Register for the activity and create a StatPearls login.This activity is designated for 1.00 ANCC contact hours. Continuing Education, Inc, is jointly accredited by the Accreditation Council for Continuing Medical Education (ACCME), the Accreditation Council for Pharmacy Education (ACPE), and the American Nurses Credentialing Center (ANCC), to provide continuing education for the healthcare team. In support of improving patient care, this activity has been jointly planned and implemented by Continuing Education, Inc./University at Sea® and StatPearls, LLC. An autopsy on this patient could have assisted in clarifying the pathology behind this manifestation.Credit provided by Continuing Education, Inc, and StatPearls, LLC. The elevated intracranial pressure was most likely secondary to impaired CSF absorption at the arachnoid villi, which is most likely related to the presence of malignant WBCs in the CSF. Thus, we propose that infiltration from the Burkitt’s lymphoma and/or increased intracranial pressure due to hydrocephalus may have caused compression over the anatomical pathway serving the efferents of the near pupillary reflex. CSF testing confirmed the absence of neurosyphilis and meningitis, leaving the presence of malignant-appearing white blood cells (WBCs) as the primary abnormality in addition to the elevated opening pressure. The differential diagnosis of this finding is difficult, as IARP itself is a rare clinical entity. It has been suggested that neurons in the primate pretectal olivary nucleus are solely related to the pupillary light reflex and that the cortical projections to this pretectal nucleus are related to this reflex and do not play a role in the pupillary near response ( Zhang et al 1996). These reflexes are mediated by the oculomotor nerve and associated parasympathetic fibers originating from the Edinger-Westphal nucleus ( Bron et al 1997), however, the basis of this finding is difficult to explain. Incidently, the patient had no pupillary constriction on near gaze with preserved accommodation and convergence the light reflex, however, was also maintained. ![]() The long intracranial course of the trochlear nerve renders it vulnerable, which in this case was most likely affected by the elevated intracranial pressure. The patient’s symptom of diplopia can be attributed to the palsy of the right superior oblique muscle. Base out prisms (used to induce convergence) failed to evoke pupillary constriction. This was tested several times with full patient cooperation. The pupillary response of the near reflex was absent ( Figure 2B). Pupils were 3 mm, equal, and reactive to light ( Figure 2A). Slit-lamp examination and fundoscopy were normal with no involvement of optic nerves. Visual fields, color vision and optokinetic nystagmus were normal. Patient had hypertropia of the right eye ( Figure 1B), maximal on left gaze with Beilschowsky test positive confirming right superior oblique palsy. There was 6 diopters of accommodation by objective retinoscopy. ![]() On examination visual acuity was 20/20 in both eyes at near. Despite improvement in the hydrocephalus, ten days after the shunting procedure the patient developed diplopia for which the ophthalmology service was consulted. Based on elevated opening pressure and the presence of hydrocephalus on neuro-imaging, the patient underwent ventricular-peritoneal shunting. Subsequent bone marrow biopsy and HIV testing were also negative. The CSF tested negative for cryptococcal antigen and Venereal Disease Research Laboratory (VDRL) test, with no growth on bacterial and fungal cultures. Cerebrospinal fluid (CSF) analysis revealed normal protein and glucose levels with 220 WBC’s per high-powered field, which exhibited malignant morphologic characteristics. Spinal tap revealed an opening pressure of 41 cm H 2O. The studies demonstrated noncommunicating hydrocephalus without focal deficits or infiltrates. Size of the pupil 3 mm.Ĭomputed tomography (CT) scans with and without contrast and magnetic resonance imaging (MRI) of the head was obtained secondary to the presence of headache in order to evaluate for central nervous system involvement. ( B): Primary gaze: Showing right eye hypertropia. ( A): Lymph node biopsy: Showing the typical ‘Starry sky appearance’ seen in Burkitt’s lymphoma.
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