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4.5. Neurology and SS > 10. Neurological Conditions/syndromes > Flashcards

10. Neurological Conditions/syndromes Flashcards

1
Q

Outline Wernicke’s encephalopathy

A

Thiamine (vit B1) def (decreased intake/absorption)=
- Acute neurological triad of ophthalmoparesis:
1) confusion
2) wide based gait ataxia
3) ophthalmoplegia (nystagmus, conjugate gaze, bilateral lateral rectus palsies)
Additional = decrease consciousness, memory disturbance, peripheral neuropathy, hypotension, hypothermia, ptosis

  • due to haemorrhage in the midbrain

Mx = high dose IV/Im thiamine over 1w, then oral until no risk

Comp = Korsakoff syndrome (hypothalamic damage and cerebral atropy due to repeated thiamine def) - short-term mem loss with disorientation to time

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2
Q

Outline the Olfactory Nerve (CN I) and its palsy

A

Function = olfaction (SS)

Journey = olfactory N –> cribiform foramine –> olfactory bulb –> olfactory tract –> forebrain

Test = smelling salts

PALSY =

  • Ae = ethmoid bone fracture, Alzheimer disease
  • S+S = anosmia
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3
Q

Outline the Optic Nerve (CN II) and its palsy

A

Function = vision (SS)

Journey = retina –> optic N –> optic canal –> optic chiasm –> optic tracts –> visual cortex (occipital lobe)

Test = one eye at a time, visual tests, testing pupils

PALSY =

  • Ae =
  • Ischemic optic neuropathy (microvascular disease)
  • Inflam: MS, sarcoidosis, viral infections (measles, mumps)
  • Trauma
  • Tumors (optic nerve glioma)
  • Impaired nutrition: vitamin B def
  • Drugs: sildenafil, amiodarone, ethambutol
  • S+S = complete transection (ipsilateral blindness + loss of direct pupillary light reflex), increased intracranial pressure (papilledema), compression (optic atrophy)
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4
Q

Outline the Oculomotor Nerve (CN III) and its palsy

A

Function = most extra-ocular muscles, levator palpebrae superioris (M), sphincter pupillae, ciliary muscle (A)

Journey = midbrain –> oculomotor N –> Cavernous sinus –> superior orbital fissure –> Muscles surrounding the eye

Test = inspection of eyelid and pupils

PALSY

  • Ae = ischaemia (HTN, DM), posterior communicating artery aneurysm, RICP tentorial herniation
  • S+S = paralytic squint, ptosis, horizontal diplopia dilated pupil or sparing of the pupil

***external compression sees parasympathetic autonomic function lost first as they are on the outside

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5
Q

Outline the Trochlear Nerve (CN IV) and its palsy

A

Function = superior oblique muscle of the eye (M)

Journey = dorsal aspect of midbrain –> cavernous sinus –> Superior orbital fissure –> superior oblique

Test = eye movements

PALSY =

  • Ae = microvascular damage (diabetes, hypertension, arteriosclerosis), cavernous sinus thrombosis, trauma
  • S+S = diplopia, inability to depress and adduct simultaneously
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6
Q

Outline the Trigeminal Nerve (CN V) and its palsy

A

Function = main sensory nerve of the face (GS), nerve of mastication (M)

Journey = pons –> Trigeminal ganglion 1) Ophthalmic (S): superior orbital fissure –> Sensation to cornea/conjunctiva, 2) Maxillary (S): foramen rotundum –> cheek, 3) Mandibular: foramen ovale –> mandible

Test = sensation to face, muscles of mastication, corneal reflex

PALSY =

  • Ae = tumour, vascular compression, oral surgery, inflam of N, cavernous sinus thrombosis
  • S+S =
  • V1: absent corneal reflex (afferent limb), anesthesia of the forehead
  • V2: anaesthesia of the midface
  • V3: anaesthesia of the chin, lower lip, and anterior two-thirds of the tongue; muscles of mastication are paralyzed
  • Tensor tympani branch: hearing impairment
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7
Q

Outline the Abducens Nerve (CN VI) and its palsy

A

Function = lateral rectus (M)

Journey = lower pons –> cavernous sinus –> superior orbital fissure –> Lateral rectus

Test = eye movements

PALSY

  • Ae = trauma (superior orbital fissure), cavernous sinus thrombosis, SOL causing downward pressure, DM neuropathy
  • S+S = horizontal diplopia, esotropia, Inability to abduct eye (will rotate the head to look to side)
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8
Q

