of Dementia?

A

Dementia is the decline in cognitive function enough to interfere with daily life. Dementia is very common affecting 1 in 79, or 1 in 14 of those over the age of 65.

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7
Q

What are the different types/causes of Dementia?

A

The causes of dementia in order of prevalence are:
1. Alzheimer’s disease accounts for 65% of dementia cases - cortical atrophy in frontal, parietal and temporal areas caused by beta-amyloid plaques and neurofibrillary tangles.

  1. Dementia with Lewy bodies accounts for 15% of dementia in the UK. Affected neurones form Lewy bodies instead of tangles.
  2. Vascular dementia accounts for at least 10%, but possibly more with a mixed disease with Alzheimer’s. Caused by widespread small vessel disease within the brain (diabetes or hypertension), or due to mini-strokes.
  3. Other progressive intracranial pathology (e.g. brain tumour, MS, Pick’s disease, MND)
  4. Alcohol and drugs
  5. Rare infections and deficiencies (e.g. HIV-AIDS, syphilis, B vitamin deficiency)
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8
Q

What are the clinical features of Alzheimer’s Disease?

A

Patients usually have one or more of the risk factors, including advancing age, family history, genetics, Down’s syndrome, cerebrovascular disease, and hyperlipidaemia.

There can be said to be a pre-dementia stage for up to 8 years before criteria for AD is met. In this stage, patients noticeably lose their short-term memory, and have subtle problems with executive functions of planning, abstract thinking, semantic memory. Apathy (patient becomes passive, not wanting to perform daily activities, sleeping more) can also be noticed at this stage.

The hallmark of AD is memory decline with loss of recent memories first. Memory is usually affected first. Later language is affected with nominal dysphasia (difficulty naming people and objects). Spatial memory is also affected, causing disorientation, and misplacing items/often getting lost.

These all lead to a decline in Activities of Daily Living (ADL).

The patient has unremarkable initial physical examination, but in terminal disease may be unable to walk or speak.

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9
Q

What are the clinical features of Dementia with Lewy Bodies?

A

Intellectual symptoms due to cognitive decline similar to those of Alzheimer’s disease. However, patients are likely to develop issues with executive function, impaired attention, and visuospacial function before memory is affected.

Patients are also much more likely to develop parkinsonism, visual hallucinations and episodes of confusion.

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10
Q

What are the clinical features of Vascular Dementia?

A

As Alzheimer’s dementia can often co-occur with vascular dementia, it can be difficult to separate the two. Vascular dementia patients have risk factors of stroke including: obesity, age, hypertension and cigarette smoking, diabetes.

Patients present with problems with executive function such as problem solving, and signs of frontal cognitive syndrome (apathy, disinhibition, slowed processing of information, poor attention).

Patients tend to have better recall and fewer intrusions than those suffering from Alzheimer’s, though also suffer from memory impairment.

If dementia is caused my mini-strokes, impairment progresses in a stepwise way, with every mini-stroke.

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11
Q

How can dementia be investigated?

A

Blood tests such as:
• FBC - to rule out anaemia
• Metabolic panel - to exclude sodium, calcium and glucose levels causing the dementia
• Serum TSH - exclude hypothyroidism
• Serum B12 - as B12 deficiency can cause dementia.

Bedside cognitive testing will show cognitive impairments. The pattern of which can help differentiate the dementias, alongside a good history.

CT and MRI are useful, and can show characteristic changes for the different dementias.

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12
Q

What is the definition and epidemiology of Epilepsy?

A

Epilepsy refers to a group of neurological disorders characterised by seizures (>2). A seizure/ictus refers to a brief episode of signs/symptoms caused by synchronised electrical activity in the brain. Epilepsy is common, affecting 1% of the population.

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13
Q

What are the types of Epilepsy?

A

Epilepsy falls within two main categories: generalised (affecting all parts of the cerebral cortex) or partial/focal (affecting one region or lobe or hemisphere, but can spread to other regions and lead to a secondary generalised seizure).

In generalised epilepsy, there tends to be a loss of consciousness (not necessarily, such as in myoclonic seizures). Types of generalised epilepsy include:
• Tonic-clonic seizure (grand maI)- A tonic phase (sustained muscle contraction) followed by a clonic phase (random jerks and contractions).
• Absence seizure (petite mal)- patient remains ‘absent’ just staring for a few minutes.
• Myoclonic seizure - brief jerks of a muscle or muscle group.
• Clonic - a monoclonus that is regularly repeating.
• Tonic - Sustained muscle contraction, patient goes stiff causing them to fall backwards.
• Atonic - Loss of muscle tone, causing patient to drop to the floor forwards.

