Assessment of cranial nerve mononeuropathy

sudden and profound painless vision loss may have new onset headache, jaw claudication, proximal muscle weakness, anorexia and weight loss, incidence increases with age (more so at >80 years)

severe visual loss (often unable to see hand motions or worse), visual field defect, relative afferent pupillary defect, pale optic nerve swelling in affected eye with small optic nerve in fellow eye, optic nerve haemorrhages may be present; tenderness of scalp over temporal areas; abnormalities of the temporal, occipital, and facial arteries including thickening, tenderness, and nodularity; check for signs of involvement of extracranial arteries, such as bruits over the subclavian and axillary arteries

sudden loss of part of visual field in one eye (often inferior eye); painless; may have headache; may be noted on waking; 75% of affected people are >50 years; history of phosphodiesterase type 5 inhibitor use for erectile dysfunction (which may potentially be associated with an increased risk of non-arteritic anterior ischaemic optic neuropathy)

visual field defect; relative afferent pupillary defect; optic nerve swelling in affected eye with small optic nerve in fellow eye; optic nerve haemorrhages may be present

woman 18-40 years old, acute, painful monocular visual loss, loss of colour vision, sensory disturbances, tingling, numbness, weakness of limbs

afferent pupillary defect (asymmetric reaction to light when shined back and forth between the two eyes), normal fundus or mild optic disc oedema, tremor, gait disturbance, limb weakness

child, recent viral infection or immunisation, peri-ocular pain

triad of visual loss, swollen optic disc, and a macular star

sudden-onset severe headache, neck pain, photophobia, nausea

may have altered level of consciousness, abnormal pupillary function

neck pain, stiffness, photophobia

non-blanching rash, may have additional cranial nerve deficits, altered level of consciousness, or fever; meningismus, positive Kernig's and Brudzinski's signs

most commonly unilateral, often progressive, paroxysmal, lancinating pain, lasts a few seconds to minutes, often precipitated by triggers (e.g., touch, chewing), commonly in V2 and V3 distributions

may have loss of sensation in the distribution of one or all trigeminal branches or weakness of the muscles of mastication

3-4 days of burning or lancinating pain with or without vesicular rash in the distribution of 1 or more trigeminal nerve branches; postherpetic neuralgia: previous shingles infection, pain persisting despite resolution of rash

erythematous maculopapular rash followed by clear vesicles, rash does not cross midline

known MS, advanced disease, woman 18-40 years old, acute painful monocular visual loss, loss of colour vision, tingling, numbness, weakness of limbs

normal fundus or mild optic disc oedema, tremor, gait disturbance, limb weakness

acute onset of unilateral facial weakness, affects upper and lower face, may have history of viral prodrome

unilateral facial weakness involving the forehead, no other neurological findings

sudden-onset (<72 hours) unilateral facial weakness (partial or complete), affects upper and lower face, severe ear and facial pain, vesicular lesions involving the pinna, possible hearing loss, tinnitus, or vertigo

parotid and/or neck masses, vesicular rash or blisters on the head, neck, and shoulders, cranial neuropathies (dermatomal rash, facial weakness, and ocular findings), presence of vesicles or blisters in the pinna and ear canal

acute onset, possible contralateral limb weakness, dysphagia, dysarthria

sparing of upper facial muscles, may have decreased power of contralateral limbs, associated cranial nerve palsies, may have altered mental status

acute-onset vertigo over several hours, lasts days to weeks, slowly remitting, nausea, vomiting, sweating, imbalance, disequilibrium, occasionally unilateral hearing loss

horizontal-torsional nystagmus in primary gaze, worse with gaze deviation to opposite direction of slow phase of nystagmus, head thrusts; corrective saccades in direction of abnormality, past-pointing; deviation of arms to side of lesion; Unterberger stepping test: rotation to abnormal side

age-related; difficulty in speech discrimination; slow, gradual hearing loss, usually bilateral

normal otoscopic exam, Weber's test may lateralise to least affected side

new-onset vestibulocochlear nerve dysfunction after starting drug: aminoglycosides, platinum-based chemotherapeutic agents, salicylates, quinine, loop diuretics; tinnitus often bilateral and symmetrical

