Cranial nerve palsies

• Etiology
  • Trauma: especially impact to the lateral and occipital regions (e.g., ethmoid bone fracture)
  • Neurodegenerative diseases (e.g., Alzheimer disease)
  • Congenital (e.g., Kallmann syndrome)
  • Intracranial space-occupying lesion
• Clinical features: anosmia

• Etiology
  • Ischemic optic neuropathy (i.e., caused by microvascular disease)
  • Inflammation: multiple sclerosis, sarcoidosis, viral infections (e.g., measles, mumps)
  • Trauma
  • Tumors (e.g., optic nerve glioma)
  • Impaired nutrition: vitamin B deficiency
  • Drugs: sildenafil; , amiodarone; , ethambutol
• Clinical features: impaired vision
  • Complete transection → ipsilateral blindness + loss of direct pupillary light reflex
  • Increased intracranial pressure → papilledema
  • Compression (e.g., tumor) → optic atrophy

Neuroanatomy

Structure	Anatomy	Typical lesions	Localizing clinical features
Oculomotor nuclei	A pair of oculomotor nuclei are located at the level of the midbrain, ventral to the aqueduct of Sylvius Fascicles (efferent fibers) from each oculomotor nucleus pass through the red nuclei and the emerge anteriorly through the medial aspect of the cerebral peduncle as the oculomotor nerve	Ischemic stroke involving branches of the posterior cerebral artery	Benedikt syndrome Weber syndrome
Basilar segment	The oculomotor nerve emerges anteriorly from the midbrain into the interpeduncular fossa and passes between the posterior cerebellar artery and the superior cerebellar artery. The oculomotor nerve then courses in the subarachnoid space over the base of the skull alongside the posterior communicating artery. The parasympathetic pupillomotor fibers from the Edinger-Westphal nucleus join the oculomotor nerve in the basilar segment and lie superficially on the nerve.	Aneurysm of the posterior communicating artery Base of skull fractures Basal meningitis	Recent history of headache Isolated oculomotor nerve palsy with a fixed, dilated pupil
		Tentorial (uncal) herniation	Features of elevated intracranial pressure Abducens nerve palsy Hutchinson pupil
Intracavernous segment	The oculomotor nerve pierces the dura mater, enters the cavernous sinus, and runs along the lateral wall. In the anterior part of the cavernous sinus, the oculomotor nerve divides into a superior division and an inferior division, which enter the orbital cavity via the superior orbital fissure.	Cavernous sinus thrombosis Tolosa-Hunt syndrome Internal carotid artery aneurysms Large parasellar tumors (e.g., meningioma)	Associated palsies of the trochlear (IV), trigeminal (V1, V2 nerve), and/or abducens (VI) nerve
Intraorbital segment	The superior division innervates the levator palpebrae superioris and the superior rectus muscle. The inferior division inferior innervates the inferior rectus, the medial rectus, the inferior oblique muscle.	Tumors within the orbital cavity Trauma Orbital cellulitis	Loss of vision Pain Proptosis Associated palsies of the trochlear (IV) and/or abducens nerve (VI) Non-reactive pupil
Ischemic microangiopathy can affect any part of the oculomotor nerve from the basilar segment and typically results in an oculomotor nerve palsy with pupillary sparing.

Isolated oculomotor nerve palsy

• Etiology
  • Ischemic microangiopathy (more common in patients above the age of 60)
    • Diabetic cranial mononeuropathy
    • Hypertension
  • Compression or transection
    • Posterior communicating artery aneurysm
    • Base of skull fractures
  • Demyelinating neuropathy
    • Guillain-Barre syndrome and its variant (Miller-Fisher syndrome)
    • Multiple sclerosis
• Clinical features
  • Paralytic squint
    • Adduction weakness
    • The affected eye looks outwards (exotropia) and downwards (hypotropia)
  • Ptosis
  • Horizontal diplopia that is worse when the head is turned away from the side of the nerve palsy
  • Pupillary involvement
    • Compressive lesions: a non-reactive, dilated pupil
    • Ischemic microangiopathy; or demyelinating cranial neuropathies: typically sparing of the pupil
• Diagnostics
  • A dilated pupil → often a compressive lesion (e.g., posterior communicating artery aneurysm)
    • Best initial test: urgent MRI with MR angiography
    • If MRI is normal: perform a lumbar puncture
  • Pupillary sparing → often due to ischemic microangiopathy
    • Assess risk factors for atherosclerosis or arteritis (e.g., blood pressure, glucose, ESR, lipid profile)
    • If no recovery at 3 months: perform an MRI
• Treatment
  • Compressive lesions: surgery
    • Posterior communicating artery aneurysm: urgent neurosurgical clipping or endovascular coiling
  • Ischemic microangiopathy or demyelinating lesions: medical management with adequate control of the underlying disease
• Prognosis
  • Compressive lesions: Partial recovery may occur within 6–7 months.
  • Ischemic microangiopathy or demyelinating lesion: usually resolves spontaneously within 3–4 months.

