Dados do Trabalho

Título

Neurocryptococcosis presented as acute motor axonal neuropathy: an atypical manifestation

RESUMO

Case
A 33-year-old man, who worked as air conditioning technician, attended to the emergency department with progressive tetraparesis, ptosis in the left eye, hearing loss and sleepiness, associated with a 2-weeks history of malaise, headache, lumbar and cervical pain. Physical exam revealed arterial hypertension (164/88 mmHg) and tachycardia (116 bpm), with no fever and a normal cardiovascular auscultation. The patient presented with flaccid dysarthria, anisocoric pupils (left 3mm and right 2mm), with direct and consensual reflex preserved, left-ptosis and impaired adduction and upward gaze of left eye. At the motor exames there was tetraparesis, with MRC score of the right arm of 4, left arm 4- and 3 of both legs accompanying global areflexia. CT brain scan was normal and cerebrospinal fluid (CSF) showed pleocytosis (1350 cells/mm³) and hyperproteinorrhachia (215 mg/dL), suggesting an infectious process, the opening pressure was 54 cmH20. Gram staining was positive for encapsulated yeast , and cultures growth of Cryptococcus gatti. VDRL and HIV, HTLV, B and C virus serology were negative. Electromyography confirmed the presence of predominant motor acute axonal neuropathy (AMAN), an Guillain-Barre Syndrome (GBS) variant. It was promptly initialized treatment with immunoglobulin (IVIG), amphotericin B and fluconazole. With the treatment there was improvement of the strength, however he developed complications of the cryptococcal meningoencephalitis with refractory intracranial hypertension (a ventricular shunt was installed), severe hipoacusia and visual impairment.
Discussion
GBS is an aberrant reaction of the immune system, in genetically susceptible individuals, triggered by typically gastrointestinal infection (Campylobacter jejuni). The exact mechanism of the autoimmune reaction is not yet clarified. The association with fungal infection is very rare, there are very few reports of this association. It is not clear if this case was classic Guillain-Barre Syndrome, or a rare manifestation of cryptococcus infection as acute motor neuropathy.
Final comments
This case highlighted a very rare manifestation of neurocryptococcosis, and despite the fungal infection IVIG was introduced to treat the AMAN with relative success. Further studies are needed to clearly demonstrate this association.

Palavras Chave

Guillai-Barre Syndrome, AMAN, IgIV, Neurocrypyococcosis,