Female schoolboy, from Antofagasta, who consulted for the first time in the Department of Surgery characterized by volume increase at Luis Calvo Mackenna Hospital at the age of 5 years 4 months, for an inla
Biopsy of the lesion had been performed at the site of origin, showing a chronic nonspecific granulomatous process, with negative staining for Mycobacterium and Ziehl-Neelsen staining.
Among the tests previously performed, the following stood out: blood count with mild microcytic anemia, high ESR (105 mm/h), CRP of 35 mg/l and a chest X-ray reported as normal.
The physical examination at admission revealed a thickened nose with erythema and induration of both nasal wings and telangiectasic lesions; the left ciliary erythematous lesion was also present.
The diagnosis was a facial granulomatosis, possible sarcoidosis.
In the study performed there were: CBC with microcytic anemia, ESR of 75 mm/h negative autoimmunity study, quantification of immunoglobulins and normal complement components C3 and C4 sputum smears negative CD4+ secretion, with negative subpopulations
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A new biopsy of the facial lesions showed a non-granulomatous histiocytic infiltrate of Mycobacterium tuberculosis and tuberculous dermatitis was interpreted as a positive tuberculous study with few eosinophils, which formed foci with some multinucleated giant cells.
For this reason, treatment for mycobacteriosis was initiated extrapulmonary with streptomycin, isoniazid, rifampicin and ciprofloxacin, despite the fact that the affected cutaneous area was extended.
The patient returned to her place of origin to continue treatment on an outpatient basis.
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Approximately three months after starting treatment she developed a right VIo cranial nerve palsy, which resolved spontaneously.
On that occasion, a brain CT scan showed a small nonspecific sequel calcification of the right parietal cortex.
Due to the clinical evolution, with extension of the affected skin area and persistently elevated ESR (about 81 mm/h), prednisone was added to the treatment previously described, oriented towards mycobacteriosis.
A skin biopsy was repeated, showing a new chronic granulomatous dermatitis. The PCR for Mycobacterium was repeated, which in this occasion was positive for concomitant negative M. tuberculosis gene sequences, with culture of Koch.
Subsequently, due to the progression of facial lesions, methotrexate was added.
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It was followed up for six months when the patient presented progressive gait instability and diplopia, associated with general malaise, headache and persistent fever up to 38.5 °C axillary.
On physical examination, cutaneous lesions, pairs of third and fourth cranial nerves bilaterally and probable involvement of the eighth cranial nerve were evident. A multiple cranioneuritis was diagnosed.
Lumbar puncture was compatible with clear liquid meningitis and treatment was modified to rifampicin, pyrazinamide, streptomycin and isoniazid.
A normal determination of adenosine deaminase was requested in the CSF, Ziehl-Neelsen stain, current culture and Koch culture were negative.
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Despite the treatment she presented progressive consciousness compromise, so a brain MRI was performed showing lesions compatible with acute disseminated encephalomyelitis (ADEM).
Considering the clinical and radiological evolution, the differential diagnosis was proposed with an infection by LVA, HIV encephalitis, neurosarcoidosis and neurolúes; for this reason, it was decided to repeat the lumbar puncture.
Among the tests performed in the CSF, the following should be highlighted: a negative PCR for M. tuberculosis, direct examination of honeycombing free life that did not demonstrate the presence of this oligoclonal band and positive (negative).
Serology of VDRL and ELISA for HIV were repeated with new negative results.
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Considering the diagnosis of ADEM, she received treatment with methylprednisolone and immunoglobulin, despite which she presented progressive neurological deterioration.
It was then decided to perform a brain biopsy to clarify the diagnosis; however, the patient presented severe stenosis with extensive involvement of the brainstem, dying approximately one year after the onset of symptoms.
In the anatomopathological study, the most significant findings were limited to the CNS.
Macroscopic evaluation of the brain revealed flattened circumlocutions, congestive leptospirosis and severe brainstem compromise.
Histological examination showed the presence of granulomatous lesions in skin and lungs Spherules of 30 μ were observed in the spinal cord with a thickened, sharp refractive wall consistent with AVL cysts and surroundings.
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The sample was evaluated in the laboratory of Parasitology at Luis Calvo Mackenna Hospital, the suspicion of LVA was confirmed and the sample was sent for study with PCR for Acanthamoeba negative reference to the Instituto Salud.
A consultation made at the CDC reference center, Atlanta, E.U.A., confirmed the presence of B. mand due to its morphological aspect, which was concluded in the postmortem diagnosis.
