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Title of Case(s)

Spontanous resolution, and setback thereafter, of fever and severe headaches - a "typical" presentation of a not-so-rare disease with an unusual complication.

Background

This case highlights important findings of leptospirosis:

1. Leptospirosis often presents with two phases, a complete remission of symptoms in between phases is possible.

2. Leptospirosis-induced acute kidney injury usually shows low potassium levels.

3. Meningitis can lead to intracranial hypertension.

Case Presentation Summary

A 15 year old girl presented to the ER late at night with 5 days of fever (40°C), neck pain, back pain and severe headaches (9/10 VNRS), no meningism, no pathologic neurological findings. Two days before presentation she came back from a summercamp in northern italy. Laboratory workup showed a highly elevated CRP, elevated creatinine, hyponatriemia, hypopotassemia, thrombocytpenia, lymphopenia. The main suspicion was a viral infection, but due to the severe symptoms we planned for a cMRI and LP.

However, for rounds before the tests (8 hours after admission) she presented with completely resolved symptoms. We deceided to cancel the diagnostics, but to keep her in the hospital for observation. 24 hours later she complained of a sudden severe headache and showed a clear meningism as well as papilledema. MRI showed flattened bulbi but no other pathological findings. LP opening pressure was 60cmH2O, and CSF showed pleocytosis, elevated lactate, low glucose, elevated protein. She was started on cefotaxime.

Our standard meningitis-workup includes culture, borrellia and TBE. Due to the two-phasic course of the illness, the AKI with low potassium levels, the meningitis and the camping holiday we performed leptospirosis-PCR of the urine, wich came back positive. With treatment her symptoms resolved quickly and had no squellae in her follow-up.

Key Learning Points

1. Leptospirosis is a two-phasic, often underdiagnosed zoonotic disease, can affect all organs while often leading to meningitis (with possible intracranial hypertension being rare); a peculiarity being the low potassium levels following kidney-involvement.

2. Travelhistory and exact analysis of all test-results are equally important for infectious diagnostic reasoning.

Title of Case(s)

DISSEMINATED DISEASE IN AN IMMUNOCOMPROMISED CHILD

Background

Invasive mould infections are a significant cause of morbidity and mortality in immunocompromised children.

The emergence of azole-resistant disease, with associated poor clinical outcome, is an ever-increasing challenge, requiring expert MDT input.

Case Presentation Summary

An 11 year old girl, with a background of recently diagnosed ALL, presented with acute onset of headache and decreased level of consciousness. Urgent neuroimaging revealed right cerebellar abscess (4x3x2cm) with supratentorial extension, moderate mass effect and early obstructive hydrocephalaus. Emergency neurosurgery involved partial resection of abscess and broad spectrum antibiotic and antifungal agents were commenced. Further imaging revealed pulmonary and hepatic lesions consistent with invasive fungal disease(IFD).

Cerebellar biopsy grew Aspergillus fumigatas which was pan-azole resistant (MIC ~4) but highly sensitive to amphotericin (MIC 0.5) International experts recommended dual-agent approach with amphotericin (l-amb) 3mg/kg OD and isavuconazole (Isavu) 10mg/kg OD. Serum & CSF TDM was strictly monitored, with aim for supratherapeutic Isavu levels of 4-6mg/L.

She is now almost 18 months into IFD treatment.No further surgical interventions have occurred. Serial imaging has revealed >50% size reduction of the cerebellar abscess remnant, improvement in pulmonary nodules and normalisation of liver appearances. Her treatment course has been complicated due to repeated acute kidney injuries requiring multiple hospital readmissions, prompting initial dose adjustment and then discontinuation of l-amb. Recently, new persistent neutropenia (ANC 0.3-0.9) has warranted investigations to exclude primary disease relapse. Despite the significant improvement and progress, her journey remains challenging and prognosis guarded.

