Travis Smith, D.O. FAAEM Profile picture
Medical Advisor @ OASH Former Senior Advisor HHS IOS/HRSA. EM Boarded @UFJAXTrauma. #Noles. Private Physician/Aging/Longevity/God/Family/Golf #MAHA

Sep 22, 2020, 24 tweets

We didn't have a #Neuro case today on @CPSolvers #VMR with @AaronLBerkowitz but don't worry! Let's recap a prior episode for some #SpacedLearning @DxRxEdu @rabihmgeha

With @ddeng_22 @KannuBansalMD

clinicalproblemsolving.com/morning-report…

A 26 yo M w/ a PMH of nodular sclerosing hodgkins lymphoma p/w left-sided facial droop, L arm weakness, and dysarthria.

He presented 12 hours after onset and his symptoms had resolved.

3 months prior he had an autologous stem cell transplant and is currently on Brentuximab.

First of remembering that E=MC2

Time of onset and localization

Sudden Onset: Think stroke, seizure, Todd's paralysis, toxic metabolic, migraine with aura

Localization: Brainstem ipsilateral face and contralateral body

Dysarthria = motor problem

Aphasia = language- think cortex

Facial Weakness: UMN vs LMN according to forehead sparing. LMN it is paradoxically worse and affects forehead and lower face. UMN spares upper affecting only lower face.

What is nodular sclerosing Hodgkin lymphoma?

Hodgkin lymphoma (HL) is a rare monoclonal lymphoid neoplasm with 2 distinct categories:

1. classical Hodgkin lymphoma
2. nodular lymphocyte-predominant Hodgkin lymphoma (NLP-HL)

HL represents 11% of all lymphomas seen in the US

Classical Hodgkin lymphoma accounts for 95% of all HL, and it is further subdivided into four subgroups:

1. Nodular sclerosis (NSHL)
2. Lymphocyte-rich (LRHL)
3. Mixed cellularity (MCHL)
4. lymphocyte-depleted (LDHL)

Nodular sclerosis will show a partially nodular growth pattern, with fibrous bands and inflammatory background.

RS cells are rare and lacunar cells are more common.

What is Brentuximab?

Its trade name Adcetris) is an anti-CD30 antibody-drug conjugate medication used to tx relapsed or refractory Hodgkin lymphoma

relapsed/refractory Hodgkin lymphoma, anaplastic large cell lymphoma, or primary cutaneous CD30-+lymphoproliferative disorders

Peripheral sensory neuropathy is a significant adverse event as well as neutropenia and multifocal leukoencephalopathy

The MC adverse events: fatigue (36%), pyrexia (33%), diarrhea (22%), nausea (22%), neutropenia (22%), and peripheral neuropathy (22%). ncbi.nlm.nih.gov/pmc/articles/P…

Back to the case:

His vitals were normal...

His Neuro was normal...

His labs and serological fungal, viral, and bacterial tests were normal

Even the LP was normal: No WBC, No protein, No JC, No BK, No toxo or crytpo

Okay fine. Give us some imaging.

MRI Brain: Showed multifocal lesions with hyperintensity and diffuse restriction in the periventricular white matter, basal ganglia, subcortical areas, also with enhancement.

As corny as it sounds, Tissue is the issue.

No PML- but you should suspect w/immunosuppresion (JC virus reactivation) and subacute deficits

Brain biopsy showed negative fungal and bacterial cultures and the pathology reported diffuse large B cell lymphoma.

Remember that the multifocality of small lesions might have overt symptoms.

When bilateral brain lesions are present, think embolic from cardiogenic sources.

When there is contrast enhancement, the blood-brain barrier is open and usually means infection or inflammation.

What is Diffuse large B cell lymphoma?

Well out of more than 30 subtypes of NHL, diffuse large B-cell lymphoma (DLBCL) is one of them.

Genetic alterations in the BCL6 gene can be seen in 20% to 40% of the patients.

Just as chronic immunodeficiency of T cells and B cell stimulation can possible causes as these infectious agents can directly manipulate the DNA, the use of an immunosuppressive medication is a risk factor for the development of B-cell lymphomas.

Like in our patient!

DLBCL accounts for about 25% of all NHL cases worldwide.

DLBCL is the most common NHL, followed by Follicular Lymphoma.

Seen more frequently in whites, followed by African Americans and Asians w/male preponderance and a median age of 64 years.

Though DLBCL is aggressive, w/ appropriate chemotherapy, survival can be long, but with a limited cure rate.

Patients w/ GCB DLBCL respond well to 6 cycles of rituximab along w/ cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) regimen given every 21 days.

The more aggressive approach is rituximab, doxorubicin, cyclophosphamide, vindesine, bleomycin, and prednisone (R-ACVBP), followed by consolidation with methotrexate and leucovorin.

Would like input from my pals @haematognomist and @Anand_88_Patel on this though

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