Partial/focal seizures can be further divided into simple partial (where consciousness is preserved), or complex partial

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14
Q

What are the causes of Epilepsy?

A

The majority of cases are idiopathic. These fall within primary epilepsy syndromes: idiopathic generalised epilepsy, temporal lobe epilepsy, or juvenile myoclonic epilepsy.

Sometimes, seizures can occur secondary to a cause, including:
• Tumour
• Infection (e.g. meningitis, encephalitis, abscess)
• Inflammation (e.g. vasculitis, rarely MS)
• Toxic/metabolic (Na imbalance, hyper/hypoglycaemia, hypocalcaemia, hypoxia, porphyria, liver failure)
• Drugs, including withdrawal from alcohol or benzodiazepines.
• Vascular, Trauma, Congenital abnormalities.

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15
Q

What are the clinical features of Focal Seizures?

A
  • Frontal lobe seizure: motor convulsions. May demonstrate Jacksonian march (convulsions start from fingers or mouth, and spread). There may be post-ictal flaccid weakness. On other hand, may have sensory seizure where strong sensations are felt in one part of the body, face or limbs.
  • Temporal lobe seizure: patient experiences an aura, Déjà vu, or hallucinations of smell or taste.
  • TFrontal lobe complex partial seizure: loss of consciousness, with associated automatisms and rapid recovery.
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16
Q

What are the clinical features of Generalised Seizures?

A
  • Tonic-clonic: Vague symptoms before attack such as irritability. Tonic phase (sustained muscle contraction) followed by a clonic phase (random jerks and contractions). Associated faecal or urinary incontinence, tongue biting. Typical post-ictal phase of altered consciousness, lethargy, confusion, headache, back pain, stiffness.
  • Absence: Starts in childhood. Characterised by loss of consciousness but maintenance of posture. Child often reported as staring blankly. No post-ictal phase.
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17
Q

What is status epilepticus?

A

Status epilepticus refers to a seizure (unless stated assume a generalised tonic-clonic seizure) that lasts for more than 30 minutes

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18
Q

What are the complications of Epilepsy?

A

Fractures in tonic-clonic seizures. Hypoxia/brain damage if status epilepticus is not controlled.

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19
Q

How can Epilepsy be investigated?

A

Bloods:
• Glucose as hyperglycaemia or hypoglycaemia can cause seizures.
• FBC to look for generalised infections.
• U&Es as electrolyte imbalances, particularly Na+ imbalances or uraemia can cause seizures.
• Serum prolactin can help differentiate generalised seizures from non-epileptic seizure disorder, as seizures increase serum prolactin.

EEG (Electroencephalogram) can help classify the seizure disorder.

A head CT/MRI should be performed to look for any lesions, which could explain the seizure.

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20
Q

How is Epilepsy managed?

A

Status Epilepticus (Generalised Convulsive)”
Although defined as seizure lasting for more than 30 minutes, treatment is started after 5 minutes:
1. ABC - protect airways, breathing and circulation.
2. I.V, rectal or I.M benzodiazepine such as lorazepam (IV) or diazepam (PR).
3. If seizures reoccur or do not stop, give I.V phenytoin under ECG monitoring.
4. If this fails, give IV phenobarbital.
5. If this fails, administer general anaesthesia and ventilate the patient.

Treat the cause if possible i.e. hypoglycaemia.

General Practise:
Only start anti-convulsant therapy after >2 unprovoked seizures.

Patient education is very important. This includes avoiding triggers, and keeping a seizure diary. Recommended supervision for swimming or climbing. Driving is only permitted if not had a seizure in more than 6 months.

Surgery for refractory epilepsy?

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21
Q

Describe the features of a Tension Headache

A

A tension headache can either be episodic or chronic. It is very common.

It is frequently described as a tight band around the head, often radiating into the neck. The source of the pain is believed to be chronic contraction of the head and neck muscles.

The patient usually has a background of stress and worry, sometimes with clinically significant anxiety or depression. The patient may be worried about it being a brain tumour, fuelling the stress and anxiety further.

They are rarely disabling and are not associated with autonomic dysfunction.

22
Q

How is a Tension Headache managed?

A

Treatment usually starts by helping the patient understand the nature of the headache, with reassurance (after careful neurological examination).

Advise them to take simple analgesics such as paracetamol or aspirin.