Rinne's test positive (normal air conduction > bone conduction), Weber's test lateralises to normal side, high-frequency hearing loss

recent thoracic or neck surgery, concomitant hoarseness

no palatal droop or uvular deviation

new-onset hoarseness, history of smoking, cough

auscultation: may hear decreased air entry, decreased percussion note, or normal

isolated spinal accessory nerve palsy, recent lymph node biopsy, neck dissection, jugular vein cannulation, carotid endarterectomy, or cosmetic rhytidectomy (face lift)

weakness of sternocleidomastoid (SCM) and trapezius muscles, mild scapular winging

acute onset, contralateral limb weakness, associated cranial nerve deficits; with progressive bulbar palsy: difficulty in chewing, swallowing, or talking

tongue weakness, deviation to ipsilateral side on protrusion, atrophy, fasciculations, flaccidity; contralateral hemiplegia with facial sparing, contralateral loss of position and vibration sensation, sparing of pain and temperature

temporal relationship to head trauma, occipital and side impacts most likely to cause olfactory loss

scar, may have other residual neurological deficits

Alzheimer's disease: poor short-term memory, disorientation in time and place; Parkinson's disease: slow movement and rigidity, problems with posture and balance

Alzheimer's: difficulty in performing familiar tasks; Parkinson's: pill-rolling tremor most obvious at rest, increased tone, may have quiet or monotonous speech

isolated or associated with absent or incomplete puberty, dyspareunia, decreased libido, and erectile dysfunction or amenorrhoea (Kallmann's syndrome)

lack of secondary sexual characteristics, micropenis, lack of scrotal pigmentation, decreased muscle mass in Kallmann's syndrome

early-morning headache, vomiting, ipsilateral anosmia, contralateral seizures, and altered level of consciousness (late signs)

optic atrophy and contralateral papilloedema, ipsilateral central scotoma (Foster Kennedy syndrome)

trauma to the outer brow or adjacent temporal bone

normal fundus or optic pallor after 3 to 4 weeks

bilateral symptoms, progressive visual loss, signs of raised intracranial pressure; early-morning headache, vomiting, seizures and altered level of consciousness (late signs); meningioma: middle-aged woman; glioma: insidious visual loss, proptosis

optic pallor, optociliary shunts; large tortuous vascular loops on optic disc, raised intracranial pressure; papilloedema

young woman, obesity, headache worse in the morning and on coughing or sneezing, nausea, vomiting, tinnitus, visual obscurations

bilateral papilloedema, associated cranial nerve palsies of III, IV, VI

SLE: fatigue, weight loss, fever, anaemia, arthralgia; Sjogren's syndrome: fatigue, dry eyes, dry mouth; granulomatosis with polyangiitis: ocular symptoms of redness, pain, diplopia, cutaneous rash; Behcet's disease: painful ulceration, impaired speech and balance, eye pain and blurred vision with CNS involvement

SLE: butterfly rash, photosensitive rash, oral ulceration; Sjogren's: dental caries, corneal ulceration; granulomatosis with polyangiitis: proptosis, retinal haemorrhage/exudate, skin lesions; Behcet's: oral and genital ulceration, uveitis

young man, may have maternal family history of optic neuropathy, acute visual loss in one eye, contralateral eye affected within weeks

optic disc pallor, decreased visual acuity, abnormalities of pupillary reflex

exposure to ethambutol, infliximab, sildenafil, or amiodarone; tobacco use; deficiency of vitamins B1, B2, B9, and B12 due to famine or excessive ethanol; simultaneous bilateral symptoms, blurred central vision

central scotoma, colour vision reduction; with nutritional cause: optic disc may be slightly hyperaemic; with amiodarone: optic disc swelling and haemorrhage; with ethambutol: disc usually normal

typically middle-aged man or woman; visual loss in one eye, often followed by visual loss in the other eye after days to months; may or may not have history of myelitis

reduced visual acuity and reduced colour vision ± brainstem syndrome ± spasticity, weakness, or sensory disturbance in legs

sudden-onset non-pupil-sparing third nerve palsy, may have history of severe headache that is worse in the morning, nausea and vomiting

may have contralateral or ipsilateral hemiplegia with or without significant alteration in consciousness, ipsilateral pupillary dilation

prolonged headache, photophobia, nausea and vomiting, family history

no specific examination findings, temporary palsy of III, IV, or V resolves with resolution of migraine, pupil is not typically affected

acute head trauma or history of past head trauma with loss of consciousness

altered level of consciousness, associated residual focal neurology

can be asymptomatic, may have peripheral visual defect, loss of balance, co-ordination or speech problems

may have pupillary involvement, visual field defects

pulsatile tinnitus, progressive visual loss, proptosis, eye pain, history of trauma or connective tissue disease

pulsatile exophthalmos, chemosis, ocular bruit

eye pain and unilateral headache, proptosis, chemosis, and ophthalmoplegia, may have periorbital oedema

ptosis and mydriasis, papilloedema and retinal vein dilation, decreased corneal reflex, may have sensory loss in skin supplied by first 2 branches of trigeminal nerve

craniopharyngioma in a child, acute loss of vision, macrocephaly, growth failure; in adults: insidious loss of vision, amenorrhoea or erectile dysfunction

