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Print version ISSN 0004-282X
Arq. Neuro-Psiquiatr. vol.70 no.1 São Paulo Jan. 2012
Múltiplos infartos subcorticais causados por mucormicose em paciente com linfoma
Pedro Enrique Jiménez Caballero; Alfonso Miguel Falcón García; Juan Carlos Portilla Cuenca; Ignacio Casado Naranjo
Department of Neurology, San Pedro de Alcántara Hospital, Spain
Mucormycosis is caused by a saprophytic fungal infection by non-septate hyphae of the genus Mucor. Five classic forms are recognized: rhinocerebral, pulmonary, gastrointestinal, cutaneous, and disseminated. Rhinocerebral mucormycosis (RCM) frequently occurs in immunosuppressed patients, such as those with uncontrolled diabetes mellitus or haematological malignancy1.
A 56-year-old man was diagnosed with follicular non-Hodgkin's lymphoma. Four months prior to admission, he presented a right headache attributed to rhinosinusitis. Biopsy showed mucormycosis. There was no affectation intracranial in the brain magnetic resonance imaging (MRI), but the patient became blind due to involvement of both central retinal arteries. He underwent debridement of the paranasal sinuses and treatment with liposomal amphotericin B for two months and posaconazole continuously.
He was admitted by recurrent episodes of nonfluent aphasia, right sensory disturbance, and motor weakness. In the last episode, the symptoms persisted and the patient had transcortical motor aphasia, right hemiparesis with dominance of the lower limb (manual muscle testing: upper limb 2/5, lower limb 1/5) and right unilateral sensory impairment, which was restricted at his perioral area and homolateral distal hand and foot.
Hemogram showed lymphopenia (0,3x109/L) and thrombocytopenia (23x109/L). Blood clotting was normal. Plasma and urine ketone bodies were absent and other laboratory studies were normal. Echocardiogram, cardiac monitoring and Duplex of the supra-aortic trunk were normal. Transcranial duplex showed 70% stenosis at the M1 segment of left middle cerebral artery (MCA) with no other significant changes. MRI brain on T1 sequences with contrast (Fig A) showed an uptake at both cavernous sinus and internal carotid wall, which determines a 70% arterial lumen stenosis in the left side. On T2 sequences (Fig B), multiple subcortical ischemic lesions in the left MCA territory could be seen.
The patient was diagnosed from multiple subcortical strokes in the territory of left MCA secondary to infiltration of its wall caused by mucormycosis. He was again treated with liposomal amphotericin B for two months, with clinical and radiological improvement. In the six-month follow-up, no new cerebrovascular disorders have appeared.
Mucor is attracted to blood vessels and invasion of their wall (particularly arterial) is the pathological trademark of the infection. The integrity of host defense mechanisms plays a key role. The presence of certain underlying diseases as lymphoma provides a favourable microenvironment for fungal growth. The disease usually spreads to the cavernous sinus, internal carotid artery and subsequently to the brain2. Vascular manifestations of mucormycosis include pseudoaneurysms, partial thrombosis, narrowing and arteritic irregularities of intracranial arteries. Their pathologic basis is believed to be a combination of direct endothelial injury and growth of hyphae into the lumen, with resultant distal infarcts and mycotic emboli. Vasculitis usually occurs in the supraclinoid portion of the internal carotid artery. Mycotic pseudoaneurysms, arterial dissection, or venous congestion could lead to intracranial hemorrhages3. Rapid thickening and enhancement of the carotid artery wall on serial MRI establishes the nature of the arterial involvement by mucormycosis rather than atherosclerosis4.
The prognosis is always very poor once the carotid artery is involved. A few successful cases have been reported following extensive sinus debridement and intravenous amphotericin B therapy5.
1. Mathur SC, Friedman HD, Kende AI, Davis RL, Graziano SL. Cryptic mucor infection leading to massive cerebral infarction at initiation of antileukemic chemotherapy. Ann Hematol 1999;78:241-245. [ Links ]
2. Simmons JH, Zeitter PS, Fenton LZ, Abzug MJ, Fiallo-Scharer RV, Klingensmith GJ. Rhinocerebral mucormycosis complicated by internal carotid artery thrombosis in a pediatric patient with type 1 diabetes mellitus: a case report and review of the literature. Pediatr Diabetes 2005;6:234-238. [ Links ]
3. Takahashi S, Horiguchi T, Mikami S, Kitamura Y, Kawase T. Subcortical intracerebral hemorrhage caused by mucormycosis in a patient with a history of bone-marrow transplantion. J Stroke Cerebrovasc Dis 2009;18:405-406. [ Links ]
4. Calli C, Savas R, Parildar M, Pekindil G, Alper H, Yunten N. Isolated pontine infarction due to rhinocerebral mucormycosis. Neuroradiology 1999;41:179-181. [ Links ]
5. Galetta SL, Wule AE, Goldberg HI, Nichols CW, Glaser JS. Rhinocerebral mucormycosis: management and survival after carotid occlusion. Ann Neurol 1990;28:103-107. [ Links ]
Pedro Enrique Jiménez Caballero
Calle Dionisio Acedo, 9 - Portal 7, 4-1ª, 10001 Cáceres - Spain
Conflict of interest:
There is no conflict of interest to declare.
Received 19 June 2011
Received in final form 22 July 2011
Accepted 29 July 2011