Case 1- 38yo male, ophthalmologist

DX- reactive arthritis [(triad of arthritis, conjunctivitis/uveitis, urethritis/cervicitis), present after 1-3wks
with mucocutaneous lesions; mostly knee or sacroiliac joints involved; common agents- Salmonella,
Shigella, c.jejuni, Yersinia, chlamydia, ureaplasma and group A strep.]

- right eye conjunctivitis 5h earlier – prescribed Chloramphenicol eye drops for dx of viral
conjunctivitis.
- Next day became bilateral and purulent + sore throat
- 3d later tenderness and swelling of PIP of index finger of left hand => dx of septic arthritis- tx-
amoxicillin 500mg TID, flucloxacillin 500mg QID
- After 2d, dx of reactive arthritis was made

Tests-

1. Xray of finger
2. ANCA –ve, RF –ve, anti-DNA ab and ANA –ve
3. Joint aspiration – no pathogen
4. HLA-B27 - -ve

Tx-

NSAIDS – resolved in 48h

[usually ESR and CRP elevated, if not resolved in 6 m treat with sulfasalazine/MTX]
Case 2- 13yo female

DX- Acute disseminated encephalomyelopathy [presents with lethargy, confusion, tremors, dementia,
muscle twitching, seizures, paralysis, N&V]

- Presented with progressive paralysis and encephalopathy

- Mild viral like illness preceding week
- Later developed back pain, difficulty walk.
- Morning of adm, developed headache, vomiting, LOC and leg paralysis
- No fever, rash, lymphadenopathy, HSMG or sepsis

History-
ALL B type at age 2, tx by UKALL XI protocol (induction with Vincistrine, Prednisolone, Asparaginase and
MTX)- after 6 m -> encephalopathy due to relapse. Received cranial irradiation and UKALL R2 relapse
protocol. Proceeded with BMT.

Tests-

- Absent DTR and abdominal reflexes

- Urinary retention and stool incontinence
- MRI- diffuse CNS involvement- enhancement with gladolinium, swollen spinal cord-
hyperintense from C1
- CSF- 125 WBC- lymphoblastic, 5 RBC, raised protein, CSF:blood lactate ratio raised, low
CSF:glucose
- CSF cytospin showed lymphocytes
- Immunophenotyping revealed T cells of donor type – confirmed no relapse of ALL
- No viral infection found by serology – dx ADEM

Tx-

IV high dose methylprednisolone and Acyclovir

Barthel Index scoring system used for assessment of recovery

Differential diagnoses for ADEM after ALL BMT

Treatment side-effects

Cyclosporine (posterior leukoencephalopathy syndrome)

Amphotericin (parkinsonism)

Radiation sequelae (arteriopathy, vacuolating encephalomyelopathy)

Infections

Viruses (HSV, VZV, CMV, EBV, HHV6, HHV7, JC, BK, adenovirus, West Nile Virus)

Parasites (Toxoplasma, amoeba)

Differential diagnoses for ADEM after ALL BMT

Fungi (Aspergillus, Candida)

Bacteria (abcesses, Listeria, Mycoplasma, TB)

CNS relapse of leukaemia

Inflammation

Acute disseminated encephalomyelitis

Multiple sclerosis

Vasculitides (SLE, CNS angiitis)

Haemorrhage/infarction

Thrombocytopoenic thrombotic purpura

Secondary to radiation arteriopathy

Idiopathic subarachnoid and subdural haemorrhage

To rule out relapse and other causes

- Absence of meningitic contrast enhancement due to leukemic infiltrates and presence of

parenchymal lesions and intramedullary spine involvement
- Immunophenotyping showed absence of CD10 antigen, presence of CD2 and CD7, absence of
tdt (immature cells marker)
- Cytogenetics showed donor male cells, and no clonal expansion
- Infections are early sequelae rather than late
- The classical lesions of ADEM on MRI are multifocal lesions in the brain white matter, cortical
grey matter and basal ganglia