Underlying depression should be treated.

Chronic:
Antidepressants such as amitriptylinehave been shown to help treat tension headaches, even if dose is lower than needed to treat depression.

Non-drug relaxing therapies such as relaxation training, EMG biofeedback, cognitive behavioural therapy can be used.

Physical measures,including physiotherapy, acupuncture, and spinal manipulation, may also provide benefit but, as with hypnosis, the evidence for their effectiveness is weak.

23
Q

Describe the features of a Cluster Headache

A

A cluster headache is considered to be one of the most painful conditions known to humanity. Predominantly affect men more than women, and 1 in 500.

They tend to occur in clusters, repetitively, once or twice a day, for several weeks. After, they have a long interval of a year or more until it recurs in the same way.

The attacks themselves are brief, lasting 30-120 minutes. They often occur at the same time in each 24h cycle. The pain is extremely severe and tends be felt around one eye. It is usually accompanied by ipsilateral signs of autonomic dysfunction including redness, swelling, nasal congestion or Horner’s syndrome.

The patient may also complain of nausea, vomiting, photophobia, and a migranous aura.

A brain CT/MRI is normal in primary cluster headaches. However abnormalities can point towards secondary cause.

24
Q

Define Meningits

A

Meningitis is the inflammation of the meninges, most commonly caused by infection.

25
Q

What is the aetiology and risk factors for Meningitis?

A

Aetiology:
• Bacterial:
○ Neonates: Group B Streptococci, E. coli, Listeria monocytogenes
○ Children: Haemophilus influenzae, Neisseria meningitidis, Streptococcus pneumoniae
○ Adults: Neisseria meningitidis, Streptococcus pneumoniae, Tuberculosis.
○ Elderly: Streptococcus pneumoniae and Listeria monocytogenes
• Viral: Enteroviruses, mumps, HSV, VZV, HIV.
• Fungal: Cryptococcus (with HIV)
• Others: Aseptic meningitis (not due to infection).

Risk factors include: close communities such as student dorms, basal skull fractures, mastoiditis, sinusitis, inner ear infection, immunodeficiency, splenectomy, sickle-cell anaemia, CSF shunts, intercranial surgery.

26
Q

What are the clinical features of Meningitis?

A

Patients present with a severe headache, photophobia, neck or back ache and fever and vomiting. Patients are also often irritable, drowsy and confused. Seizures are also common.

A careful history should reveal risk factors such as recent travel, or exposure to rodents or ticks.

On examination there are signs of:
• Infection: fever, tachycardia, hypotension, and skin rash.
• Meningism: photophobia, neck stiffness, positive Kernig’s sign (patient supine with hip flexed at 90o, knee cannot be fully extended) and Brudzinski’s sign (Passive flexion of neck causes flexion of both legs and thighs).

27
Q

How can Meningitis be investigated?

A

A CT scan is necessary in certain patients before a lumbar puncture. This is to exclude causes that would lead to increased cranial pressure (which would lead to herniation during CSF removal). If the patient has recent neurological deficit, new-onset seizures, papilledema, abnormal level of consciousness, or is immunocompromised, perform a head CT before CSF removal.

A lumbar puncture should be the first test, to analyse the CSF for microscopy, culture and sensitivity testing.
• In bacterial meningitis, the CSF will appear cloudy, and have ↑neutrophils, ↑protein and ↓glucose.
• Whereas in viral meningitis, there will be ↑lymphocytes and ↑protein but normal glucose.

In patients where LP is delayed, two sets of blood cultures should be sent off.

PCR can be used to amplify bacterial DNA and diagnose bacterial meningitis over viral meningitis.

28
Q

How is Meningitis managed?

A

Immediate IV antibiotics (even before LP) if meningitis is suspected. Give broad-spectrum antimicrobials such as vancomycin or meropenem or chloramphenicol before specific antibiotics.

Give adequate supportive care (ABC). Administering fluids and vasoconstrictors if necessary. Patient is best managed in ITU.

Dexamethasone is given shortly after or with the first dose of antibiotics, and continued for 4 days. Has been shown to improve outcome and mortality. Avoid if HIV cause is suspected.

If viral meningitis cause is confirmed, continue supportive care and consider antiviral therapy.

29
Q

What are the complications of Meningitis?

A
  • Septicaemia
  • Shock
  • DIC
  • Renal failure
  • Fits
  • Peripheral gangrene
  • Cerebral oedema
  • Cranial nerve lesions, cerebral venous thrombus, hydrocephalus.
30
Q

What is the prognosis of Meningitis?