may have papilloedema (with raised intracranial pressure), bitemporal hemianopia

complete ophthalmoplegia, recent use of sildenafil or cocaine

normal pupil

typically obese young adult woman with headaches, but no focal neurological signs; bilateral visual loss

constriction of visual field due to papilloedema

<3 months old, present since birth

no specific associated features; if III affected: pupil involvement and ptosis

recent LP, transient symptoms of lateral gaze palsy

isolated lateral rectus palsy

neck pain, stiffness, photophobia

non-blanching rash, may have additional cranial nerve deficits, altered level of consciousness or fever; meningismus, positive Kernig's and Brudzinski's signs

most commonly unilateral, often progressive, paroxysmal, lancinating pain, lasts a few seconds to minutes, often precipitated by triggers (e.g., touch, chewing), commonly in V2 and V3 distributions

may have loss of sensation in the distribution of one or all trigeminal branches or weakness of the muscles of mastication

history of autoimmune disease, numbness with or without associated dysaesthesias and paraesthesias, often bilateral symptoms; SLE: fatigue, weight loss, fever, anaemia, arthralgia; Sjogren's syndrome: fatigue, dry eyes, dry mouth

impairment of trigeminal nerve reflexes, except jaw-jerk reflex (preserved); SLE: butterfly rash, photosensitivity, oral ulceration; Sjogren's: dental caries, corneal ulceration

otalgia, otorrhoea, hearing loss, headaches, neuropathic pain

loss of sensation in trigeminal distribution or weakness of the muscles of mastication

recent or remote history of trauma to orbit, mid-face, mandible, or skull base

crepitus over facial fracture site, inability to open jaw, malalignment of teeth; with skull-base fracture: Battle's sign, periorbital ecchymosis, CSF rhinorrhoea, bleeding from nose or ear

toothache, throbbing pain, possible loosening of tooth

pain exacerbated by tapping on top of tooth, gum recession, erythematous gum line

symptoms of trigeminal neuralgia: unilateral, often progressive, paroxysmal, lancinating pain, lasts a few seconds to minutes, often precipitated by triggers (e.g., touch, chewing), commonly in V2 and V3 distributions

ipsilateral facial pain and temperature loss

recent oral surgery before onset

loss of sensation in the distribution of the inferior alveolar nerve

known extracranial tumour (e.g., nasopharyngeal carcinoma or neck malignancy with mandibular metastases)

unilateral chin or jaw numbness (numb chin syndrome)

Chiari I and II with syringomyelia: stiffness and pain in back and shoulders, facial pain, fatigue, severe headaches worse on straining, coughing, or sneezing

isolated or may include multiple cranial nerve neuropathies with Chiari I and II malformations

severe unilateral headache, acute-onset painful ophthalmoplegia, numbness across the forehead, diplopia, fatigue

mild proptosis, ophthalmoplegia, may have Horner's syndrome

difficulty in swallowing and speaking, ataxia, facial pain, vertigo

sensory impairment of trunk and limbs on contralateral side, and sensory and motor impairment of the face on the ipsilateral side, nystagmus, absent corneal reflex on ipsilateral side

cloudy vision, diplopia, systemic involvement; fatigue, malaise, cough, shortness of breath

chorioretinal granulomas, swelling of optic nerve head with haemorrhage or elevation; systemic involvement: papilloedema, fine inspiratory crackles on lung auscultation, lymphadenopathy, skin lesions

slowly progressive, hearing loss often present, may have tinnitus or hyperacusis, dysgeusia, and reduced tearing, contralateral limb weakness, ipsilateral sixth nerve palsy

weakness of both upper and lower facial muscles, ipsilateral sensorineural hearing loss, reduced power in contralateral limbs

recent trauma, dysgeusia

bruising and crepitus in temporal region, weakness of both upper and lower facial muscles, basal skull fracture; periorbital ecchymosis, Battle's sign, CSF rhinorrhoea, bleeding from nose or ear

neck pain, stiffness, photophobia

non-blanching rash, may have additional cranial nerve deficits, altered level of consciousness, or fever; meningismus, positive Kernig's and Brudzinski's signs

temporal relationship to recent parotid gland surgery or otological surgery (tympanoplasty, mastoidectomy, removal of exostoses)

paralysis of muscles supplied by one or more branches

otalgia, otorrhoea, retroauricular pain

unilateral facial weakness, retroauricular cellulitis or swelling, often not febrile

painless mass in the cheek, increasing size

may have regional lymphadenopathy, rarely blood or pus exudate from Stensen's duct

