Foster Kennedy Syndrome: A Case Report

Foster Kennedy Syndrome is a classic, yet rare, neuro-ophthalmologic syndrome due to an intracranial mass, most often a tumor, that consists of optic atrophy on the same side of the lesion and contralateral papilledema. We present the case of a 48-year-old female patient with decreased visual acuity and the typical clinical features described above due to a sphenoid wing meningioma. Although not a common condition, Foster Kennedy Syndrome should always be kept in mind in a patient with visual disturbances secondary to an intracranial mass.

NCMP-17. BEWARE: ALL POSTERIOR FOSSA MASSES IN CHILDREN ARE NOT BRAIN TUMORS

INTRODUCTION Isolated Myeloid sarcoma (MS) of the central nervous system (CNS-MS) is a very rare presentation of Acute Myeloid Leukemia (AML). A 10-year-old boy with a two-week history of intermittent headaches was referred to the emergency department (ED) by an optometrist for evaluation of bilateral papilledema. Physical examination in the ED confirmed the presence of papilledema with no other abnormal neurologic findings. Emergent MRI of the brain showed an avidly homogeneously enhancing extra-axial dural based mass in the right posterior fossa measuring 4.8 cm (AP) x 3.2 cm (CC) x 4.6cm (T), with significant mass effect and peri-tumoral vasogenic edema. Neuro-surgery consultation was obtained and complete blood count (CBC) was normal except for the presence of 13% blasts with occasional Auer rods seen on the peripheral blood smear. Bone marrow aspirate demonstrated 56% myeloblasts; A diagnosis of AML with CNS-MS (CNS-3c) was made and the initial lumbar puncture (LP) was deferred due to the risk of CNS herniation. Induction chemotherapy was initiated as per protocol. A CT scan done two weeks later showed complete resolution of the intracranial mass. DISCUSSION The incidental finding of papilledema, with imaging to investigate the intracranial pathology revealed a large posterior fossa mass in our patient. However, the atypical features of the mass (dural based extra-axial location; extensive infiltration into adjacent structures) and the finding of blasts with Auer rods in peripheral blood helped us to diagnose CNS-MS, and avoided an extensive neurosurgical procedure and a delay in definitive therapy. Our patient had a normal CBC and the review of the peripheral blood film by an experienced Hematologist was very crucial in making a clinical diagnosis of MS. CONCLUSION Myeloid sarcomas should be considered in the differential diagnosis of an intracranial mass, particularly when accompanied by abnormal findings in the CBC.

Panhypopituitarism Presenting as Progressive Visual Acuity Deterioration Secondary to Suprasellar Germinoma

Background: Overall the incidence of germ cell tumors worldwide is 0.1% with 60% comprising of germinomas. These may present in the suprasellar region a third of the time and thus impart variability in its presenting characteristics. Clinical Case: A 27-year male presented with progressive blurring of vision with incongruent visual acuity and refraction grade with optic disc pallor assessed with normal angiography. Patient also presented with increasing docility, sluggishness and more withdrawn behavior paired with polyuria, polydipsia and cold intolerance. Progressive worsening of vision led to cranial CT revealing an enhancing hyper density in the right caudate head later elucidated via MRI with several interspersed non-enhancing foci in the right caudate nucleus, both internal capsules with associated mild mass effect. The largest component located in the right capsular region with measurement of 21 x 21 x 16 mm with enhancing lesions in the hypothalamus and pituitary stalk. Endocrine evaluation revealed low serum cortisol at 91.03nmol/L (NV 138-685 nmol/L), responsive to ACTH stimulation test (65.94nmol/L to 387nmol/L), high prolactin at 856.9 mIU/L (42.4-296.8mIU/L) and low FT4 8.62pmol/l (9.01-19.05 pmol/L) despite normal TSH 1.528uIU/ml (0.35-4.94 uIU/mL). Optic nerve atrophy was now attributed to compressive intracranial mass. Secondary adrenal insufficiency was managed with hydrocortisone 50mgIV every 8 hours, and central hypothyroidism was managed with levothyroxine 75mcgtab 1tab once a day. Increased urinary output assessed as diabetes insipidus was given desmopressin 60mcg/tab ½ tab 2x a day at this time. Stereotactic biopsy of the brain revealed a germinoma of the right caudate lobe. Given the nature of the intracranial mass with high sensitivity to chemotherapeutic and radiotherapy, a multidisciplinary approach to treatment was taken with radiotherapy, hormonal and steroid replacement. Patient underwent external beam radiation therapy of the brain and spine for a total of 51 treatments allowing for improvement of visual acuity to counting, with polyuria less than 2-3 diaper changes per day. Conclusion: Optic disc pallor is a clinical sign that indicates optic nerve atrophy reflective of the optic tract. This may be an important clinical sign to increase clinical suspicion for intracranial mass lesion especially with correlation to manifestation of pituitary hormone deficiencies.

Discrimination of fetal intracranial mass and hemorrhage via superb microvascular imaging in a case and review of literature

In this case we aimed to present use of superb microvascular imaging (SMI) in the discrimination of fetal intracranial mass- haemorrhage with the light of literature. 21 weeks pregnancy woman applied to the clinic for routine obstetrics control. Ultrasonography (US) was applied to the patient. In the gray scale US, intracranial midline a large mass was detected. In the colour doppler (CD), the blood flow (BF) to the mass was not clearly seem. Power doppler (PD) was very artifact. In color SMI (cSMI) and monochrome SMI (mSMI), blood flow (BF) of the mass was seem clearly and fetal intracranial mass was prediagnosed. Magnetic Resonance imaging (MRI) was performed to determine the location of the mass, brain parenchyma and origin of the mass. Intracranial midline a large mass was detected in fetal MRI too. Although there are cases in the literature regarding the use of SMI in obstetrics, there is no case report in which fetal intracranial mass was evaluated by SMI to our knowledge. In our case; we have detected fetal intracranial mass by gray scale US, and we have evaluated vascularization of the mass by CD, PD, cSMI and mSMI. SMI modes were showing superiority when compared to CD and PD. Vascularization could be seen more clearly by mSMI when compared to cSMI.