Normal MRI but....

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climbingdocs

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Here is a case:

55 y/o hispanic female. Presented with dysarthria, right side weakness and sensory deficit.
On physical exam:
EOMI, PERLA, sensory deficit on right side of the face, musles of mastication weak, muscles of facial expression weak all of that on the worse on the right, but can wrinkle forhead bilateraly. Right side of the body weak and all sensory modalities decreased on Right, but Babinski negative. She is having involuntary right arm movement. She is mentally intact, I spent about an hour with her trying to figure out her symptoms, she is trying to use sign language, write little with her left hand. She "said" that she feels like her right side of her face is pulling to ther right and her R eye feels like is getting pulled out of her orbit.
Can't really obtain PMHx but we know she had seizure two days ago.

So, MRI showed nothing. EEG is pending. She had elevated WBCs but it's decreased over 2 days. Any ideas what to do next?
 
A Todd's paralysis might fit this scenario. You said she had a seizure and she is still within the typical 48 hour window. If it clears up in the next day or so I would put that pretty high up on my list. WBC elevation is often seen in patients whom are post-ictal. The decreased sensory modalities is a little odd, but often times people describe a decreased or unusual sensation when they have stimulation of a paralyzed body part. Otherwise you have described an exam of someone with a lesion somewhere from the rostral medulla up (vertical localization due to the presence of a cranial nerve V lesion and the non-crossed facial/body findings). The sensation of pulling in the orbit may represent a subtle CN III, IV, or VI lesion, so you may want to pay close attention to the EOM. Furthermore, visual field testing could help localize if the lesion is more rostral.
 
so this is day 4, and no improvement. Its really hard to do visual exam becuase she can not speak...
 
You wrote that she was making efforts to communicate with her left side. Can't you just have her raise her hand when she sees the stimulus? If this doesn't work, why not test her to visual threat. It is more crude, but it should allow for detection of a big quadrant defect.
 
In such cases, look for 'non-physiologic/non-neurologic' symptoms, in other words - malingering for some secondary gain or any stressors in their life.
On clinical exam look for-
1. if there is clear midline splitting on the forehead and face (there is overlap in the midline in all 3 divisions of the V nerve). Also you may check how brisk the corneal reflex is.
2. First check vibration sense in the trunk, upper and lower extremities. This will make the patient feel you are looking for loss of vibrarory sensation. Then check for vibration sense on both sides of the head. If she has intact hearing and doesn't feel vibration on one side, then she is malingering (this is weber test).
3. Regarding weakness on the right side, place your hand under the 'left' foot and then ask her to raise the right foot. You should be able to feel pressure under your hand if she is making a genuine effort to raise the right leg (due to reciprocal innervation of agonist/antagonist muscles). If she is malingering then you won't feel the contraction of the antagonists in the left leg.
Lastly, from what I have seen, patients with migraine with stroke like symptoms may have normal MRI as well.
Also, it is important to note - in brain stem infarcts- MRI including DWI can be false negative in 5% patients within 24 hours of symptom onset.
These are the references-
[FONT=verdana,arial,helvetica,sans-serif][SIZE=-1][FONT=verdana,arial,helvetica,sans-serif][SIZE=-1] Rakesh Khatri, James Leach, and Matthew L. Flaherty. False-negative diffusion-weighted imaging with lateral medullary infarction
Neurology, Nov 2006; 67: E19.

[/SIZE].[/SIZE].Oppenheim C, Stanescu R, Dormont D, et al. False-negative diffusion-weighted MR findings in acute ischemic stroke. AJNR Am J Neuroradiol 2000;21:1434–1440
 
I checked with vibration, when I placed the tunnig fork to ther left forhead she squinted and communicated that it was lound. She also have hyperacusia in her left year and if she was faking she probably would say that she can't hear it on that side. Also today she cannot lift her left leg as well. I watched her quads were flexing while she was trying to move her left leg. Also when she tries to do any movement with her legs her R arm would move unvoluntarly. Today she wasn't able to open her mouth wide enough to stick out her tounge. Her EEG showed some abnormality in left temporal lobe. LP was negative for bacterial and viral infection (low protein, high glucose). Still waiting for HIV results, and did MRA, and VRLD and ANA.

I really have no clue what is happening. I read neurologist's note, who wrote that patient has aphasia, and that she is not able to follow simple commands. And obviously patient has dysarthria not aphasia, she can't operate her mouth, so even if she did have aphasia (which I doubt, because she can write) you can't really diagnose aphasia. And she follows all the commands as well as she can. I called neurologist, and told him that my PE was different from his, I tried to be nice about it (since I am a student and he is a doc I didn't want to step on his toes) , and I just hope he will re examine the patient. I don't trust him now, since he obviously didn't take the time to examine patient properly.

I am going to suggest to re-do MRI, but not sure how soon.... any ideas? Also are there any infectous diseases that could cause seizures and didn't show on LP?
 
1. where is the lesion? Initially she presented with brain stem syndrome. likely rostral medulla on the right. But now other areas in the brain stem are being involved without irrefutable evidence of involvement of other areas.

2. What caused it? Temporal features are important here, but she can't give a clear history. If she had sudden onset (that would strongly suggests stroke), but we don't know the onset. We do know about this progression over 4 days suggest a progressive local process: I wonder about oedema after stroke or maybe it's not stroke at all, Rhombencephalitis (encephalitis of the brainstem) is a possibility. Maybe another LP with CSF culture for Listeria is warranted. Or maybe it's a noninfectious inflammatory process i.e. acute demyelinating encephalomyelitis. the onset is atypical but if she is progressing to developing a locked-in-syndrome then we have to consider osmotic demyelination syndrome i.e. central pontine myelinolysis, What's the Na been like?

