Week 15 Neuro

Question 1

Intracerebral Haemorrhage stroke

Answer 1

Risk factors that contribute to this type of haemorrhage include

Cigarette smoking
Obesity
An unhealthy diet (such as one that is high in saturated fats, trans fats, and calories)
Using cocaine or amphetamines can cause temporary but very high blood pressure and haemorrhage. In some older people, an abnormal protein called amyloid accumulates in arteries of the brain.

A severe headache is common. Soon loss of consciousness. Nausea, vomiting, and seizures are common. Can be young. Can be middle meningeal artery.

Question 2

Subarachnoid Haemorrhage stroke

Answer 2

Subarachnoid haemorrhage is considered a stroke only when it occurs spontaneously—that is, when the haemorrhage does not result from external forces, such as an accident or a fall. A spontaneous haemorrhage usually results from the following:

The sudden rupture of an aneurysm in an artery in the brain
Aneurysms are bulges in a weakened area of an artery’s wall. Aneurysms typically occur where an artery branches. Most spontaneous subarachnoid haemorrhages result from congenital aneurysms. Is most common aged 40 to 65.

Less commonly, subarachnoid haemorrhage results from rupture of arteriovenous malformation or Infected heart valve emboli travelling to the brain.

More commonly in the older, middle age, shows signs of meningism, thunderclap headache, collapse, double or blurred vision. Ring of hematoma.

Question 3

Ischemic stroke risk factors

Answer 3

Risk factors:

hypertension
diabetes
smoking
atrial fibrillation  
mechanical valves
valvular abnormalities
patent foramen ovale 
significant decreased ejection fraction
hypercoagulable state
family history
prior history of
stroke
vascular disease

Question 4

Large vessel stroke characteristics AMP

Answer 4

Middle cerebral artery-
Contralateral weakness and sensory loss in face and upper limbs, broca (expressive: incoherent) if superior division, wernicke’s (receptive: x produce speech well) if inferior, right superior quadrat vision loss.

Anterior cerebral artery-
Contralateral weakness and sensory loss in lower limbs.

Posterior cerebral artery-
Contralateral hemianopsia with macular sparing, occipital lobe localisation.

Question 5

Lacunar lateral pontine stroke

Answer 5

Basilar artery occlusion.

Question 6

Lacunar medial and lateral medullary strokes

Answer 6

Medial medullary syndrome- can be Vertebral artery occlusion. Ipsilateral hypoglossal palsy, contralateral hemiparesis and proprioception loss.

Lateral medullary syndrome- can be anterior spinal artery occlusion. Dysphagia, hoarse voice, reduced gag, horner’s, vertigo, reduced temperature and pain ipsilateral in face, contralateral in body.

Question 7

Internal carotid stroke vs common carotid stroke

Answer 7

Internal carotid artery occlusion - ipsilateral amurosis fugax, tongue deviation to side of the lesion.

Common carotid shows horner’s syndrome and signs of MCA stroke:

Contralateral weakness and sensory loss in face and upper limbs, broca (expressive: incoherent) if superior division, wernicke’s (receptive: x produce speech well) if inferior, right superior quadrat vision loss.

Question 8

Spinal infarct

Answer 8

Spinal cord infarction usually results from ischemia originating in an extra vertebral artery. Symptoms include sudden and severe back pain, followed immediately by rapidly progressive bilateral flaccid limb weakness and loss of sensation, particularly for pain and temperature. Diagnosis is by MRI. Treatment is generally supportive.

Injury to an extra vertebral feeder artery or the aorta (eg, due to atherosclerosis, dissection, or clamping during surgery) causes infarction more commonly than do intrinsic disorders of spinal arteries. Thrombosis is an uncommon cause, and polyarteritis nodosa is a rare cause.

Question 9

Signs of cerebellar infarct

Answer 9

Vertigo, or an illusion of movement stemming from some sort of disease or a disorder-based process. It’s a sensation that you or your surroundings are spinning when they’re actually not.

Problems with balance

Difficulty walking normally, as evidenced by a wide-based stance or a propensity to fall over

Improper coordination of the trunk and limbs

Tremors

Question 10

Autonomic dysreflexia

Answer 10

A potential medical emergency classically characterized by uncontrolled hypertension and bradycardia, although tachycardia is known to commonly occur.

AD occurs most often in individuals with spinal cord injuries with lesions at or above the T6 spinal cord level, or Gillain barre syndrome, or UTI.

High blood pressure, intense headaches, profuse sweating, facial erythema, goosebumps, nasal stuffiness, a “feeling of doom” or apprehension, and blurred vision. An elevation of 20 mm Hg over baseline systolic blood pressure, with a potential source below the neurological level of injury, meets the current definition of dysreflexia.