Outline the Facial Nerve (CN VII) and its palsy

A

Branches = temporal, zygomatic, buckle, mandibular, cervical

Function = muscles of facial expression (M), taste ant 2/3 tongue (SS), parasympathetic lacrimal/salivary glands (A)

Journey = bottom of pons –> internal auditory meatus –> petrous part of temporal bone –> few branches inside skull (SS) (A) –> exits skull –> 5 terminal branches (M)

Test = facial expression, corneal reflex

PALSY

  • Ae = Bells (75%), trauma, infection (herpes zoster, HSV, HIV), tumour, preg, sarcoidosis, stroke, COM
  • S+S = central (eyelid/forehead preserved), peripheral (all paralysed)
  • Mx = lubricant ointment, cross taping, acyclovir, pred

***close relation ship to middle ear, parotid gland

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9
Q

Outline the Vestibulocochlear Nerve (CN VIII) and its palsy

A

Function = hearing (SS)

Journey = pons –> internal auditory meatus –> Cochlear

Test = hearing, enquire about balance

PALSY =

  • Ae = bacterial meningitis, lyme disease, acoustic neuroma
  • S+S = sensorineural hearing loss, vertigo, horizontal nystagmus, motion sickness
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10
Q

Outline the Glossopharyngeal Nerve (CN IX) and its palsy

A

Function = sensation of soft palate, tonsils, pharynx, middle ear (GS), posterior 1/3 tongue taste and general sensation (GS/SS), parotid gland (A)

Journey = medulla –> jugular foramen –> down the neck –> tongue, pharynx, carotid sinus, parotid gland

Test = gag reflex, testing in conjunction with vagus nerve

PALSY =

  • Ae = idiopathic, compression by a blood vessel
  • S+S = loss of the gag reflex, flaccid paralysis of soft palate (deviation of uvula away from lesion), sensory loss over soft palate, upper pharynx, posterior third of the tongue (including loss of taste sensation), mild dysphagia

***isolated lesions of glossopharyngeal are rare

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11
Q

Outline the Vagus Nerve (CN X) and its palsy

A

Function = sensation to lower pharynx, larynx (GS), muscles of soft palate, pharynx, larynx (M), parasympathetic to thoracic, abdo viscera (A)

Journey = medulla –> jugular foramen –> down the neck via carotid sheath –> Branches in the neck –> branches in the thorax

Test = noting speech, swallow, cough, say ‘ahhh’ observing uvula

PALSY

  • Ae = trauma, DM, inflam, aortic aneurysms, tumours
  • S+S = loss of the gag reflex, flaccid paralysis of soft palate (deviation of the uvula away), epiglottic paralysis (aspiration), dysphagia, features of vocal cord paralysis, gastroparesis
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12
Q

Outline the (spinal) Accessory Nerve (CN XI) and its palsy

A

Function = trapezius, SCM (M)

Journey = spinal roots from medulla -> enters foramen magnum –> exits jugular foramen –> down IJV –> SCM –> trapezius

Test = shrug shoulders against resistance, turn head against resistance

PALSY

  • Ae = surgeries of lateral cervical region (LN)
  • S+S = trap, SCM paresis
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13
Q

Outline the Hypoglossal Nerve (CN XII) and its palsy

A

Function = movement of the tongue (M)

Journey = medulla –> hypoglossal canal –> Runs down neck –> floor of the tongue

Test = move the tongue

PALSY =

  • Ae = tumour, trauma
  • S+S = atrophy/fasciculation of tongue on side of lesion, also deviates to side of lesion when protruded
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14
Q

What is an acoustic neuromas, which CN can they compress and how would this present?

