[CIS PIDD] [cis-pidd] CVID with dementia

[dmvascon at usp.br]

Dear Joe, 

JC and BK virus were tested, as well as protein 14-3-3 for prion disease. 
All were negative in the CSF. 

Thank you for your suggestion. 

Best regards, 

Dewton 

----- Mensagem original -----


> De: "Joseph Church" <JChurch at chla.usc.edu>

> Para: "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org>

> Enviadas: Terça-feira, 21 de Janeiro de 2014 15:52:25

> Assunto: RE: [cis-pidd] CVID with dementia



> JC Virus or prion disease?

> Joe Church



> From: Dewton Vasconcelos [mailto:dmvascon at usp.br]

> Sent: Tuesday, January 21, 2014 9:02 AM

> To: CIS-PIDD

> Subject: [cis-pidd] CVID with dementia



> Dear all

> We are following up a possible "CVID" patient that is really worrying

> us.

> GPP, 24 years old male patient, born to non-consanguineous parents.

> History of frequent diarrheas due to Giardia, upper respiratory

> infections and a few pneumonias since childhood. When he was 22

> years old (in 2011) he was at the college and noted progressive loss

> of memory for recent events leading to dementia in two years. At the

> end of 2011 he was evaluated by a neurologist who diagnosed

> neurologic Behçet’s disease based on NMR images and increase of

> lymphocytes in CSF. The CSF lymphocytes at that time suggested

> clonality not observed later on. He was treated with pulse

> methylprednisolone and immunosuppression with azathioprine for one

> year, changed to methotrexate and high dose IVIg. There was a dosage

> of serum Igs showing hypogammaglobulinemia of all isotypes during

> this period, but the neurologic team that was treating the patient

> didn’t note.

> When he was treated with IVIg and MTX he improved a lot from the

> respiratory infectious manifestations and presented some improvement

> of the neurologic manifestations. Nevertheless the initial

> neurologic improvement subsided and full-blown dementia developed,

> followed by progressive ataxia. At that time a new neurologist was

> consulted and thought about an enteroviral disease not confirmed by

> PCR in the CSF and sent to evaluation by immunology clinic.

> The patient was sent to us a few months ago with this clinical

> picture.

> We thought initially of an X-linked agammaglobulinemia with

> enteroviral infection (there was a coincidence of his neurologic

> deterioration with the born of his daughter) and late-onset ADA

> deficiency with neurological involvement. At that time Brazilian

> Ministry of Health used to distribute Oral Polio Vaccine (Sabin -

> OPV) for administration at the 2 nd , 4 th and 6 th month of life.

> The immunological evaluation excluded XLA and was highly suggestive

> of CVID, with normal counts of T, B and NK cells, decrease of

> switched memory B cells, plasmablasts and low CD21+ B lymphocytes.

> ADA activity was normal, as well as lymphoproliferation to PHA,

> anti-CD3, PWM and CMV, and low to tetanus toxoid. At that time IgM,

> IgE, IgM below limit of detection and IgG normal (receiving IVIg

> anti-inflammatory dosage).

> The evaluation of pathogens (fungal, mycobacterial, bacterial and

> mainly viral) was all negative. We tested by PCR for herpes (1-8),

> adenovirus, JC and BK viruses, HTLV/HIV, enteroviruses, astrovirus,

> flaviviruses, sapovirus, norovirus, arboviruses in CSF (all

> negative).

> Nuclear magnetic resonance images showed diffuse CNS atrophy, without

> any area suggestive of inflammation that could drive our biopsy.

> We are thinking in performing a brain biopsy in order to improve the

> sensitivity of the detection of any possible pathogen by PCR and

> looking at electron micrography to look for any possible viral

> particle.

> We are eager for any suggestion of possible diagnoses and tests that

> we didn´t think until now.

> Thank you very much,



> Dewton -- Dewton de Moraes Vasconcelos, MD, PhD Primary

> Immunodeficiencies Outpatient Unit ADEE3003 Lab. of Medical

> Investigation Unit 56 University of São Paulo School of Medicine

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