Severe hypertension may result in potentially life-threatening complications including seizure, intracranial bleed, or retinal detachment.

Topical nitroglycerin ointment is a convenient and safe treatment.

Question 11

Cavernoma

Answer 11

A cavernoma is a cluster of abnormal blood vessels with small bubbles of blood, usually found in the brain and spinal cord.

They’re sometimes known as cavernous angiomas, cavernous haemangiomas, or cerebral cavernous malformation (CCM).

A cavernoma often does not cause symptoms, but when symptoms do occur they can include:
haemorrhage

seizures

headaches

neurological problems, such as dizziness, slurred speech (dysarthria), double vision, balance problems and tremor

weakness, numbness, tiredness, memory problems and difficulty concentrating

haemorrhagic stroke

Question 12

Risk of occipital strokes

Answer 12

When the occipital lobes of the brain are completely affected by a stroke, it causes total vision loss. This is called “cortical blindness.”

Question 13

Functional neurological disorder

Answer 13

Loss of motor control, sensory issues, speech problems, seizures, visual symptoms, cognitive problems.

Question 14

Gillain barre syndrome

Answer 14

Infection triggered neuropathy from campylobacter jejuni, epstein barr/mono, cmv, mycoplasma pneumoniae. Autoimmune antibody response to pathogen that damages nerves. Can also be caused by things like rheumatoid arthritis and can be chronic.

Symptoms- weakness in extremities, autonomic depression, tingling/numbness, deep aching muscle pain, weakness can affect breathing and blinking, hyporeflexia.

Symptoms peak after 1-4 weeks. Want to take a csf sample and electrodiagnositics. FVC, LP, FBC.

Treatment is time, immunoglobins, plasma exchange, dvt prevention, nerve pain relief: gabapentin and carbamazepine. Physio. Most recover in 6-12 months, some have residual muscle damage, wasting, pain or trouble walking.

Question 15

Myasthenia gravis

Answer 15

Autoimmune disease that destroys acetylcholine receptors on muscles- can’t contract effectively.

Signs- extraoccular muscle weakness, ptosis, diplopia, weakness spreads facially to trunk and limbs (is worse proximally), more severe signs in mornings, resp muscle weakness in 40%, myasthenic resp crisis in 20%. Early onset is <50.

Detect with antiACHR. Treatment is plasmapharesis and ivig or azathioprine and prednisone, thymectomy for thymomas too.

Can give Pyridostigmine to those with just occular symptoms, is an achase inhibitor good to control symptoms.

Question 16

Motor Neurone disease

Answer 16

Neurodegenerative disease of brain and spinal cord leading to paralysis and death. Most commonly due to amyotrophic lateral sclerosis, but can also be progressive bulbar or muscular atrophy, primary lateral sclerosis. Older men are at higher risk. Survival 2-4 yrs.

Signs:
Upper limb weakness, wrist drop/foot drop, hand wasting, fasciculations prior to weakness, gait disorder, trips, chair issues, fatigue on walking, hyper or hyporeflexia. Bulbar onset affects speech and wasting of the tongue with emotional lability. Limb onset is asymmetrical.

Treatment is riluzole neuroprotective glutamate release inhibitor.
Quinine or baclofen for cramping, stiffness/spasticity use tizanidine, dantrolene. For sialorrhea glycopyrrolate antimuscarinic. Lorazepam for choking. Oromorph, sc diamorphine and fentanyl patches for pain.

Question 17

Multiple sclerosis

Answer 17

Multiple sclerosis is an idiopathic autoimmune inflammatory disease of the CNS characterized by inflammation and demyelination of the white matter in brain and spinal cord. . Onset is usually between the ages of 30-40.

Typically patients initially suffer relapsing episodes of neurological dysfunction, with full or partial remissions (relapsing-remitting MS). The relapses may be provoked by infection.
After around ten years patients tend to slowly become more disabled even between relapses (secondary progressive phase MS). Rarely the condition causes a slowly progressive disability from the outset, without relapses (primary progressive MS).

Signs:
Common forms of neurological disturbance in MS include transverse myelitis, optic neuritis (a subacute usually unilateral visual loss with pain on eye movement and a relative afferent pupillary defect – ie the pupil of the affected eye does not respond to light but the consensual reflex is maintained- there is usually a full clinical recovery), and brainstem or cerebellar disturbances. Differential diagnoses include systemic inflammatory conditions such as SLE, sarcoidosis, Hughes syndrome, B12 deficiency, and even rarer conditions such as other causes of white matter CNS diseases (leucodytrophies), or mitochondrial disorders.

