[CIS PIDD] [cis-pidd] ADA SCID with partial reconstitution and chronic leptomeningitis/encephalitis

[Nacho Gonzalez]

Hi,
I would only add neurobrucellosis to the differential diagnosis.
Regards,

Luis Ignacio Gonzalez-Granado
Pediatric Infectious Diseases. Immunodeficiencies
Hospital 12 octubre. Madrid. Spain


2013/8/8 Donald Cuong Vinh, Dr <donald.vinh at mcgill.ca>


>  Hi Christine,

>

> Toxoplasma may be a consideration, depending on risk factors/epidemiology.

> Cound possibly also account for the uveitis

>

> A CSF Toxo PCR could address this

>

>

>

> Best wishes,

>

> Don

>

>

>   Donald C. Vinh, MD

> Assistant Professor

> Division of Infectious Diseases,

> Division of Allergy & Clinical Immunology

> Dept of Medicine; Dept of Medical Microbiology; Dept of Human Genetics

> McGill University Health Centre - Montreal General Hospital

> 1650 Cedar Ave, Rm A5-156

> Montreal, Quebec,  Canada H3G 1A4

> Ph: 514-934-1934 x42419 (office); x42811 (admin assist)

> Fax: 514-934-8423

> e-mail: donald.vinh at mcgill.ca

>

>

>

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>    ------------------------------

> *From:* Christine M. Seroogy, MD [cmseroogy at pediatrics.wisc.edu]

> *Sent:* Thursday, August 08, 2013 8:42 AM

> *To:* CIS-PIDD

> *Subject:* [cis-pidd] ADA SCID with partial reconstitution and chronic

> leptomeningitis/encephalitis

>

>

>    *Dear Colleagues,*

>

> *

> *

>

> We are caring for a 14 y/o with ADA SCID s/p non-conditioned

> haploidentical BMT x 2 with a 4 month history of intermittent fevers,

> headaches, weight loss, myalgias.  He has 100% donor T cells with adequate

> function and low T cell numbers, on IG therapy, and off of PEG-ADA for

> years but empirically restarted 2 weeks ago.  His most recent brain/spine

> MRI demonstrated enhancement of meninges and some parenchymal enhancement

> (worsening from previous studies).  His CSF has increased lymphs/monos with

> high protein and low glucose.He had a brain bx 10 days ago:  with no

> viral inclusions, fungi or bacteria visible (EM and special stains

> including AFB). There is granulomatous inflammation with no discrete

> granulomas with numerous macrophages and T cells (CD4:CD8 ratio 3:1), no

> B cells. One possibility is a neurosarcoid-like picture. Imaging elsewhere

> does not demonstrate obvious organ involvement outside the CNS.

>

>

>  His notable history in the past year is one episode of ITP responsive to

> 4 doses of rituxan and non-infectious uveitis responsive to topical

> steroids.

>

>

>  He has had an extensive infectious disease work-up and my question to

> the group is if there is an infectious agent we may be missing? Have any of

> you seen such a case?

> Here is the infectious work up to date:

>

>

>  *Studies:*

>

> Bacterial--

>

> 4/29/13 Culture, CSF negative

>

> 4/29/13 Lyme PCR (CSF) negative

>

> 4/29/13 Bartonella PCR (CSF) negative

>

> 4/29/13 AFB smear/culture (CSF) negative

>

> 7/11/13 blood, peripheral: negative

>

> 7/12/13 blood, peripheral: negative

>

> 7/14/13 CSF culture, negative

>

> 7/14/13 AFB smear & culture, CSF Negative

>

> 7/14/13 16s ribosomal PCR, CSF negative

>

> 7/26/13 CSF culture negative

>

> Viral--

>

> 4/8/13 EBV PCR negative

>

> 4/8/13 CMV PCR negative

>

> 4/8/13 Flu A/B, RSV PCR negative

>

> 4/9/13 Enterovirus PCR negative

>

> 4/23/13 Respiratory viral panel (WI state lab): negative

>

> 4/29/13 West Nile PCR (CSF) negative

>

> 4/29/13 adenovirus PCR (CSF) negative

>

> 4/29/13 CMV PCR (CSF) negative

>

> 4/29/13 Arbovirus Ig panel (CSF) Negative

>

> 4/29/13 HHV-6 PCR (CSF) Negative

>

> 4/29/13 HHV-8 PCR (CSF) negative

>

> 4/29/13 HSV PCR (CSF) negative

>

> 4/29/13 Enteroviral PCR (CSF) negative

>

> 4/29/13 VZV PCR (CSF) negative

>

> 4/29/13 EBV PCR (CSF) negative

>

> 5/1/13 Enterovirus RNA PCR, stool negative

>

> 7/8/13 Resp viral panel (Viracor): positive for rhinovirus

>

> 7/8/13 Enterovirus PCR negative

>

> 7/11/13 BK virus qPCR: negative

>

> 7/12/13 JC virus PCR (blood): *positive (very low levels: initially

> postive at 42 cycles, repeat positive at 36 cycles)*

>

> 7/12/13 adenovirus PCR: negative

>

> 7/12/13 enterovirus PCR: negative

>

> 7/14/13 CMV PCR (CSF) negative

>

> 7/14/13 JC virus PCR (CSF): *negative*

>

> 7/14/13 BK virus PCR (CSF) negative

>

> 7/14/13 Enterovirus PCR (CSF) negative

>

> 7/14/13 Adenovirus PCR (CSF) negative

>

> 7/14/13 EBV PCR (CSF) negative

>

> 7/14/13 LCM IgG/M (CSF) pending

>

> 7/14/13 Herpes simplex PCR (CSF) negative

>

> 7/16/13 respiratory viral panel (Viracor) positive for rhinovirus

>

> 7/16/13 Herpes simplex PCR (blood) negative

>

> 7/18/13 blood JC virus qPCR *<500/mL*

>

> 7/23/13 Blood HTLV PCR Negative

>

> 7/23/13 Blood HIV RNA, qPCR Not detected

>

> 7/26/13 VZV PCR from brain biopsy negative

>

> 7/26/13 EBV PCR from brain biopsy negative

>

> 7/26/13 CMV PCR from brain biopsy negative

>

> 7/26/13 adenovirus PCR from brain biopsy negative

>

> 7/26/13 adenovirus PCR from CSF negative

>

> 7/26/13 enterovirus PCR from brain biopsy negative

>

> 7/26/13 coxsackie/echovirus PCR from rectal swab negative

>

> 7/26/13 coxsackie/echovirus PCR, throat swab negative

>

> 7/26/13 EBV RTPCR, CSF negative

>

> 7/26/13 VZV RTPCR, CSF negative

>

> 7/26/13 CMV RTPCR, CSF negative

>

> 7/26/13 enterovirus RTPCR, CSF negative

>

> 7/26/13 enterovirus culture from CSF, brain, and dura negative at 10 days

>

> 8/2/13 respiratory virus panel (in-house) negative

>

> Fungal--

>

> 4/29/13 Fungal culture, CSF negative

>

> 4/29/13 Histoplasma Ag (CSF) negative

>

> 4/29/13 Blastomyces Ag (CSF) negative

>

> 4/29/13 Cryptococcal AG, CSF negative

>

> 7/14/13 Blastomyces antigen, urine negative

>

> 7/14/13 Histoplasma antigen, urine negative

>

> 7/14/13 CSF fungal culture NGTD

>

> 7/14/13 Cryptococcus antigen, CSF negative

>

> 7/26/13 Fungal culture, CSF NGTD

>

> Other--

>

> 4/29/13 Mycoplasma PCR CSF negative

>

> 7/14/13 Mycoplasma PCR CSF negative

>

> 7/14/13 Toxoplasma PCR CSF negative

>

>

>  Thank you

>

>  Chris

>

>

> Christine M. Seroogy MD,  FAAAAI

> Associate Professor

> University of Wisconsin

> Department of Pediatrics

> Division of Allergy, Immunology & Rheumatology

> 1111 Highland Avenue

> 4139 WIMR

> Madison, WI  53705-2275

> phone: 608-263-2652

> fax: 608-265-9721

>

>

>

>

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