HIV-positive; fevers, night sweats, diarrhoea

signs of HIV: lymphadenopathy, skin rashes, thrush infection, Kaposi's sarcoma

tick exposure, often bilateral facial weakness, preceding erythema migrans

tick bite, bilateral facial weakness

hearing loss, ipsilateral high-pitched tinnitus, vertigo, disequilibrium, unilateral facial weakness; if VII involved: neurofibromatosis; bilateral symptoms possible

Rinne's test positive (normal air conduction > bone conduction), Weber's test lateralises to normal side; involvement of VII: unilateral facial weakness

gradual development, other cranial nerves affected; with VIII: unilateral hearing loss or tinnitus; with IX: hoarseness, dysphagia, or dyspnoea

IX; often asymptomatic, may have speech difficulty, problems in swallowing or breathing

neck or oropharyngeal mass, dysphagia or dyspnoea, Eustachian tube dysfunction, hoarseness

painless palpable neck mass, thrill to auscultation if vascular

neck pain, stiffness, photophobia

non-blanching rash, may have additional cranial nerve deficits, altered level of consciousness, or fever; meningismus, positive Kernig's and Brudzinski's signs

otalgia, otorrhoea, hearing loss, headaches, slurred speech, difficulty swallowing

dysarthria and weakness of palatal elevation associated with loss of sensation in trigeminal distribution, neuropathic pain, weakness of the muscles of mastication

recent trauma, temporal relationship to nerve palsy, may have associated cranial nerve deficits

periorbital ecchymosis, Battle's sign, CSF rhinorrhoea, bleeding from nose or ear

possible neck pain, hoarseness, dysphagia

may have tenderness to neck palpation, fever

paroxysmal unilateral pain at base of tongue and deep neck, elicited by chewing or swallowing

reproducible pain at base of tongue or deep neck with chewing or swallowing, palpable styloid possible

history of cardiovascular disease, hoarseness

no palatal droop or uvular deviation

blunt or penetrating trauma to the neck

weakness of sternocleidomastoid (SCM) and trapezius muscles, mild scapular winging

pain and decreased shoulder function; associated tenth nerve involvement: hoarseness, dysphagia

trapezius atrophy, drooping shoulder at rest; with tenth nerve involvement: palatal droop and uvular deviation

acute onset, contralateral limb weakness, associated cranial nerve deficits; with progressive bulbar palsy: difficulty chewing, swallowing, or talking

tongue weakness, deviation to ipsilateral side on protrusion, atrophy, fasciculations, flaccidity; contralateral hemiplegia with facial sparing, contralateral loss of position and vibration sensation, sparing of pain and temperature

progressive symptoms, difficulty chewing, swallowing, or talking, muscle weakness in neck, arms or legs; respiratory weakness

tongue wasting, weakness, and fasciculations; nasal speech, reduced or absent gag reflex, drooling of saliva; sensory examination of cranial nerves and limbs normal

with syringomyelia: stiffness and pain in back and shoulders, facial pain, fatigue, severe headaches worse on straining, coughing, or sneezing

tongue weakness, deviation to ipsilateral side on protrusion (if unilateral), atrophy, fasciculations, flaccidity; frequent involvement of other cranial nerves

gradual development, common involvement of other cranial nerves

tongue weakness, deviation to ipsilateral side on protrusion, wasting, fasciculations, flaccidity

neck pain, stiffness, photophobia

non-blanching rash, may have additional cranial nerve deficits, altered level of consciousness, or fever; meningismus, positive Kernig's and Brudzinski's signs

otalgia, otorrhoea, hearing loss, headaches

tongue weakness, deviation to ipsilateral side on protrusion, atrophy, fasciculations, flaccidity; often no fever

possible neck pain, fever; tenth nerve involvement: dysphagia and hoarseness

tongue weakness, deviation to ipsilateral side on protrusion; may have tenderness to neck palpation, fever

recent or remote history of blunt head trauma or penetrating, gunshot, or stab wound to neck, acute onset of XII palsy

tongue weakness, deviation to ipsilateral side on protrusion, atrophy, fasciculations, flaccidity; entry wound, bleeding, other signs of head trauma, associated cranial nerve deficits, altered level of consciousness

headache, pulsatile tinnitus, seizures, stroke

tongue weakness, deviation to ipsilateral side on protrusion, atrophy, fasciculations, flaccidity; vision disturbance, balance problems, hemiparesis, numbness and tingling, speech difficulty

history of trauma, neck manipulation, headache, dysarthria, ipsilateral neck pain

tongue weakness, deviation to ipsilateral side on protrusion; carotid bruit, Horner's syndrome may be seen

neck irradiation, carotid endarterectomy, or central line placement

tongue weakness, deviation to ipsilateral side on protrusion, atrophy, fasciculations, flaccidity