Thread by @rkchoi

1/ Time for another illustrative learning case in the form of a 🧵#tweetorial #medtwitter #neurotwitter #neurocriticalcare #neurology. Case deidentified as always

2/ Young human with no medical history but IVDA (heroin, cocaine, PCP) with multiple prior overdoses and recent discharge from drug rehab 2 days prior who presents to #ED after acting funny

3/They were noted to be lethargic, GCS 13, not able to provide history. CT demonstrates cerebellar edema with #hydrocephalus (💧) and some ⬆️ transtentorial 🧠 herniation

4/ What is your diagnosis?

5/ They become hypoxemic and bradycardic. Panic! What do you do?

6/ Phew, they improved with narcan! Don’t forget to look at the eyes! His pupils were small! 😅The next day, still confused but somehow non focal, GCS14.

7/ MRI (shown) w/ patchy DWI, transependymal edema on FLAIR and leptomeningeal enhancement c/f cerebellitis on T1 post but broad differential

8/ Work up that ensues includes HIV, quantiferon, mycoplasma, CMV negative. + EBV and hepatitis C (chronic). LP 💉not obtained due to risk of herniation. MRI spine with some enhancement of roots. CT C/A/P w/o other malignancy

9/ Repeat MRI 1 week later is unchanged, leading differential syphilis or TB, also sarcoidosis. Given persistent 💧, EVD placed and revealed 655 WBC, 4 RBC, protein 10, glu 82, and the following (-): VDRL/cytology/crypto/ACE/culture

10/ The usual suspects are thrown out: Erdheim-Chester, CLIPPERS, histiocytosis. Search is broadened with autoimmune work up. Paraneoplastic panel negative 🙄

11/ The patient has symptomatic improvement after CSF diversion but would you shunt this person without knowing etiology?

12/ We clamped the EVD and the patient tolerated it. This was removed after 48 hours and repeat imaging without additional hydro. Repeat MRI later showed no 💧 recurrence but persistent enhancement and edema

13/ The patient then went for bx and this revealed necrotizing granulomas with pleomorphic yeast noted concerning for histo vs crypto (but crypto serum and csf negative) 😩

14/ They were started on ampho and flucytosine and had symptomatic improvement but then cultures revealed gram + rods! Meanwhile serum/urine histo negative.

15/ Hmmmm what could be responsive to antifungals but is not histo or crypto? 🤔 ID became concerned for nocardia or actinomyces. However, cryptococcus PCR came back from biopsy PCR= final diagnosis and GPCs were p.acnes related

16/ Crypto is an opportunistic infection usually in the immunocompromised (HIV, transplant, steroids though 1/3 w/o in case series of 157) and p/w meningoencephalitis following inhalation. 

pubmed.ncbi.nlm.nih.gov/11477526/

17/ presentation is variable, months to days, and non specific (HA, fever, lethargy). Dx via encapsulated yeast on India Ink stain, PCR from CSF. CSF usually reveals ⬆️ Wbc/protein and low glucose though these can be normal as in our case. (Image from: researchgate.net/figure/India-i…)

18/ Treatment consists of induction with anti-fungal: ampho B + flucytosine x 2 weeks, though this is often extended 4-6 weeks, depending on comorbidities, seizures, 🧠 involvement

19/ Repeat LP 💉 at 2 weeks to assess for ongoing crypto in CSF and determine if patient is ready for consolidation therapy if immunocompromised or non-compromised but severe (as out pt) but not o/w. Consolidation tx = fluconazole x 8 weeks.

20/ If no mass lesion or 💧, should also do LP to assess ICP. If elevated can do daily LPs vs. lumbar drain/EVD and this series shows 2/3 of those with VP shunt did ok long term

pubmed.ncbi.nlm.nih.gov/15936373/

End/ Prognosis is typically linked to underlying reason for immunocompromised and severity of infection. Other thoughts @caseyalbin @CajalButterfly @JayKinariwala @JimmySuhMD @DrAtulRamesh1 @RamaniBalu1 @TJUHNeuroCrit @PulmCrit @sigman_md @a_charidimou?