Make the diagnosis

[DocMike]

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What would your differential be for a 35 year old women who presents with weakness. The next day she has aphasia and weakness throughout her body.

Angiogram shows left and right stenotic occlusions of the vertebral arteries. ESR is not elevated, she's negative for all viruses (HSV, mumps, etc) except that she once had st. luis encephalitis (as shown by IgG and no IgM).

What in the world can cause BILATERAL vertebral artery stenosis in such a young patient? Her lipid panel is perfect, it can't be athersclerosis. I'm thinking it has to be a congenital related problem.

I appreciate any information you guys suggest.

 

[DocMike]

Note, it's not:

Ehler Danlos Syndrome
MoyaMoya Disease
Vasculitis (ESR is negative!)

 

[Brave Heart]

Bilateral osteophytes compression (head and neck x-ray or any imaging?)

 

[DocMike]

Bilateral osteophytes compression (head and neck x-ray or any imaging?)

Angiograms = all negative except for the vertebral arteries

CT shows areas consistent with hemorrhage in the frontal lobe, these hemorrhages were not present on admission. (forgot to mention this)

 

[14022]

Angiograms = all negative except for the vertebral arteries

CT shows areas consistent with hemorrhage in the frontal lobe, these hemorrhages were not present on admission. (forgot to mention this)

Fibromuscular dysplasia can occur in the vertebral arteries. More commonly a cause of renal artery stenosis and renal artery hypertension in young females.

If you search pub med (www.pubmed.com) with "fibromuscular dysplasia + vertebral artery" you will find several case reports.

 

[Idiopathic]

It could certainly be a congenital narrowing, but why do we see symptoms now, especially if there appears to be no acute precipitating event.

What is the patient's BP and other vitals? Are there ANY cardiac concerns? i.e. flutter/fib reports, syncope? Were the carotids imaged also? I doubt that they were, but maybe the CT saw something? Why a frontal bleed in a px with vertebral stenosis?

Honestly, this is most likely a vasculitis of some sort (I think). Run a lupus panel (ESR is often normal, even in active SLE), as she is in the classic population. Might as well check c-reactive protein, ANCA studies and RF, while you are at it.

Just a thought

 

[Dr Alo]

I'd also ask if she's on birth control, a smoker, or any other risk factors for a stroke?

 

[Whisker Barrel Cortex]

It could certainly be a congenital narrowing, but why do we see symptoms now, especially if there appears to be no acute precipitating event.

What is the patient's BP and other vitals? Are there ANY cardiac concerns? i.e. flutter/fib reports, syncope? Were the carotids imaged also? I doubt that they were, but maybe the CT saw something? Why a frontal bleed in a px with vertebral stenosis?

Honestly, this is most likely a vasculitis of some sort (I think). Run a lupus panel (ESR is often normal, even in active SLE), as she is in the classic population. Might as well check c-reactive protein, ANCA studies and RF, while you are at it.

Just a thought

Its rare to do a verterbral angiogram without a carotid angiogram and full cerebral angio.

Some sort of vasculitis or connective tissue disease would be the best bet. Her age and sex make Takayasu's arteritis a distinct possibility, although isolated vertebral involvement would be unusual, I'm sure its still possible. ESR is normal in up to 1/3 of patients with active Takayasu's arteritis, so that doesn't rule it out.

 

[Idiopathic]

Its rare to do a verterbral angiogram without a carotid angiogram and full cerebral angio.

Thanks. I did not know this, although it makes complete sense.

 

[electra]

I vote fibromuscular dysplasia.

Adam's and Victor's Principles of Neurology.

"FD is a segmental, nonatheromatous, noninflammatory arterial disease of unknown etiology. First described in the renal artery by Leadbetter and Burkland in 1938, FD is now known to affect other vessels, including cervicocerebral ones. Of the latter, the internal carotid is involved most frequently, followed by the vertebral and cerebral arteries. It is observed bilaterally in 75% of cases."

Treatment for patients with symptoms can range from excision to dilation via endovascular techniques. Important to note! Some 7-10% of affected individuals may have associated intracranial saccular aneurysms which may be a source of SAH and some 12% develop arterial dissections.

Interesting case. Consider sending her to rehab when stable.

 

[GrandMasterB]

there's no chance she has a coagulopathy? is it definite stenosis and not thrombosis? an acute change like that would suggest something changed right? like if she had a vasculitits and then embolized from elsewhere? how bout a venous thrombosis?

 

[Dr Alo]

Venous thrombosis where? All venous emboli end up in the lungs, unless there is a patent foramen ovale. So unless there is another etiology of the thrombosis, then where would you mean? And how did it get there?

 

[Ramoray]

Venous thrombosis where? All venous emboli end up in the lungs, unless there is a patent foramen ovale. So unless there is another etiology of the thrombosis, then where would you mean? And how did it get there?

i think he meant just an emboli. how about the young woman had taken cocaine, had an atrial fibb and developed a thrombus in the LA that embolized bilaterally.. eh ya never know!

 

[GrandMasterB]

you can get venous thrombosis in the brain as well... it is an emergency with an onset of like a day or so i think- i dont want to make anatomical conjectures without an atlas being pretty far removed from this- so i wont. but posterior cerebral venous circulation sounds about right...

 

[pikachu]

have you tapped her? seems like CSF would be useful in guiding your diagnosis. Although the presentation is consistent with vasculitis, you might also want to rule out MS in this young woman with sudden onset neurologic symptoms. I'm trying to think of a reason that vertebral artery stenosis would cause aphasia as speech is generally affected by lesions in the MCA territory. I would tend to think more of dizziness, diplopia, or blurred vision in a pt with vertebral artery stenosis, or possibly symptoms deriving from the posterior cerebral circulation. You could certainly get weakness from ischemia in the vertebro-basilar territory, but I'm still having trouble accounting for the aphasia. Just a thought.