Interesting cases

[danielmd06]

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Okay...I really, really would love to see some of the knowledgeable individuals on this forum start posting some complex/funny/interesting/high teaching value cases. Neurology is a specialty that is rich in physical examination findings, and historical nuances.

This has been tried before...but tapered off. Yes, I've searched, read the NEJM articles regularly (including the back log), and am working through the Baylor Case series.

But I think it'd be fun to do here. Especially with some of the Ivy Leaguers and senior attendings who float about.

Is anyone interested? I have two bunny cases to start off with...

 

[bluntdissector]

Crises. I meant to reply to this thread earlier. I don't have a case, sorry, but we can't pass up this opportunity to get some 'neurology' on the 'neurology' forum...

Please post your two bunny cases and let's get this thing going!

 

[bluntdissector]

Okay, here's a (partial) case. I didn't see the patient myself, but a friend of mine did, and I heard the presentation on the ward-round. I'll try and be as comprehensive as possible! 🙂

3 y/o girl presents with 2/12 Hx of 'drooping' of the right eye, a new direction dependent squint and the inability to look up with either eye. According to the mom, the drooping has remained fairly stable over the last two months, doesn't change in severity throughout the day, and only ever involved the right eye. With regards to the squint, the right eye can't 'move in' when she looks left. This started at the same time as the 'drooping', as similarly has remained constant. Lastly, she noticed her daughter would tilt her head up excessively when trying to make eye contact, and when asked, the girl confirmed that she couldn't look up 'for a while now'.

No history of LOW, vomiting, headache, seizures, trauma, fever, no sick contacts...(ask if you want to know something specific)

Vitals were stable and within normal limits

General: Child appears well. No pallor. Growth and development normal for age.
Cardiovascular, Respiratory and Abdominal examination normal.
Neuro: Higher functions intact, no meningism, normal head circumference, seems of average intelligence.
CN II visual acuity normal, as were fundi and pupils.
CN II,IV and VI revealed partial ptosis of the right eye, with failure to adduct. Neither eye could elevate. Other extraoccular movements intact. Again, pupils normal.
CN V, VII - XII normal
Motor examination normal.
Sensory normal.
Coordination - some past pointing, obviously more severe with both eyes open and when looking left. Missed a few of the rapid alternating movements, but good for a 3 y/o. Heel shin appeared ataxic, but she is 3 y/o. Gait was essentially normal.

DDx based on this picture? What's for and against some of the options? Seen a similar case? Au fait with the literature? Contribute!

(I'll post some of the blood results, imaging, etc tomorrow, provided they add to the discussion.)

 

[cyneuron]

thumbs up bluntdissector for posting a case...i think we can keep this thread alive and make it a good learning source.

 

[neglect]

Okay, here's a (partial) case. I didn't see the patient myself, but a friend of mine did, and I heard the presentation on the ward-round. I'll try and be as comprehensive as possible! 🙂

3 y/o girl presents with 2/12 Hx of 'drooping' of the right eye, a new direction dependent squint and the inability to look up with either eye. According to the mom, the drooping has remained fairly stable over the last two months, doesn't change in severity throughout the day, and only ever involved the right eye. With regards to the squint, the right eye can't 'move in' when she looks left. This started at the same time as the 'drooping', as similarly has remained constant. Lastly, she noticed her daughter would tilt her head up excessively when trying to make eye contact, and when asked, the girl confirmed that she couldn't look up 'for a while now'.

No history of LOW, vomiting, headache, seizures, trauma, fever, no sick contacts...(ask if you want to know something specific)

Vitals were stable and within normal limits

General: Child appears well. No pallor. Growth and development normal for age.
Cardiovascular, Respiratory and Abdominal examination normal.
Neuro: Higher functions intact, no meningism, normal head circumference, seems of average intelligence.
CN II visual acuity normal, as were fundi and pupils.
CN II,IV and VI revealed partial ptosis of the right eye, with failure to adduct. Neither eye could elevate. Other extraoccular movements intact. Again, pupils normal.
CN V, VII - XII normal
Motor examination normal.
Sensory normal.
Coordination - some past pointing, obviously more severe with both eyes open and when looking left. Missed a few of the rapid alternating movements, but good for a 3 y/o. Heel shin appeared ataxic, but she is 3 y/o. Gait was essentially normal.

DDx based on this picture? What's for and against some of the options? Seen a similar case? Au fait with the literature? Contribute!

(I'll post some of the blood results, imaging, etc tomorrow, provided they add to the discussion.)