Any White cells in the first LP? maybe we should repeat imaging in light of new findings.

Also with any progressive brainstem pathology, you'd have to monitor for & worry about worsening of respiratory drive & other vital structures which would necessitate frequent monitoring, ICU transfer etc

Last question: I'm curious, has she had any hiccups yet?
 
So I got a call from patient daughter who didn't really want to talk to me (since I am a student) but she did say that patient had car accident in october after which, patient had seizures. Today she was taken for spine MRI. Not sure if that will help.
 
Looks like we might have a multifocal process on our hands, first involving either the left brainstem or the left cerebral hemisphere (with right hemibody signs and symptoms) and now the opposite side as well. Spinal cord unlikely with prominent facial weakness, unless we have multiple levels of the neuroaxis involved. I get the impression it's either acute or subacute in onset, but is stepwise, making stroke unlikely, especially in the face of a normal brain MRI. What kind of involuntary movements is she having in her right arm? Chorea, tremor, athetosis? All of these suggest basal gangliar involvement, although you could have a rubral tremor from a cerebellar/midbrain lesion (but that would be most obvious with movement of the limb). Could they be simple partial seizures (given the left temporal abnormalilty on EEG)? What exactly did the EEG report say?

Was the brain MRI actually normal, or did it say something about non-specific, age-related white matter changes or something of that nature? What happened with the ANA?

Does she have bowel/bladder symptoms? Are her reflexes symmetric and normal? Any sensory deficits on the left side now? Do you perhaps have a sensory level?

At this point, I would start thinking about a multifocal etiology like vasculitis or paraneoplastic syndrome. Did you send off an esr (which may be normal in CNS angiitis anyway) or a paraneoplastic panel (both blood and CSF)? Is she a smoker? Does the breast exam show anything? Did you get an HIV result back (that would open up a whole new differential)? How's her renal funtion and EKG? Any systemic findings on exam? Any rashes recently?

I agree, repeat the MRI brain with contrast, maybe another EEG, and she definitely needs another LP, sometimes abnormalities do not show up on the first one. Did you send off a CSF ACE, HSV-PCR, West Nile or Lyme titers? The case is atypical for any of these, but worth checking. Would also check CSF for malignant cells.

Just curious, what is the neurologist's differential/leading diagnosis in his notes? What does he think is going on?
 
What is her formal past medical and surgical history? Any psychiatric history or history of headaches? Any recent post-partum history? What medications does she take at home? Does she have any risk factors for stroke? I know that we didn't originally have this information...but you said you subsequently spoke to her daughter.

What are the nature of her seizures/What are they like? How long do they last? Is she postictal afterwards? Does she have a diagnosis of a particular type of seizure? What anti-epileptic medications is she taking? How often does she typically have them? Is there a proposed etiology for her seizures?

What is her full physical exam? Does she truly have dysarthria, or does she have expressive aphasia? What does her cranial nerve testing reveal? Any nystagmus or ocular pathology?

What are her reflexes on exam? What does her cerebellar testing reveal? What is her gait like? What is her sensory exam like...is there a sensory level?

Does she have any abormal basic labs? Have repeat CSF analysis or neuroimaging shown anything?
 
What is her formal past medical and surgical history? Any psychiatric history or history of headaches? Any recent post-partum history? What medications does she take at home? Does she have any risk factors for stroke? I know that we didn't originally have this information...but you said you subsequently spoke to her daughter.

What are the nature of her seizures/What are they like? How long do they last? Is she postictal afterwards? Does she have a diagnosis of a particular type of seizure? What anti-epileptic medications is she taking? How often does she typically have them? Is there a proposed etiology for her seizures?

What is her full physical exam? Does she truly have dysarthria, or does she have expressive aphasia? What does her cranial nerve testing reveal? Any nystagmus or ocular pathology?

What are her reflexes on exam? What does her cerebellar testing reveal? What is her gait like? What is her sensory exam like...is there a sensory level?

Does she have any abormal basic labs? Have repeat CSF analysis or neuroimaging shown anything?

So we found that she had 6 similar episodes last year. She is 55 so no postpartum. She did have empty sella on MRI, but not sure if that matters. I called neurologist again, suggested SSEPs, he didn't think it would help, but we are going to order them anyway.
She doesn't have aphasia as far as we can tell, she still can't open her mouth but she does eat when we are not there. So we are leaning towards psychogenic cause. So SSEPs will show us if there is something organic there... may be.... ANA still not back. And she had another seizure last night, so we are going to go 24 hour EEG too. Seems like people are slowly giving up on her 🙁
 
She doesn't have aphasia as far as we can tell, she still can't open her mouth but she does eat when we are not there.

I haven't gotten a chance to read the whole thing (and I'm just a student too), but this jumped out at me. Do you have a speech/lang pathologist there to evaluate patients for a proper diet? If she is truly unable to open her mouth to eat on their testing, then I can't imagine the SLP would feel comfortable prescribing a regular diet. Telling the pt she has to get an NG tube would be one way to sort out a psychogenic cause!

(disclaimer: not necessarily what I would recommend, but honestly, say she aspirated on a regular diet...and you have recorded that she was unable even to open her mouth...for that reason I don't know that the NG tube approach is completely unjustified, although maybe mean).
 
Telling the pt she has to get an NG tube would be one way to sort out a psychogenic cause!

From my humble experience patients with psychogenic causes will undergo testing the same way that other patients would e.g. LP, EMG/NCS, ICU stay, intubation etc.
 
she still can't open her mouth but she does eat when we are not there.

This may be a really stupid question, bot how would she eat if she can't open her mouth at all?😕
 
What was the result of the EEG? R facial weakness described is UMN, which puts it above the facial nucleus. Just because you can't see it on MRI doesn't mean the "lesion" does not exist.
 
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