Left Eye Ptosis Research Articles

Nasopharyngeal B-cell lymphoma with pan-hypopituitarism and oculomotor nerve palsy: a case report and review of the literature

BackgroundPrimary nasopharyngeal lymphoma (NPL) is a very rare tumor of Waldeyer ring (WR) lymphoid tissue. It is challenging to differentiate lymphoma infiltration of pituitary from a pituitary adenoma, meningioma infiltration, and other sellar lesions to plan a suitable treatment strategy. We presented for the first time a unique case of NPL with an unusual presentation of oculomotor nerve palsy associated with pan-pituitary involvement in a diabetic patient.Case presentationA 64-year old diabetic woman with no previous history of malignancy presented with intermittent diplopia for about the last nine months. Severe headache, left eye ptosis and hypoglycemic episodes were added to her symptoms after a while. Further complaints include generalized weakness, loss of appetite, generalized musculoskeletal pain, and 6–7 kg weight loss within six months. Her family history was unremarkable. Physical examinations of eyes indicated left eye 3rd, 4th, and 6th nerve palsy. But, she was not anisocoric, and the pupillary reflexes were normal on both eyes. No lymphadenopathy, organomegaly and other abnormalities were found. Magnetic resonance imaging (MRI) showed a heterogeneous enhancement in the seller and suprasellar regions, enlargement of the stalk, parasellar dural enhancement and thickening of the sphenoid sinus without bone erosion. Also, both cavernous sinuses were infiltrated and both internal carotid arteries were encased by the neoplastic lesion. It suggested an infiltrative neoplastic lesion which compressed the cranial nerves. Pituitary hormone levels assessment indicated a pan-hypopituitarism. Following nasopharyngeal mucosal biopsy, the immunohistochemistry (IHC) findings revealed a low-grade non-Hodgkin’s B-cell lymphoma. Systemic workup, including cerebrospinal fluid (CSF) studies, bone marrow aspiration, chest and abdominopelvic high-resolution computed tomography (HRCT) indicated no other involvement by the lymphoma. After chemotherapy courses, central adrenal insufficiency, partial central diabetes incipidious (CDI) and central hypothyroidism have been resolved. To our best knowledge, we found 17 cases of NPL with cranial nerve palsy, 1 case of NPL with pan-hypopituitarism and no NPL case with both cranial nerve palsy and pituitary dysfunction.ConclusionsThe incidence of cranial neuropathy in patients with diabetes should not merely be attributed to diabetic neuropathy without further evaluation.

Pituitary apoplexy induced by gonadotropin-releasing hormone agonist administration: a rare complication of prostate cancer treatment.

SummaryGonadotropin-releasing hormone (GnRH) agonists, currently used in the treatment of advanced prostate cancer, have been described as a rare cause of pituitary apoplexy, a potentially life-threatening clinical condition. We report the case of a 69-year-old man with a known pituitary macroadenoma who was diagnosed with prostate cancer and started treatment with GnRH agonist leuprorelin (other hormones were not tested before treatment). Few minutes after drug administration, the patient presented with acute-onset severe headache, followed by left eye ptosis, diplopia and vomiting. Pituitary MRI revealed tumor enlargement and T1-hyperintense signal, compatible with recent bleeding sellar content. Laboratory endocrine workup was significant for low total testosterone. The patient was managed conservatively with high-dose steroids, and symptoms significantly improved. This case describes a rare phenomenon, pituitary apoplexy induced by GnRH agonist. We review the literature regarding this condition: the pathophysiological mechanism involved is not clearly established and several hypotheses have been proposed. Although uncommon, healthcare professionals and patients should be aware of this complication and recognize the signs, preventing a delay in diagnosis and treatment.Learning points:Pituitary apoplexy (PA) is a potentially life-threatening complication that can be caused by gonadotropin-releasing hormone agonist (GnRHa) administration for the treatment of advanced prostate cancer.This complication is rare but should be taken into account when using GnRHa, particularly in the setting of a known pre-existing pituitary adenoma.PA presents with classic clinical signs and symptoms that should be promptly recognized.Patients should be instructed to seek medical care if suspicious symptoms occur.Healthcare professionals should be aware of this complication, enabling its early recognition, adequate treatment and favorable outcome.

