Rami Ibrahim Profile picture
Neurologist | Neurohospitalist @OhioStateNeuro | @cwruneuro alumni | 🇸🇾 ➡️ Ohio⛄️

May 14, 2022, 10 tweets

1/10
It is nice when you see a brain #MRI and instantly jump to a unique diagnosis in your mind.
A #tweetorial about #CLIPPERS Chronic Lymphocytic Inflammation with Pontine Perivascular enhancement Responsive to Steroids. #Neurology #NeurologyRF #NeuroRad #MedEd #NeuroTwitter

2/10
CLIPPERS is a #rare #disease being increasingly recognized. We saw a case here @UHhospitals @cwru @CwruNeuro
(The coronal brain MRIs in this tweetorial are from a published case report other than but similar to our patient- I chose to use here instead given better quality)

3/10
Middle age patient presenting with emotional liability, subacute bilateral lower extremity weakness/numbness/spasms, ataxia and pan-sphinctric dysfunction over 4 months #NeuroRad

4/10
When you look at the MRI you see the unique “PEPPERING” enhancing lesions in the pons, cerebellum and cervical spine, which if you’re familiar with CLIPPERS, it should be the first thing to jump to your mind given these radiological features. Remember “Pons” and “PEPPERING”

5/10
The diagnosis of CLIPPERS is based on proposed clinical, radiologic, and pathologic criteria without a confirmatory test. Thus we always need to exclude alternative diagnoses #lymphoma #vasculitis

6/10
As we know, workup for non-specific enhancing lesions in the brain will include looking for inflammatory, infectious, paraneoplastic etiologies etc

7/10
#CSF with lymphocytic pleocytosis at 82 cells, protein 190 and increased IgG synthesis rate. Otherwise negative profile including: oligoclonal bands, MOG/NMO/GFAP antibodies, Infectious profile, Cytology and flow cytometry
CTA head/neck, Whole body PET scan negative

8/10
Received 5 days course of IV MethylPrednisone for suspected CLIPPERS. Repeat MRI brain with significant improvement of the burden of enhancing lesions. Was discharged home on Oral Prednisone

9/10
Our patient came back a week later with agitation and psychosis. The case took an interesting turn of events as patient was found to have co-existing NMDA #encephalitis and thus completed a course of PLEX treatment and Rituximab with significant improvement

10/10
Take home points #CLIPPERS
-Remember the unique “PEPPERING” lesions on MRI and think about CLIPPERS when you see that
-As the name implies, readily responds to immunosuppression with steroids

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