History and Examination
•32yo caucasian F who presents with sudden onset of numbness from her mid chest down on both sides. Also notes blurred vision. She started wearing prism glasses about 1 year ago for diplopia.
•PMH – neg
•PsurgHx – C section x 3
•Meds – Flexeril
•Vital signs normal
•Neurologically intact except for
•Decreased vision in left eye
•3+ deep tendon reflexes throughout
•Decreased light touch and pin prick below T4 with left leg affected more than right
•4/5 bilateral lower extremity strength
- She had evaluation by ophthalmology and was found to have bilateral papilledema
- Further imaging was obtained
1. What test result would you expect in neuromyelitis optica?
2. What is the likely diagnosis?
3 .What would be your next step?
4. Which of the following describes you?
5. I practice in one of the following locations.
- needing surgical resection for L occipital mass up front - at least that is my impression from how authors presented case. This presentation c/w MS/demyelinating (sudden LE parathesias) and a year of "visual complaints" that I suspect are optic nerve related ("decreased vision L eye") and not tumor related (i.e., field cut). So, intracranial mass w/ imaging c/w meningioma seems incidental. In absence of findings c/w elevated ICP (e.g., papilledema, h/a) plus imaging findings c/w meningioma w/o significant edema - I would consider LP a relative, not absolute, contraindication. So I wouldn't fault a neurosurgeon w/ starting w/ demyelinating w/u - LP. No doubt tumor needs to be resected. Especially, knowing it's a grade II meningoma whose biology has a high rate of recurrence/progression despite GTR surgical rsx, Further, XRT probably needed at dural margins at some point b/c not able to achieve Simpson Grade 0/1 resection in this location. No right answer on when XRT - upfront or at progression - but patient will need long-term serial imaging for resection. Nice case.
- Its also called devic disease.plz let me know about correct answer.as i am resident
- Demyelination with lesion?
- beta-interferon side effect 'flu like symptom'. Plasma exchange will need anticoagulation.
where is the patient now? assuming the patient is in a room adjacent to an operation theatre and wheeling the patient to theatre will take 15 min and anaesthetising the patient will take 15 min and positioning will take 15 min, I have 45 minutes to speak to a neurologist ( and 15 min to stop the beginning of anaesthesia for the craniotomy, if the latter advises so )
- In question 2: I think it is an extra-axial tumor, most likely meningioma, but it can be another one.
•Neuromyelitis optica is associated with antibodies against AQP-4
•The patient’s imaging and presentation is most consistent with a large meningioma causing increased intracranial pressure (ICP) and papilledema as well as an underlying demyelinating disease process with intramedullary enhancing lesions and an intracranial periventricular lesion.
•The patient has increased ICP with papilledema from the large tumor. This was resected to relieve mass effect. Pathology was consistent with a grade II meningioma. She is following up with neurology for management of her demyelinating disease as an outpatient. Lumbar puncture would be contraindicated in the presence of a large intracranial mass which could result in herniation.
•Bennett JL. Finding NMO: The evolving diagnostic criteria of neuromyelitis optica. J neuroophthalmol, 2016.
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