A

Cerebellopontine angle (CPA) tumours, most common neoplasms in posterior fossa (5-10% intracranial tumours). Most CPA tumours are benign, 85% vestibular schwannomas (acoustic neuromas), lipomas, vascular malformations, haemangiomas

Benign tumour that develops on vestibular + cochlear Ns

COMPRESS:

  • Vestibulocochlear = gradual hearing loss, ringing in the ear, and dizziness
  • Facial = loss of facial expression
  • Trigeminal = loss of sensation in the face

Mx = monitoring, brain surgery, RT

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15
Q

Outline Horner’s syndrome

A

Ae/Path = interrupted function of sympathetic nerve fibres (start in hypothalamus, run via upper spinal cord, near carotid artery, to face

  • Birth trauma to the neck and shoulder
  • Stroke in the brainstem
  • Injury, blood clot, or dissection of the carotid artery
  • Trauma/surgery to neck, upper spinal cord, chest
  • Tumour: Pancoast, neuroblastoma
  • Migraines or cluster headaches
  • Demyelinating disease

S+S = miosis (constriction of the pupil), ptosis (drooping of the upper eyelid), and anhidrosis (absence of sweating of the face)

Mx = surgery, RT/chemo

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16
Q

What is CJD

A

Creutzfeldt–Jakob disease

Ae/Path = fatal degenerative brain disorder, prion (misfolded protein)

S+S = memory problems, behavioural changes, poor coordination, visual disturbances, dementia, involuntary movements, blindness, weakness, coma

Ix = MRI, EEG, spinal tap

Mx = no Tx, opioids for pain Na valproate/clonazepam for involuntary movements

*** most die of pneumonia due to impaired coughing reflexes

17
Q

What is Argyll Robertson Pupil and how should it be Mx?

A

Bilateral small pupils that reduce in size on a near object (accommodation reflex), but do not constrict when exposed to bright light (absence of direct and consensual light reflex)

  • highly specific sign of neurosyphilis
  • can also be caused by DM neuropathy

Path = believed to be the result of bilateral damage to the pretectal nuclei in the midbrain
- hallmark of tertiary NEUROSYPHILIS

Mx = crystalline penicillin 24 mU intravenous per day for 10 to 14 d

18
Q

Describe a syringomyelia

A

Generic term referring to a disorder in which a cyst (syrinx) or cavity forms within the spinal cord - can expand over time destroying spinal cord

Ae =

  • Congenital: malformation in the brain, cerebellum protrudes into cervical spinal canal, syrinx may then develop
  • Acquired: trauma, meningitis, haemorrhage, tumour, arachnoiditis

S+S = paraesthesia, paralysis, weakness, stiffness in the back, shoulders, and extremities

Ix = brain/spinal CT/MRI

Mx = surgery

19
Q

Outline Cavernous sinus syndrome

A

Ae =

  • metastatic tumours
  • direct extension of nasopharyngeal tumours
  • meningioma
  • pituitary tumours or pituitary apoplexy
  • aneurysms of the intracavernous carotid artery
  • carotid-cavernous fistula
  • bacterial infection causing cavernous sinus thrombosis
  • aseptic cavernous sinus thrombosis

Path = result from the involvement of CNs passing through cavernous sinus (Oculomotor, Trochlear, Abducens)

S+S:

  • Ophthalmoplegia = compression of the oculomotor nerve, trochlear nerve, and abducens N
  • Sensory loss = from compression of Trigeminal 3 divisions (ophthalmic V1, maxillary V2, mandibular V3)
  • Horner’s syndrome = involvement of the carotid ocular sympathetics
  • Proptosis (pulsating exophthalmos suggests a direct C-C fistula)

Ix = CT, MRI

Mx =

  • Mets: RT
  • Pit tumour: prolactinoma dopamine agonists, resection
  • Meningioma: RT
  • Aneurysms: endovascular balloon occlusion
  • Infection: Abx
20
Q

What is Subacute combined degeneration of spinal cord (Lichtheim’s disease)?

A

Degen of the posterior and lateral columns due to vit B12 def (most common), vitamin E def, copper def, usually associated with pernicious anaemia

Ae = diet def, pernicious anaemia, CF (vit E def)

S+S =

  • weakness of legs, arms, trunk, tingling and numbness that progressively worsens
  • vision changes
  • change of mental state
  • bilateral spastic paresis
  • pressure, vibration, touch sense diminished
  • +ve Babinski sign may be seen

Ix = bloods

Tx = vit B12/E supplements

21
Q

How do pituitary tumours present and how should they be Mx?

A

Benign adenoma, invasive adenoma, and carcinomas

S+S =

  • Prolactinoma (prolactin) = amenorrhea, galactorrhoea, loss of axillary and pubic hair, hypogonadism, gynecomastia, ED
  • Somatrotrophic (GH) = acromegaly in adults; gigantism in children
  • Corticotropic (ACTH) = Cushing’s
  • Visual field defects = bitemporal hemianopsia (compression of optic chiasm)
  • RICP = headache, N+V, papilledema

Ix = CT, MRI, hormones

Mx =

  • Prolactinoma = dopamine agonist (inhib prolactin)
  • Somatotrophic = long-acting somatostatin analogues (inhib GH)
  • Trans-sphenoidal adenectomy
  • RT
22
Q

What is the aetiology and pathophysiology of myasthenia gravis?