A

Mortality rate for bacterial meningitis is very high (10-40%). Viral meningitis is self-limiting.

31
Q

What is the definition and epidemiology of Migraine?

A

A migraine is a severe episodic headache that may have a prodrome of focal neurological symptoms (aura) and is associated with systemic disturbance. Can be classified as a common migraine (without aura) or classical migraine (with aura). Migraines are very common, affecting 15% of women and 6% of men. Usual onset is adolescence or early adulthood.

32
Q

What is the aetiology of Migraine?

A

Aetiology poorly understood, but evidence to suggest the brain of a migraine sufferer is hyper-excitable to a variety of stimuli.

33
Q

What are the clinical features of a Migraine?

A

A migraine produces episodes of headaches, which can be described as severe and pulsating, often on one side of the head, but can also be bilateral. It is also often felt at the front of the head. The patient is usually fine in between episodes. The migraine usually and lasts for several hours (some patients experience intermittent episodes for days).

Patients also tend to experience nausea, vomiting, photophobia and phonophobia. Classically (although uncommonly) preceded by an aura of visual disturbance or other sensory symptoms such as tingling.

Completely normal examination features.

34
Q

What are the triggers for a Migraine?

A
Triggers
• Stress and fatigue (even relaxation after stress)
• Skipping meals
• Binge eating
• Specific foods (cheese, citrus etc)
• Specific drinks (caffeine, wine)
• Menstruation or ovulation
• Oral contraceptive.
35
Q

How is a Migraine investigated?

A

Diagnosis based on history, investigations are done to exclude other causes.

Bloods including FBC, ESR etc.

CT/MRI if examination is abnormal.

Lumbar puncture if meningitis is suspected.

36
Q

How is Migraine managed?

A

If presenting to emergency department, a rescue therapy of I.M or I.V magnesium can help with aura or symptoms.

Analgesics such as NSAIDs, paracetamol or codeine can be prescribed. Antiemetics such as metoclopramide can help with nausea. Important to inform patient about analgesic overuse.

Adequate hydration is also important as dehydration can cause migraines.

For severe symptoms a triptan (5HT1 agonist) can help reduce symptoms and need for further analgesics.

37
Q

Define motor neurone disease

A

MND is a progressive neurodegenerative disorder of the cortical, brainstem, and spinal motor neurones (upper and lower motor neurones).

38
Q

What are the types of MND?

A
  • Amyotrophic lateral sclerosis (ALS): combined degeneration of UMN and LMN neurones.
  • Progressive muscular atrophy variant: Only LMN signs, e.g foot drop. Associated with better prognosis
  • Progressive bulbar palsy variant: dysarthria (difficulty articulating speech) and dysphagia as CN IX, X and XII lie in the medulla oblongata (the ‘bulb’).
  • Primary lateral sclerosis variant: UMN pattern of weakness, brisk reflexes, extensor plantar responses, without LMN signs.
39
Q

What is the aetiology and epidemiology of MND?

A

They are of unknown aetiology and rare, affecting 2 per 100,000. Mean age of onset is 55 years. 5–10% have family history with autosomal dominant inheritance.

40
Q

What are the clinical features of MND?

A

Weakness in limbs (focal or asymmetrical). Speech slurring or reduction in volume. Swallowing disturbance (choking in food, nasal regurgitation). There may be behavioural changes such as disinhibition and emotional liability.

On examination, there are a combination of UMN and LMN signs, asymmetrically.
• UMN signs include: Brisk reflexes, spastic weakness, and extensor plantars.
• LMN signs include: Muscle wasting, fasciculations, flaccid weakness, depressed or absent reflexes.

Sensory examination should be normal.

41
Q

How is MND investigated?

A

Investigations are aimed to exclude other causes of combined UMN and LMN symptoms:
• Bloods (CK may be increased)
• EMG (Electromyography) measures the electrical activity generated by muscles. It shows acute and chronic denervation.
• Nerve conduction studies are often normal (???)
MRI to exclude cord or root compression, or a brain lesion for bulbar palsy variant.

42
Q

What is the definition and epidemiology of MS?

A

Multiple Sclerosis is defined as an inflammatory demyelinating disease characterised by the presence of episodic neurological dysfunction in at least 2 areas of the CNS (brain, spinal cord, and optic nerves) separated in time and space. MS is the most common cause of neurological disability in young adults, affecting 1 in 1000 patients. Most commonly diagnosed between the ages of 20-40 years and affecting females more than males (2:1).

