Multiple Cranial Neuropathies

Multiple Cranial Neuropathies 2018-01-30T16:37:19+00:00

Chronic Meningitis

  • Always in the differential diagnosis of multiple cranial neuropathies
  • Infections
    • Tuberculous meningitis: most commonly affects abducens nerve
    • Lyme disease (2nd stage): classically bilateral / unilateral facial nerve palsy; may affect II, V and VIII
    • Neurosyphilis: classically the Argyll-Robertson pupil (pupillary constriction to accommodation but not light). Thought to be due to a lesion in the pre-tectal nucleus, disrupting the consensual light reflex. Accommodation spared as it is a cortical response, with afferent fibres ascending to the visual cortex, and efferent fibres descending to the Edinger-Westphal nucleus and the vergence cells of the reticular formation.
    • Fungal: cryptococcal meningitis, neurocysticercosis
    • Viral: human immunodeficiency virus, Epstein-Barr virus, cytomegalovirus
  • Inflammatory
    • Neurosarcoidosis: typically bilateral facial nerve palsy
      • May also involve II, VIII, IX, and X
    • Behçet’s syndrome: II and VII
    • Wegner’s granulomatosis: II, VI, VII and VIII
      • Typically, patients have renal involvement when cranial nerves are involved
      • Granulomatous masses compressing on globe may cause a restrictive ophthalmoplegia which simulates cranial nerve palsies
    • Polyarteritis nodosa: III, VIII
    • Giant cell arteritis: anterior ischaemic optic neuropathy
      • Ophthalmoplegia from involvement of extra-ocular muscles
      • Other cranial nerves usually spared
  • Neoplastic (more typically painless than the other two above)
    • Lymphomatous meningitis (systemic or primary CNS lymphoma)
    • Carcinomatous meningitis (small cell lung cancer, melanoma, breast cancer)
    • Lymphomatoid granulomatosis
      • EBV-associated systemic angiodestructive lymphoproliferative disease
      • Mimics Wegner’s granulomatosis
      • Classic pulmonary findings – disease extremely unlikely in the absence of pulmonary lesions

Skull Base Neoplasms

  • Nasopharyngeal carcinoma: tumour may erode into skull base by direct extension
    • Combination of VI and XII neuropathy is particularly suggestive of invasion of clivus
    • May also affect CN II and V, may extend in some cases to cavernous sinus
  • Chordoma: rare primary benign bone tumour which often involves clivus
  • Pre-pontine neoplasms (gliomas, dermoid, epidermoid tumours): often III, V and VI
  • Neoplastic involvement of temporal bone: VII and VIII, often associated with chronic otitis media

Orbital Apex Syndrome

  • Syndrome involving damage to the following nerves
    • Optic nerve (II)
    • Oculomotor nerve (III)
    • Trochlear nerve (IV)
    • Ophthalmic division of the trigeminal nerve (V1)
    • Abducens nerve (VI)
  • To test for optic nerve dysfunction:
    • Reduced visual acuity
    • Red desaturation
    • Afferent pupillary defect
    • Relative afferent pupillary defect
    • Offer
      • Colour vision testing
      • Formal perimetry assessment
      • Fundoscopy (optic atrophy)
  • Causes
    • Inflammatory
      • Sarcoidosis
      • Systemic lupus erythematosus
      • Churg-Strauss syndrome
      • Wegner’s granulomatosis
      • Giant cell arteritis
      • Orbital inflammatory pseudotumour
      • Thyroid orbitopathy
    • Infectious
      • Per-orbital cellulitis
      • Herpes zoster
      • Syphilis
      • Bacterial meningitis
    • Neoplastic
      • Nasopharyngeal carcinoma
      • Lymphoma
      • Nerve sheath tumours
        • Neurofibroma
        • Meningioma
        • Schwannoma
    • Traumatic
      • Orbital apex fracture
      • Penetrating injury
  • For orbital apex syndrome, consider MRI of the orbits with contrast and fat suppression sequences
  • Thyroid function tests are critical in evaluation of orbital apex syndrome
  • Consider autoimmune work-up if history suggestive

Superior Orbital Fissure Syndrome

  • Syndrome involving damage to the following nerves
    • Oculomotor nerve (III)
    • Trochlear nerve (IV)
    • Ophthalmic division of the trigeminal nerve (V1)
    • Abducens nerve (VI)
  • In contrast to the orbital apex syndrome, the optic nerve is spared in SOF lesions
  • Anatomically very close together – often SOF and OAS may involve into each other temporally

Cavernous Sinus Syndrome

  • The cavernous sinuses are paired venous channels that lie on either side of the sphenoid bone
  • They extend from the superior orbital fissure to the petrous temporal bone
  • Contents of the cavernous sinuses:
    • Internal carotid artery
    • Sympathetic fibres
    • Oculomotor nerve
    • Trochlear nerve
    • Ophthalmic division of trigeminal (V1)
    • Maxillary division of trigeminal (V2)
    • Abducens nerve
  • Particularly localizing combination: abducens nerve palsy with Horner’s syndrome on the same side
  • Any combination of nerves may be affected
  • Symptoms and signs:
    • Painful ophthalmoplegia
    • Orbital congestion
    • Orbital chemosis
    • Periorbital oedema
    • Proptosis
    • Ipsilateral Horner’s syndrome
    • Ipsilateral loss of sensation in the distribution of ophthalmic and maxillary divisions of trigeminal
  • Causes:
    • Vascular:
      • Carotid artery aneurysm
      • Carotid-cavernous fistulas
        • Classic triad: pulsatile exophthalmos, chemosis, audible bruit over eye
      • Cavernous sinus thrombosis
    • Infections: usually from surrounding sinuses, most common causes: Staphylococcus, pneumococcus
    • Tumours
      • Direct extension: nasopharyngeal carcinoma, craniopharyngioma, pituitary adenoma
      • Metastatic disease
      • Primary tumour (meningioma, lymphoma)
    • Inflammatory (typically granulomatous processes)
      • Sarcoidosis
      • Wegner’s granulomatosis
      • Tolosa-Hunt syndrome (spontaneous resolution in one third, universal response to steroids)

Cerebellopontine Angle

  • Boundaries of the cerebellopontine angle:
    • Lateral aspect of pons
    • Inferior surface of cerebellar hemisphere
    • Superior surface of inner third of the petrous ridge
  • Signs:
    • Ipsilateral sensorineural hearing loss with tinnitus
    • Ipsilateral lower motor neuron facial nerve palsy with no hyperacusis
    • Ipsilateral facial sensory loss (involvement of trigeminal nerve)
    • Rarely, in advanced cases: palsy of CN VI, IX and X
  • Causes:
    • Acoustic neuromas (most common)
    • Meningiomas
    • Cerebellar astrocytoma

Jugular Foramen Syndrome

  • CN IX, X and XI exit through the jugular foramen together with the internal jugular vein
  • Symptoms and signs:
    • Ipsilateral reduced / absent gag reflex
    • Ipsilateral reduced soft palate elevation
    • Deviation of uvula away from the side of the lesion
    • Dysphagia, dysphonia, dysarthria
    • Bovine cough
    • Weakness of shoulder shrugging
    • Weakness in turning head to the opposite side (ride sternocleidomastoid turns head left)
  • Causes:
    • Glomus tumour (jugular bulb – glomus jugulare, vagus – glomus vagale)
    • Schwannomas
    • Meningiomas
    • Retroparotid abscesses
    • Thrombosis of jugular bulb

Multiple Cranial Neuropathies

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