A

Ae = Autoimmune: AChR Ab, MuSK Ab

Path = autoantibodies (IgG) blocking postsynaptic acetylcholine receptors (AChR), impairing neuromuscular transmission

Associated with abnormalities in the thymus (thymic hyperplasia 65%, thymic tumour 30%) and other autoimmune disorders: thyroiditis, graves, RA, SLE, pernicious anaemia

Precipitated by = stress, preg, menses, sec illness, thyroid dys, trauma, temp extremes, hypoK, drugs (aminoglycosides, B-blockers, chloroquine, Mg, ACEi), surgery

23
Q

How does myasthenia gravis present and what are the possible complications?

A

Bimodal pres = 20-30y, 50-70y

FATIGABILITY of skeletal muscle

  • Extraocular muscle involvement (90%)
  • Bulbar Sx: dysphagia, dysphonia, dysarthria
  • Facial + limb weakness
  • Resp muscle involvement

Ptosis (drooping of upper eyelid)

*** no sensory or reflex loss, no autonomic involvement, pupil unaffected

COMP = resp failure (spirometry monitoring), aspiration, resp infection, myasthenic crisis (slack facial muscles, weak neck, drooling, unsafe swallow), cholinergic crisis (excess AChase inhib - excess stim of striated muscle, flaccid paralysis, resp failure, miosis, SSLUDGE syndrome), DVT

24
Q

How should suspected MG be Ix?

A

AChR Ab, MuSK Ab

Tensilon test - injection of Tensilon (edrophonium), then muscle strength is evaluated (caution asthma, MI, bradycardia)

Electromyography (EMG) - looking for fatigability, AP reduce

CT thorax - thymic tumours

Ice test - ptosis

25
Q

How should MG be Mx?

A

Supportive

Bedside spirometry = FVC falling rapidly or <20 may need ventilation

Blood gas = in CO2, dec O2, acidosis - late manifestations of T2 resp failure

MEDS

  • Symptomatic = AChase inhib (pyridostigmine): enhance neurotransmitter
  • Corticosteroids: decrease immune response
  • Azathiprine: steroid sparing

IV Ig

Plasmapheresis: removes AChR Abs

26
Q

Outline Guillian Barre’ syndrome (GBS)

A

Ae = unknown

Path =

  • Immune-mediated demyelination of LMN, sensory neurons, autonomic neurons
  • post infectious
  • most common subtype: AIDP acute inflam demyelinating polyradiculoneuropathy (95%)
  • T cells destroying schwann cells - impairing neurotransmission to periphery

S+S =

  • 1-3w post URTI/GI infection
  • backache, muscle cramps (worse at night), hyporeflexia, ascending paralysis
  • sensory loss
  • autonomic instability: BP, pulse
  • Sx peak at 4w, brief plateau, recover over weeks/months

Ix = EMG, CSF (in protein), campylobacter serology if GI upset, anti-ganglioside Ab, stool culture, throat swab

Mx = spirometry, ventilation, ECG monitoring, nutritional support +/- NG tube, DVT prophylaxis, urinary catheter, laxative, opiates
- IV Ig, plasmapheresis

Comp = autonomic dysfunction (sinus tachy, brady, tachyarrhythmias, postural hypotension, HTN, urinary retention, ileus), resp failure, DVT, SIADH, renal failure

27
Q

What is Ramsay Hunt syndrome

A

(herpes zoster oticus) occurs when a shingles outbreak affects the facial N near one of your ears

In addition to the painful shingles rash (peri-aural vesicles), Ramsay Hunt syndrome can cause facial paralysis and hearing loss in the affected ear

Mx = acyclovir 800mg 5x per day for 7d

28
Q

Outline Brown-sequard syndrome

A

Ae = hemisection of the spinal cord: tumour, trauma, ischemia, TB, MS

S+S = ipsilateral spastic paralysis and loss of proprioception, contralateral loss of pain and temperature sensation

Ix = MRI spine

Mx = directed at the cause, steroids to dec inflam

4.5. Neurology and SS (17 decks)

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