For more details about oculomotor nerve lesions and drugs affecting pupillary size, see the learning card pupillary abnormalities.

With an isolated ocuLOVEmotor nerve palsy, nobody loves you when you are down and out (the pupil points outwards and downwards)!

Compression of the oculomotor nerve can cause isolated pupillary dilation due to injury of the parasympathetic fibers. Microangiopathy (e.g., due to diabetes mellitus) typically affects the deeper somatic fibers first, causing ophthalmoplegia without pupillary dilation.

The rule of pupillary involvement is not infallible since approx. 20% of patients with ischemic microangiopathy may have pupillary involvement.

In acquired lesions of fourth nerve, patients report vertical, torsional, or oblique diplopia. Diplopia is usually worse on downgaze and gaze away from side of affected muscle.

• Etiology
  • Microvascular damage (diabetes, hypertension, arteriosclerosis)
  • Trauma
  • Cavernous sinus thrombosis
• Clinical features
  • Extorsion of the eye: inability to depress and adduct the eyeball simultaneously (the pupil shoots upward during attempted adduction of the eyeball)
  • Diplopia (double vision)
    • Vertical or oblique diplopia , which is exacerbated on downgaze (e.g., reading)
    • Diplopia worsens when the patient turns their head towards the paralyzed muscle; → The patient compensates by turning his head to the opposite side of the lesion.
  • Mild esotropia

• Etiology
  • Tumor
  • Vascular compression of the nerve
  • Oral surgery
  • Inflammation of the nerve
  • Cavernous sinus thrombosis
• Clinical features
  • Peripheral trigeminal nerve lesions
    • Ophthalmic nerve (V₁) is affected → absent corneal reflex (afferent limb), anesthesia of the forehead
    • Maxillary nerve (V₂) is affected; → anesthesia of the midface
    • Mandibular nerve (V₃) is affected → anesthesia of the chin, lower lip, and anterior two-thirds of the tongue; muscles of mastication are paralyzed
      • The jaw deviates towards the side of the lesion because of unopposed action from the opposite pterygoid muscle.
    • Involvement of any of the three branches can cause trigeminal neuralgia.
  • Lesion of the tensor tympani branch → hearing impairment (particularly difficulty hearing low-pitched sounds)
  • Lesions of the trigeminal nerve nuclei: Depending on which nuclei are affected; , the patient may present with ipsilateral weakness of muscles of mastication and/or ipsilateral loss of sensation.
  • Complete trigeminal nerve lesions are rare.

References:^[3]

• Most common ocular cranial nerve palsy
• Etiology
  • Tumor
  • Trauma (e.g., at the superior orbital fissure of the sphenoid bone)
  • Pseudotumor cerebri
  • Cavernous sinus thrombosis
  • Duane syndrome: rare, congenital palsy of the abducens nerve
• Clinical features
  • Horizontal diplopia, which is worse when looking at distant objects
  • Esotropia: medial deviation of one or both eyes at primary gaze

References:^[4][5]

• See facial nerve palsy.

• Etiology: often unknown; may be associated with compression by a blood vessel
• Clinical features
  • Loss of the gag reflex (afferent limb)
  • Loss of the carotid sinus reflex
  • Flaccid paralysis of the soft palate → deviation of the uvula away from the lesion (similar to vagus nerve lesions)
  • Sensory loss over the soft palate, upper pharynx, and posterior third of the tongue (including loss of taste sensation)
  • Mild dysphagia
  • Glossopharyngeal neuralgia: throat and ear pain

Lesions that affect the glossopharyngeal nerve typically also affect the vagus nerve because the glossopharyngeal nerve exits the medulla above the vagus nerve.

• Etiology
  • Trauma
  • Diabetes
  • Inflammation
  • Aortic aneurysms
  • Tumors
• Clinical features
  • Loss of the gag reflex (efferent limb)
  • Flaccid paralysis of the soft palate → nasal speech and deviation of the uvula away from the lesion
  • Epiglottic paralysis → aspiration
  • Dysphagia
  • Features of vocal cord paralysis
    • Dysphonia (hoarseness)
    • The vocal cord assumes a paramedian position.
  • Dysfunction of vagal nerve in the stomach → features of gastroparesis (poor gastric emptying)

• Etiology
  • Tumors
  • Trauma
• Clinical features
  • Atrophy and fasciculation of the tongue on the side of the lesion
  • The tongue deviates to the side of the lesion when protruded.