Appears to be a description of partial parinaud's. That's probably bad. Mass lesion above midbrain would not be surprising. The happy story would be myasthenia.

Although not definitive, one would want to see if the upgaze paresis is supranuclear or not. I'd still get the workup with head imaging of course and send the ACh-R-Ab's.

 

[danielmd06]

Appears to be a description of partial parinaud's. That's probably bad. Mass lesion above midbrain would not be surprising. The happy story would be myasthenia.

Although not definitive, one would want to see if the upgaze paresis is supranuclear or not. I'd still get the workup with head imaging of course and send the ACh-R-Ab's.

Sorry for not posting more previously. I'm not sure that the thread will keep enough interest up...but here's hoping!

Agree with the above. This sounds like a problem around the midbrain tectum resulting in a partial third palsy and upgaze paralysis. My guess is a Parinaud syndrome from a germinoma or pinealoma.

The test I would request is an MRI brain w/ and w/o contrast.

In adults, myasthenia and diabetes would be in the differential.

 

[bluntdissector]

For sure. When I heard the story, my immediate reaction was 'midbrain - compression, glioma, germinoma.

But there are interesting nuances here. And I might as well post some tables and pictures. If we try and localize the lesion, I think it's fair to say we are looking at the midbrain. The onset of bilateral problems with upgaze, the consistency of symptoms, the lack of deficits other than the IIIs make a more peripheral lesion or lesions highly unlikely. Having said that, the attending was adamant to keep MG on the DDx.

Daniel - you mention DM in adults. Wouldn't it still be rather unusual for it to take out portions of III like that? A small midbrain stroke in an adult would have been my guess, or MG if there was more fluctuation.

Anyway, so here's Brazis on CN III lesions.

And, if we look at nuclear IIIs in more detail:

Pure unilateral nuclear lesions are very rare. Paresis of an isolated muscle innervated by the oculomotor nerve almost always results from lesions in the orbit or from muscle disease. However, nuclear lesions may give rise to isolated weakness of one of the muscles innervated by the oculomotor nerve (e.g., the IR) with the exception of the following muscles: SR, LP superioris, and constrictor of the pupil. These muscles would be affected bilaterally even with small nuclear lesions. (Brazis)

Can you remember why the SR would be bilateral?

Of the four paired subnuclei, the most medial innervates the SR muscle. This is the only portion of the oculomotor nucleus that sends its axons to the opposite eye. Decussating fibers actually traverse the contralateral subnucleus for the SR. Hence, a destructive lesion in one SR subnucleus results in bilateral denervation of the superior recti.(Brazis)

So, we are postulating a small lesion involving the SR subnucleus on the right. And the EW-nucleus is spared, so both pupils should still be reactive. It would also have to involve the MR subnucleus.

But the ptosis is a bitch. Firstly, it's partial and secondly, nuclear thirds don't give you unilateral ptosis, nevermind partial ptosis. As Zak et al also reiterate:
Thus, unilateral ptosis could not be caused by a nuclear lesion.

So we'd have to postulate that our nuclear lesion involving the SR and MR subnuclei on the right is perhaps compressing the part of the fascicle involved with lid elevation. At least, that's all I can come up with.

Brazis, P. W., Masdeu, J. C., & Biller, J. (2007). Localization in clinical neurology. Lippincott Williams & Wilkins.
Zak, R., Slamovits, T., & Burde, R. (1994). Oculomotor brainstem anatomy : nuclei to fascicles. Journal of Neuro-Oncology, 241-248.
(pictures from Zak)

 

[danielmd06]

Daniel - you mention DM in adults. Wouldn't it still be rather unusual for it to take out portions of III like that? A small midbrain stroke in an adult would have been my guess, or MG if there was more fluctuation

Thanks for posting some good old-fashioned neurology! It's much better reading than our typical interview/match threads.

DM would be very atypical for what we're seeing here. But I would always prefer a diagnosis of an atypical presentation of a common problem (in adults) than a rare diagnosis for multiple cranial neuropathies such as neuroborreliosis, neurosarcoid, or neurosyphilis. Myasthenia should give a clue of diurnal variation...but I would definitely keep it on the differential, irregardless. I would also keep migraine filed away as a cause of transient, unilateral 3rd palsy, too. Of course, this case isn't transient.

So yes, it would indeed be rather unusual for DM to be bilateral I would say...but certainly not unusual to get a partial third from DM. It should spare the pupil, too. You mention CVA. A stroke shouldn't really isolate a single subnucleus of CNIII in my (limited) experience...or produce bilateral paralysis of upgaze. You can keep it on the ddx list, but should realize that you're likely dealing with more than one CVA to produce the constellation we're seeing here.

