Skull Base Syndromes

Diseases, ,

Subtypes:

  • Jugular foramen syndrome= Vernet’s syndrome
  • Collet-Sicard syndrome= Posterior lacerocondylar area syndrome= intercondylar space syndrome
  • Retropharyngeal space syndrome= Villaret’s syndrome
  • Hypoglossal canal syndrome,
  • Foramen magnum syndrome,
  • Carotid canal syndrome

Clinical features:

Features depend on involved nerves:

  • Symptions: Choking, dysphagia, speech changes, auditor canal pain, headache
  • Features by nerve:
    • IX: loss of gag reflex, sensation of the palate, auditor canal pain, glossopharyngeal neuralgia
    • X: hoarse voice, weak cough, difficulty swallowing, nasal regurgitation, loss of gag reflex, auditor canal pain, headache
    • XI: weak/wasted sternocleidomastoid, trapezius
    • XII: tongue atrophy, tongue deviation, difficulty swallowing, laryngeal deviation
    • Sympathetics: Horner’s syndrome
Jugular foramen syndrome (Vernet’s syndrome):

IX, X, XI
If +sympathetics or XII, the lesions is outside of the skull, see other syndromes

Retropharyngeal space syndrome (Villaret’s syndrome):

IX, X, XI, XII, Sympathetics

Collet-Sicard syndrome (Posterior lacerocondylar area syndrome= intercondylar space syndrome):

IX, X, XI, XII

Hypoglossal canal syndrome:

XII

Carotid canal syndrome:

Ipsilateral cerebral infarction, Horner’s syndrome, lower cranial nerve palsies (interrupted pharyngeal branch blood supply)

Foramen magnum syndrome:

Medulla (long tract signs), lower cranial nerves, hydrocephalus
Crossed arm & leg weakness= hemiplegia cruciata
Downbeat nystagmus

Investigations to consider:

HbA1c
CT
MRI T1+gadolinium, T2, MRA
Nasopharyngeal & laryngeal direct visualisation if above is negative
MRV, CTV: juglar vein thrombosis
CTA
Catheter angiography
Testing for Guillain Barre Syndrome
Further search for primary neoplasm or other causes depending on results

Causes of skull base syndromes including jugular foramen syndrome:

Intracranial:

  • Neoplastic:
    • Extension of cerebellopontine angle tumour
    • Meningioma
    • Cholesteotoma
    • Neurofibroma
  • Guillain-Barre syndrome & variants
  • Chronic tuburculosis
  • Syphilis
  • Diabetes mellitus

Skull:

  • Fractured base of the skull
  • Paget’s disease

Extracranial:

  • Neoplastic:
    • Lymphoma
    • Carotid body tumour
    • Glomus jugulare turmour
    • Nasopharyngeal carcinoma
    • Metastatic Squamous cell carcionoma, others
  • Jugular vein thrombosis
  • Carotid dissection