Key Learning Points

Current recommendations suggest IV Voriconazole is the most effective evidenced-based treatment for invasive aspergillosis.
Novel agent isavuconazole, whilst unlicensed and has limited long-term safety data, appears to be as effective with a more favourable side-effect profile and more predictable PK/PD behaviour.

In cases of pan-azole resistant CNS aspergillosis, a multidisciplinary team including ID experts, mycologists, microbiologists , pharmacists and neurosurgeons should be involved.

Title of Case(s)

Recurrent Encephalitis

Background

Herpes simplex (HSV-1) encephalitis is the most common cause of viral encephalitis in infants and children, a potentially devastating disease that can present with non-specific features. Recurrence of HSV-1 encephalitis has been reported in 50% of paediatric cases despite treatment with aciclovir.

Case Presentation Summary

This is a presentation of a 3 year old child with 3 discreet episodes of encephalitis. The initial presentation was at 2 months of age with febrile focal seizures requiring phenytoin. Her CSF had no significant white cells, culture and viral PCR negative. She had normal MRI but her electroencephalogram showed epileptiform discharges over mid-parietal area. Her ceftriaxone and aciclovir were discontinued at 48 hours. Her second presentation was at 2 years with fever and vomiting, progressing to lethargy, irritability and upgoing plantars on examination. She had an urgent MRI that showed a left frontal parenchyma haematoma and a subdural bleed. She was commenced on ceftriaxone, aciclovir and clarithromycin and transferred to a tertiary centre where she had a LP which was positive for Herpes Simplex Type 1 (HSV-1). She was treated with 3 weeks of aciclovir and post-treatment CSF was negative for HSV-1. However, she presented again at 3 years with fever, headache and vomiting. MRI showed encephalomalacia and interval development of multiple areas of vasogenic oedema. Her LP had high opening pressure and protein but no significant white cell count and was negative for bacterial culture, HSV-1 and all other viruses tested. She had a brain biopsy that showed florid granulomatous and necrotising inflammation with high levels of HSV-1 and considered to be post-HSV-1 granulomatous disease of the brain, a post-infectious inflammatory reaction. She was managed with prolonged high-dose aciclovir and steroids, physiotherapy and occupational therapy, and her clinical outcome is to be observed.

Key Learning Points

If HSV-1 encephalitis recurs – don’t assume is it straight-forward relapse.

Title of Case(s)

A baby with painful bones

Background

Congenital syphilis, although nowadays uncommon thanks to screening in pregnancy, should be considered in young infants with periostitis or fractures, especially if accompanied by other symptoms.

Case Presentation Summary

A 2-month-old girl, born at term following induction of labour for reduced growth velocity, was taken to her local Emergency Department (ED) with swollen wrist. A plain radiograph showed a fracture. The patient was investigated for Non-Accidental Injury (NAI): a skeletal survey showed periosteal reaction in all long bones and 2 more fractures. She was investigated for metabolic disorder and the family were referred to social services for suspected inflicted injuries. The child returned 1 month later with fever, rash, coryza, hepatosplenomegaly. Following a full septic screen she was treated on IV Ceftriaxone, with resolution of fever. Screening test for syphilis antibody was positive, therefore the patient was transferred to our paediatric infectious disease department for further investigations which showed:

Of note, maternal VDRL in first trimester was negative. The child received IV Penicillin G for 2 weeks. By the end of treatment her inflammatory markers started rising again and Xray of long bones showed new periosteal reaction, so Ceftriaxone and Clindamycin was recommenced. However an MRI full body showed that the symmetrical periosteal changes were consistent with avid healing process.

Key Learning Points

Presence of fractures in non-ambulating infants should raise the suspicion of NAI. However, when these are accompanied by osteitis, fever, rash and hepatosplenomegaly, congenital syphilis should be considered.

Repeating imaging too early in the course of the disease can lead to misinterpretation of changes reflecting normal healing process.

There are no studies of the optimal duration of treatment for severe bony syphilis.