43
Q

What are the types of MS?

A

There different types of MS (not biologically based, but clinically retrospectively):
• Relapsing-remitting MS - commonest form. Characterised by clinical attacks of demyelination with complete recovery in between attacks.
○ Secondary progressive MS - >50% of patients with relapsing-remitting MS develop an accumulative progressive course.

  • Primary-progressive MS - steady accumulation of disability with no clear relapsing-remitting pattern.
  • Clinically isolated syndrome - single clinical attach of demyelination (does not qualify as MS), but 10-50% progress to developing MS.
  • Marburg variant - severe fulminant variant of MS leading to advanced disability to death within a period of weeks. Distinct from acute disseminated encephalopathy (ADEM).
44
Q

What is the aetiology of MS?

A

Unknown aetiology with autoimmune basis. EBV exposure and prenatal vitamin D levels have been found to be associated with MS in epidemiological studies.

45
Q

What are the symptoms of MS?

A

Symptoms depend on the site of inflammation. Common ones include:
• Optic neuritis (commonest) in one eye - Impaired visual acuity (described as looking through jelly), loss of colour discrimination and pain moving the affected eye.
• Particular sensory phenomena such as pins and needles, odd sensations, numbness, burning.
• Motor symptoms such as weakness in limbs causing foot drop or spasms. (UMN signs as affects the CNS - increased tone, reflexes,
• Autonomic symptoms are also common, causing urinary urgency, hesitancy, incontinence (urinary and faecal) and impotence.
• Psychological symptoms of depression and psychosis.

46
Q

What are the examination features of MS?

A

Fundoscopy may reveal optic neuritis. In chronic disease there may be optic atrophy. Visual field examination may reveal central or field defects. There may be a abnormal RAPD (relative afferent pupillary defect).

Can elicit motor signs (UMN) such as increased tone, reflexes, weakness.

There may be cerebellar signs such as dysdiadochokinesia, intention tremor, ataxic wide-based gait, scanning speech.

May elicit Lhermitte’s phenomenon: (Electric shock-like sensation in arms and legs precipitated by neck flexion)

47
Q

How is MS investigated?

A

Diagnosis is made from two or more CNS lesions with corresponding symptoms, separated by time and space.

A lumbar puncture can be done to look for other inflammatory causes of symptoms. Oligoclonal bands and increased CSF immunoglobulins point to MS.

MRI of brain and cervical spine with gadolinium to look for plaques indicating MS.

Evoked potentials may show slow delayed conduction velocity. VEPs (visual electrode potentials) are slow in ~90% of MS patients.

48
Q

Define MND

A

Motor Neurone Disease is a progressive neurodegenerative disorder of the cortical, brainstem, and spinal motor neurones (upper and lower motor neurones).

49
Q

What are the types of MND?

A

There are various subtypes:
• Amyotrophic lateral sclerosis (ALS): combined degeneration of UMN and LMN neurones.
• Progressive muscular atrophy variant: Only LMN signs, e.g foot drop. Associated with better prognosis
• Progressive bulbar palsy variant: dysarthria (difficulty articulating speech) and dysphagia as CN IX, X and XII lie in the medulla oblongata (the ‘bulb’).
• Primary lateral sclerosis variant: UMN pattern of weakness, brisk reflexes, extensor plantar responses, without LMN signs.

50
Q

What is the aetiology and epidemiology of MND?

A

They are of unknown aetiology and rare, affecting 2 per 100,000. Mean age of onset is 55 years. 5–10% have family history with autosomal dominant inheritance.

51
Q

What are the clinical features of MND?

A

Weakness in limbs (focal or asymmetrical). Speech slurring or reduction in volume. Swallowing disturbance (choking in food, nasal regurgitation). There may be behavioural changes such as disinhibition and emotional liability.

On examination, there are a combination of UMN and LMN signs, asymmetrically.
• UMN signs include: Brisk reflexes, spastic weakness, and extensor plantars.
• LMN signs include: Muscle wasting, fasciculations, flaccid weakness, depressed or absent reflexes.

Sensory examination should be normal.

52
Q

How is MND investigated?

A

Investigation
Investigations are aimed to exclude other causes of combined UMN and LMN symptoms:
• Bloods (CK may be increased)
• EMG (Electromyography) measures the electrical activity generated by muscles. It shows acute and chronic denervation.
• Nerve conduction studies are often normal (???)
• MRI to exclude cord or root compression, or a brain lesion for bulbar palsy variant.