A definite diagnosis of MS is given when a patient has had at least two attacks of demyelination at two different sites in the central nervous system at different time-points. Visual evoked potentials may provide paraclinical evidence of demyelination. Lp helps show cause.

Question 18

Differentials to Gillain Barre (3)

Answer 18

Botulism (often a food borne disease) (caused by the bacteria clostridium Botulinum), but wound botulism is increasingly recognized in injecting drug users. Sensation remains intact; the weakness predominantly affects face and neck and is of very rapid onset. These facts help distinguish it from Guillain-Barré syndrome.

Diphtheria is an acute infectious disease caused by corynebacterium diptheriae. However early oropharyngeal symptoms and ciliary paralysis help distinguish it from Guillain-Barre syndrome. The condition is now rare.

Acute intermittent porphyria (an autosomal dominant metabolic problem with increased production and excretion of porphobilinogen can also cause a rapidly advancing symmetrical peripheral neuropathy. However it is rare and there are often accompanying features of abdominal pain.

Question 19

Carpal tunnel syndrome

Answer 19

Carpal tunnel syndrome (compression). The sensory symptoms are confined to the anterior aspect of the lateral 3 and ½ fingers. Thenar atrophy may be noted. Weakness of thumb abduction is a useful clinical sign for the motor component of carpal tunnel syndrome. Hyperflexion of the wrist for 60 seconds may elicit paraesthesia (Phalen’s sign) and tapping the volar wrist over the median nerve (ie, Tinel’s sign) may produce paraesthesia in the median distribution of the hand.

Question 20

Ulnar nerve palsy

Answer 20

Ulnar nerve palsy (compression). Complete ulnar nerve paralysis causes a claw hand; wasting of the small hand muscles results in hyperextension of the fingers at the metocarpophalangeal joints and flexion at the interphalangeal joints. The sensory disturbance of ulnar neuropathy affects the medial 1 and ½ fingers.

Question 21

Cervical root lesions

Answer 21

Cervical root lesions (compression). C5, C6 or C7 root damage can cause sensory loss in the hand though this will extend into the forearm. Muscle weakness will be in the affected myotome and the relevant reflex arcs may be affected.

Question 22

Facioscapular humeral dystrophy (FSH)

Answer 22

Facioscapular humeral dystrophy (FSH) is a slowly progressive muscular dystrophy. It is autosomal dominant. Onset is most typically in adolescence but adult presentations are fairly common. Most patients have a fairly normal life expectancy. Hallmarks are an asymmetric progressive muscle weakness, which, as the name implies particularly affects facial, scapular and humeral musculature. Shoulder girdle symptoms are most likely to trigger presentation. It can be identified as a gene deletion at 4q 35 but no single gene defect has been identified.

Question 23

Duchene’s muscular dystrophy

Answer 23

Duchene’s muscular dystrophy is the commonest childhood onset muscular dystrophy. It is an X-linked recessive disorder and affects males only. In begins in early childhood and runs a relatively rapid course with progressive weakness. Early in the disease pseudohypertrophy of the calf muscles due to fatty infiltration of the damaged muscles is observed . Patients become wheelchair bound and death often occurs in late adolescence. Involvement of cardiac muscle is common. Duchene’s muscular dystrophy is caused by an abnormal gene encoding a protein called dystrophin. This gene can be identified and is used as a diagnostic test. Becker muscular dystrophy is also X-linked and has similar clinical features but is of later onset (around 12) and much more slowly progressive. Patients often live into the fifth decade. It is also caused by abnormality in the dystrophin gene. In Duchene’s muscular dystrophy the dytrophin protein is absent while in Becker’s muscular dystrophy it is present but structurally abnormal.

Question 24

Myotonic dystrophy

Answer 24

Myotonic dystrophy is the commonest adult onset muscular dystrophy. The clinical features make it once of the more distinctive neurological disorders. The extramuscular feature include ptosis, frontal balding, diabetes mellitus, cataracts (usually in later life), atrophy of the facial muscles, and thinning of the sternomastoids. Mild mental retardation, testicular atrophy and gynaecomastia may also occur. Pharyngeal and laryngeal weakness may result in a weak voice, and diaphragmatic weakness may result in hypoventilation. Cardiac involvement is common. Tendon reflexes are often reduced. Skeletal muscle involvement tends to be primarily distal, and the small muscle of the hands are often atrophied. The phenomena of myotonia is distinctive. This is a prolonged contraction of a muscle following brief percussion or contraction. On clinical examination it can be elicited either by asking the patient to perform the provoking actions, or by inducing ‘percussion myotonia’ by tapping the thenar eminence with a reflex hammer and causing a prolonged contracture
Limb girdle dystrophies are a group of later onset muscular dystrophies. They may usually develop in during second decade of life but can present as late as middle age. The shoulder and pelvic muscles are involved with variable rates of progression. The pattern of inheritance is autosomal dominant or recessive.