Also, in my (limited) experience, third palsies result in *complete* ptosis of the affected eye, whereas sympathetic-type (Horner-producing) lesions result in a partial ptosis. But we don't have miosis here, either.

What were the results of the MRI?

 

[Pegasus22]

Okay, here's a (partial) case. I didn't see the patient myself, but a friend of mine did, and I heard the presentation on the ward-round. I'll try and be as comprehensive as possible! 🙂

3 y/o girl presents with 2/12 Hx of 'drooping' of the right eye, a new direction dependent squint and the inability to look up with either eye. According to the mom, the drooping has remained fairly stable over the last two months, doesn't change in severity throughout the day, and only ever involved the right eye. With regards to the squint, the right eye can't 'move in' when she looks left. This started at the same time as the 'drooping', as similarly has remained constant. Lastly, she noticed her daughter would tilt her head up excessively when trying to make eye contact, and when asked, the girl confirmed that she couldn't look up 'for a while now'.

No history of LOW, vomiting, headache, seizures, trauma, fever, no sick contacts...(ask if you want to know something specific)

Vitals were stable and within normal limits

General: Child appears well. No pallor. Growth and development normal for age.
Cardiovascular, Respiratory and Abdominal examination normal.
Neuro: Higher functions intact, no meningism, normal head circumference, seems of average intelligence.
CN II visual acuity normal, as were fundi and pupils.
CN II,IV and VI revealed partial ptosis of the right eye, with failure to adduct. Neither eye could elevate. Other extraoccular movements intact. Again, pupils normal.
CN V, VII - XII normal
Motor examination normal.
Sensory normal.
Coordination - some past pointing, obviously more severe with both eyes open and when looking left. Missed a few of the rapid alternating movements, but good for a 3 y/o. Heel shin appeared ataxic, but she is 3 y/o. Gait was essentially normal.

DDx based on this picture? What's for and against some of the options? Seen a similar case? Au fait with the literature? Contribute!

(I'll post some of the blood results, imaging, etc tomorrow, provided they add to the discussion.)

The inability to look up in a kid makes me think of hydrocephalus. It's referred to as the sunset sign and results from third ventrcle compression of midbrain. This of course doesn't explain the ptosis or the adduction problem.

Also (and I'm not sure if this is useful in the least) difficulty in adduction in one eye with an apparently intact the inferior rectus, inferior oblique and the lateral rectus intact in the opposite eye localizes to the MLF (or possibly to the muscle itself). PPRF lesions and above would cause problems with opposite eye abduction, and CN III lesions and below would cause the remainder of the CN III muscles to be compromised in the eye. (this is of course not accounting the possibility of very discrete subnuclear lesions. This is implausible though as there would have to be multiple to account for all the symptoms)

Did you guys end up doing some imaging?

 

[bluntdissector]

Yes, but (annoyingly) there's a 2-3 week waiting period to get the MRI. We grabbed a CT, but the contrasted and uncontrasted scans were 'normal'. Not that the brainstem is anything but a cone shaped mess on a CT. Her picture hasn't changed during the week or so in the ward.
About the MLF lesion - they classically cause INOs, which she didn't have. Hydrocephalus for the upgaze palsy would have featured in the DDx if she hadn't been so 'well', without any signs or symptoms of raised ICP.
If you look at the last picture I posted - isn't it possible that a small lesion taking out the SR and the MR and compromising some of the LP fibers could account for all the problems? Those things are at least close together.
Since the imaging will take a (ridiculously long) while, I'll entertain you with a case where I do have the results.
Briefly, we saw a man at the neuro-clinic with a 2-3 month history of weakness and spasticity in his legs (mostly) and arms. He had recently been diagnosed with HIV (or rather, AIDS, with a CD4 of ~80). It sounded like a slowly progressive spastic quadriparesis, and other features fit - he complained of erectile dysfunction, and even had a period of urinary incontinence in the office, globally increased tone and reflexes, higher functions and CNs seemingly intact, etc. By this stage he was confined to a wheelchair. Whether this myelopathy was due to HTLV or HIV itself or B12 deficiency or any number of other causes was the presumed question. We admitted him and (again) waited for the MRI-spine to be done, and sent away tests for the other 'queries'.
However, whilst in the ward, we noticed that his higher functions weren't that great. Over the course of a few days, this became more and more clear, and eventually he was delerious. So the MRI-spine was changed to a MRI-brain, and the delerium bumped him up the list...

