ZSFG neuro report: multiple cranial neuropathies – spotlight on the cavernous sinus

Thanks to Arturo Montano for presenting a fascinating case of a patient with monocular vision loss who was found to have GPA in her cavernous sinus.
Approach to multiple cranial neuropathies
  • Start by documenting the distribution of all neuropathies and any other neurologic deficits. Is there a place where all of those nerves come together, such as the brain stem, skull foramen or cavernous sinus? Or is the distribution truly random?
    • random distribution – think about systemic disease like viral infections, MS, guillain-barre and its friends, leptomeningeal spread of malignancy or diabetes
    • anatomic distribution – think masses or strokes
  • Always have a high index of suspicion for malignancy or mass – either a solid tumor or leptomeningeal spread of metastatic disease.
    • in a case series of ~1K people with multiple cranial neuropathies, tumor or leptomeningeal spread caused  1/3 of cases
Leptomeningeal carcinomatosis
  • when malignancies spread into the subarachnoid space. this should be distinguished from dural metastases, which are much more common.
  • most common associated malignancies – breast, lung, GI and melanoma
  • very difficult to diagnose! Often requires serial, large volume LPs. MRI with gad can also sometimes aid in diagnosis
  • prognosis of 2 months or less.
Eye pain history
  • Primary goal is to differentiate lesions within the globe from skin, soft tissue and bone lesions from neuromuscular lesions
  • pain with extraocular movements make you concerned about either optic neuritis or a inflammation of the muscular cone that encases the eye
  • optic classically presents with red desaturation – red objects look like they’ve been washed out
Cavernous sinus masses
A quick reminder of what runs through the cavernous sinus
cavernous_sinus_anatomy
  • Cranial nerves III, IV, V1 & V2 branches and VI run through it
Ddx of cavernous sinous masses
  • infectious
    • fungal
    • TB
    • septic thrombosis
  • autoimmune/rheum
    • Telosa-Hunt syndrome
    • sarcoid
    • IgG4 dz
    • GPA
  • malignant
    • lymphoma
  • Vascular
    • thrombus
Citations
JR Keane Multiple Cranial Nerve Palsies: analysis of 979 cases. Arch Neurol. 2005;62(11):1714-1717.
BL Lampson, SW Gray, ES Cibas, BD Levy, J Loscalzo.Tip of the Tongue N Engl J Med 2016; 375:880-886
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