Abstract Introduction MPAL (Multi Phenotypic Acute Leukemia), BAL (Biphenotypic Acute Leukemia), AUL (Acute Undifferentiated Leukemia), ALAL (Acute Leukemia of Ambiguous Lineage), HAL (Hybrid Acute ...Leukemia), HAL (Hybrid Acute Leukemia), MLL (Mixed Lineage Leukemia) and aBLL (acute BiLineal Leukemia) represent different names of the same pathology or do these entities represent completely separate disease processes? These rather uncommon manifestations of acute leukemia complicate strict taxonomical sub grouping as well as their management. Rapid identification & swift management may restrict further neurological damage, while achieving hematological remission. Case description A 16 year female presented with gradual onset paraplegia for 7 months, a history of multiple blood transfusions in the past 2 years, pancytopenia, atypical cells in peripheral smear; the hematological and bone marrow work up revealed a diagnosis of: MPAL, B/Myeloid , NOS Multi Phenotypic Acute Leukemia, B cell {lymphoid}/Myeloid, not otherwise specified. An ALL type induction regimen was started. The management strategy, it's rationale and the clinical outcome are discussed. Conclusion For routine neurosurgical practice, these entities are extremely rare; and hence a working knowledge is very essential for appropriate & timely management notwithstanding the neurosurgical desire to rule out the compressive lesions first. Neurological status deterioration may be halted with timely institution of appropriate chemotherapy. In the extensive literature review in pubmed, this may be only the 1st case of MPAL with extramedullary neurological manifestation, at the first clinical presentation.
Background Isolated orbital neurofibroma unassociated with systemic neurofibromatosis is relatively rare and may be difficult to clinically differentiate from other orbital tumors. Sensory branches ...of the trigeminal nerve—namely lacrimal, nasociliary, and frontal—are the most common nerves of origin for intraorbital neurofibroma, but we discovered a neurofibroma arising out of the right trochlear nerve, in absence of clinical stigmata of neurofibromatosis type 1, which is rare. Case Description A 41-year-old adult presented with painless progressive proptosis of the right eye for 10 years without history of visual problems or diplopia. The right eye had axial proptosis with periorbital swelling. On magnetic resonance imaging (MRI), a right orbital extraconal, expansile, lobulated, cystic space-occupying lesion was seen with an enhancing component, pushing the lateral rectus with T1 isointensity and T2 hyperintensity, suggesting a preoperative working diagnosis of pseudotumor or lymphoproliferative tumor. Intraoperatively, a rudimentary slender, white, elongated structure was passing through the length of the tumor. The elongated tumor engulfing the trochlear nerve was traced up to the lateral part of the superior orbital fissure. The tumor was excised completely and was found to be a neurofibroma. Conclusion Isolated trochlear nerve neurofibromas, in the absence of clinical stigmata of NF1, are rare. Multiplicity, multilobulation, ring-configured contrast enhancement, and heterogenous MRI signal intensities help in the accurate preoperative imaging diagnosis. A possible cure is thus achievable with complete excision without damaging important adjacent neurovasculo-musculotendinous structures in the orbit. To the best of our knowledge, this is the fourth reported case of isolated trochlear nerve neurofibroma.
Background Cerebral aspergillosis, often encountered in immunocompromised patients, is almost always fatal despite radical surgical and medical management and frequently is a finding at autopsy. ...Attempts at fungal isolation often are unsuccessful, and a high index of radiologic suspicion is necessary. Case Description A premature, 5-month-old female infant, born via normal vaginal delivery, presented with a progressive increase in head size since birth, delayed developmental milestones, and intermittent vomiting for 1 month. There was no history of trauma and no evidence of diabetes mellitus or hypertension, family history of tuberculosis, gestational diabetes, jaundice, or febrile eruptions. Neonatal jaundice was present after day 1, remained for 2 days, and was treated with phototherapy. Findings of the general and systemic examinations were unremarkable. Findings of a neurologic examination revealed a hypoactive infant with papilledema and extensor plantar bilaterally. Computed tomography scan of brain showed hydrocephalus attributable to aqueductal stenosis. On endoscopic third ventriculostomy, dense, friable, whitish yellow, nonvascular masses were encountered in the third ventricle, obstructing it. Her endoscopic third ventriculostomy success score was 10 + 0 + 10 = 20%. Histopathologic examination and culture revealed Aspergillus flavus . A week later, a medium-pressure ventriculoperitoneal shunt was done, and voriconazole was added. Results At discharge on the 7th postoperative day, 4 weeks and 12 weeks later (ie, first and second follow-up) the child was active, playful, and feeding normally. The first reported case of an aqueductal stenosis due to aspergillosis was in 2000 by van Landeghem FK et al. (Clin Neuropathol 19:26−29, 2000). To the best of our knowledge, this may only be the second reported case of hydrocephalus attributable to aqueductal stenosis caused by Aspergillosis. In conclusion, aspergillosis should not be ruled out as a differential diagnosis in aqueductal stenosis, even when the patient seems to be immunocompetent.
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