 

[bluntdissector]

AAAAND this is what we found:

 

[neglect]

How terrible. Appears to be multiple mets, but the differential includes inflammation and infections. The lesion on top of the midbrain best seen on sagital imaging was responsible for the presentation. Unless there's a primary on the CT of the rest of the body, it'll need a biopsy.

 

[mmmcdowe]

AAAAND this is what we found:

Enjoyed reading this case. I have no particular input other than I wanted to say F***.

 

[bluntdissector]

This is actually a case of REALLY bad toxoplasmosis. The weird part was the fact that his clinical picture didn't even whisper "I have have tons of focal lesions in my brain". Crazy.

When I saw the scan, I was like

 

[NeurologyICU]

Three differentials that came to my mind looking at the images were:

1) Brain mets
2) Toxoplasmosis
3) Lymphoma

 

[Mattchiavelli]

The first thing I thought of was "Mad Tox"... but then I need to work on expanding my differential 🙂

I thought about the possibility of a well-compensated AIDS dementia complex should the scan be clear but reading up it seems by the time the motor involvement would have reached wheelchair stage the cognitive aspect would have been to advanced for even the trickiest patient to compensate for. Then I looked at that scan and oy..

 

[tartrate]

Reading this thread gets me really excited for some reason...

 

[clement]

What are possible mechanisms of a cerebellar bleed after running into a metal light pole with your head really hard face-foward?

 

[Pegasus22]

About the MLF lesion - they classically cause INOs, which she didn't have. Hydrocephalus for the upgaze palsy would have featured in the DDx if she hadn't been so 'well', without any signs or symptoms of raised ICP.
If you look at the last picture I posted - isn't it possible that a small lesion taking out the SR and the MR and compromising some of the LP fibers could account for all the problems? Those things are at least close together.

Actually I thought your theory was really very creative. It's just that in my experience things never work out that neatly. It would be really pretty cool if it turns out you're right though.

About the lateral eye movements - from the way you've described it, it seems as if she has something not unlike an INO. She can't adduct her right eye while looking left.

Did you guys look at her fundi?
Also, (and this is lower on the differential than the stuff suggested so far as the findings are assymmetric) with bilateral ptosis and difficulties in eye movements NMJ stuff is on the table. Did you do any testing for that sort of thing? Antibodies, Chest CT, nerve conduction studies etc.?

 

[Pegasus22]

Actually I thought your theory was really very creative. It's just that in my experience things never work out that neatly. It would be really pretty cool if it turns out you're right though.

About the lateral eye movements - from the way you've described it, it seems as if she has something not unlike an INO. She can't adduct her right eye while looking left.

Did you guys look at her fundi?
Also, (and this is lower on the differential than the stuff suggested so far as the findings are assymmetric) with bilateral ptosis and difficulties in eye movements NMJ stuff is on the table. Did you do any testing for that sort of thing? Antibodies, Chest CT, nerve conduction studies etc.?

Scratch that last bit. I forgot the ptosis was unilateral.

 

[clement]

What are possible mechanisms of a cerebellar bleed after running into a metal light pole with your head really hard face-foward?

anyone anyone? best i could come up with was contrecoup or cervical hyperextension but that didn't fly as there was no cervical injury.

 

[Head]

anyone anyone? best i could come up with was contrecoup or cervical hyperextension but that didn't fly as there was no cervical injury.

How about just saying it was due to trauma? I don't know if you can get contrecoup injuries to the cerebellum. I've never heard of cervical hyperextension causing a bleed unless there was a dissected vert and resulting infarct that then had hemorrhagic conversion.

Other possibilities (assuming this is an intraparenchymal bleed) - person was on meth or cocaine leading to a spontaneous hypertensive hemorrhage (which caused them to run into the light pole in the first place), underlying AVM or cavernous malformation.

 

[FictionalGirl]

Having spent some time in the emergency room, and after consulting with my roommate who is also an SDN regular and applying in emergency medicine we have agreed on a mechanism: They jiggled their brain.

Seriously.

You just whack yourself so hard that your brain bounces all around. Physics makes it possible that what stops the cerebellum from flying out from above your neck is your skull. But that's not really a great plan either, is it? Hence the bleeding 🙂

Not the most sophisticated diagnosis, and it sure won't win me any points on the wards (I could stick a tuning fork on it?) but JiggledBrainSyndrome explains a lot of things, not the least of which are some of my classmate's personalities......