Question 25

Eligibility for consideration intravenous thrombolysis (IVT)

Answer 25

• Clinical diagnosis of stroke
• Time of symptom onset is known to be less than 3 hours (if patients aged 18 years or older) or 4.5 hours (if patients aged 18-80 years old); symptoms present on waking are timed from onset of sleep.
• If time of symptom onset is between 4.5-6 hours, intravenous thrombolysis may benefit patients aged 18-80 years old. However as alteplase is unlicensed for use > 4.5 hours from stroke onset, careful risks: benefits assessment of treatment must be made by the thrombolysing doctor
• The neurological signs are not resolving spontaneously
• No intracranial bleed on imaging (CT or MRI)

Watch after for 24hrs for vital signs, intracranial or extracranial bleeding, anaphylaxis.

Question 26

Eligibility for consideration intra-arterial thrombectomy/thrombolysis (IAT)

Answer 26

• Clinical diagnosis of stroke
• Symptom onset is definitely known to be less than 6 hours. Time window for IAT treatment of basilar artery thrombosis can be much longer than 6 hours
• No intracranial bleed on imaging (CT or MRI)

Question 27

Management of Suspected Thrombolysis-related Intracranial Haemorrhage

Answer 27

If bp is higher than 220 systolic and/or 121-140 diastolic with two readings 15 minutes apart, give labetalol up to twice six hours apart to reduce bp below threshold 180 systolic and/or 105 diastolic for thrombolysis then do it.

If diastolic over 140, give GTN infusion and do not thrombolyse. If bp raises after, suspect new intracranial haemorrhage. then discontinue, fbc, request cryoprecipitate, CT, tell stroke team, measure with NIH stroke scale. If haemorrhage-
• Consult haematologist regarding the use of cryo-precipitate or FFP (± platelets if count is low)
• Consult neurosurgeon regarding possible haematoma evacuation
• Consider giving 20% mannitol at 0.5g/kg (dose may be repeated)

If cerebral oedema instead- give mannitol.

Question 28

Transverse myelitis

Answer 28

Acute transverse myelitis is inflammation of gray and white matter in one or more adjacent spinal cord segments, usually thoracic. Causes include multiple sclerosis, neuromyelitis optica, infections, autoimmune or postinfectious inflammation, vasculitis, and certain drugs.

Symptoms include bilateral motor, sensory, and sphincter deficits below the level of the lesion. Includes bowel and bladder dysfunction.

Diagnosis is usually by MRI, cerebrospinal fluid analysis, and blood tests. aquaporin-4-IgG auto-antibody .

Likely 10-40 years old.

methylprednisolone or dexamethasone

Question 29

Mitral stenosis (causes, progression, signs)

Answer 29

Causes: congenital or rheumatic fever

Progression: Blood flow forwards is reduced- back pressure- atrial enlargement- risk of turning to afib- emboli and strokes- left sided heart failure and pulmonary oedema.

Signs: Often asymptomatic for 7-9 years. Tapping apex beat, opening snap and mid diastolic murmur.

Question 30

Aortic stenosis (causes, progression, signs)

Answer 30

Causes: congenital or aortic stenosis

Progression: stenosis - inc LV pressure- hypertrophy- risk heart failure and sudden death.

Signs: slow rising pulse, ejection systolic murmur, tight chest, breathless on exertion, angina, dizzy.

Question 31

Mitral regurgitation (causes, progression, signs)

Answer 31

Causes: congenital, MI, rheumatic fever, endocarditis, coronary artery disease, EDS, marfans

Progression: Left atrium dilated- risk afib bc of valves movement- risk emboli and strokes - heart failure and pulmonary oedema- ventricular dilation.

Signs: Often asymptomatic, palpitations, short breath, pansystolic murmur/whistle, midsystolic click, late murmur if prolapses.

Question 32

Aortic regurgitation (causes, progression, signs)

Answer 32

Causes: acute = endocarditis, aortic dissection chronic = syphilis, ankylosing spondylitis, bicuspid aortic valve, rheumatic fever.

Progression: Backwards leak inc volume and pressure in the left ventricle- hypertrophy and dilation- heart failure and pulmonary oedema.

Signs: Often asymptomatic without HF, diastolic murmur, wide pulse pressure, angina, collapsing pulse because backflow quickly reduces blood pressure after contraction.