MON-239 Pituitary Apoplexy Induced by Gonadotropin-Releasing Hormone Agonist Administration: A Rare Complication of Prostate Cancer Treatment

Background: Pituitary apoplexy is a potentially life-threatening clinical condition associated with bleeding and/or infarction into the pituitary gland, usually within a tumor. Gonadotropin-releasing hormone (GnRH) agonists, currently used in the treatment of advanced prostate cancer, have been described in the literature as a rare cause of pituitary apoplexy. Case: We report the case of a 69 year-old man with a known pituitary macroadenoma incidentally detected in 2016, without further investigation. He was diagnosed with prostate cancer in 2017 and underwent retropubic prostatectomy. Two years later there was evidence of histologic prostate tumor progression, so he started treatment with leuprorelin 45mg (GnRH agonist). Immediately after the first subcutaneous injection he presented with acute-onset severe headache, followed by left eye ptosis, diplopia and vomiting. Left cranial nerve III palsy was confirmed by examination in the emergency department. Head computed tomography showed a lesion in the sellar region; laboratory endocrine workup was significant for total testosterone 72.07 ng/dL (86.49 – 788.22), with no other abnormalities. Magnetic resonance imaging of the pituitary revealed tumor enlargement and a T1-hyperintense signal, compatible with recent haemorrhagic sellar content. The patient was managed conservatively with high dose steroids, and symptoms were significantly improved on discharge. Discussion: Pituitary apoplexy induced by GnRH agonist administration is a rare complication, described in only 20 documented cases to date. The pathophysiologic mechanism involved is not clearly established and several hypotheses have been proposed: a combination of metabolic hyperactivity, cell division/tumor growth and increased intrasellar pressure, inducing ischemia in a poorly perfused adenomatous tissue given the demand. Although uncommon, healthcare professionals should be aware of this serious consequence of GnRH agonist administration and recognize the signs, preventing a delay in diagnosis and treatment.

MON-255 Oral Contraceptive Pills Mask an Aggressive Crooke’s Cell Pituitary Adenoma

BackgroundCrooke’s cell tumors are rare and aggressive forms of pituitary adenomas. This variant of Cushing’s disease requires prompt diagnosis to avoid life-threatening complications. We report a unique case of Crooke’s cell tumor with longstanding history of irregular menstrual cycles, undiagnosed and later presented as acute unilateral ptosis and diplopia due to aggressive tumor invasion.Clinical Case23-year-old female presented to the ER with facial swelling, left eye droop and diplopia for 3 days. She had a past medical history of oligomenorrhea and hirsutism which was normalized by oral contraceptive pills (OCP)- a combination of ethinyl estradiol and drospirenone for the last 3 years. Years prior, workup of her oligomenorrhea showed normal androgenic profile with normal DHEA-S, testosterone and 17-OH progesterone. Current exam was also significant for elevated blood pressure 200/110mmHg, BMI 37, pigmented abdominal striae and terminal hair over her chin. Labs remarkable for hypokalemia K+ 2.7 mmol/L (3.5-5.3), elevated AM cortisol 51 mcg/dL (4-20), low TSH 0.152 mcUnit/mL (0.4-5.0), low IGF-1 170 ng/mL (222-566) and FSH 1.4 mUnit/mL (1.0-9.0), with normal prolactin 24.3 ng/mL (<0.5-25) and free T4 0.87ng/dL (0.8-1.8). MRI brain showed 2.8cm homogenous enhancing soft tissue mass involving the central skull base, sphenoid sinus, sella, suprasellar cistern, and parasellar regions; displacing the optic chiasm, and invading the cavernous sinuses bilaterally and orbital apices. Post trans-sphenoidal surgery (TSS) of the pituitary mass, her left eye ptosis and diplopia resolved. Post-op MRI showed subtotal resection of the extra-axial enhancing abnormality at the central skull base with extension to multiple other anatomic spaces. Pathology read consistent with aggressive Crooke’s cell adenoma, showing invasive biologic behavior without an elevated proliferation index with positive ACTH immunohistochemistry supportive of corticotroph cell adenoma. Post-op ACTH level 73 pg/mL (6-50) and cortisol 12.5 mcg/dL (4-20), while on dexamethasone. Repeat TSS was performed for residual adenoma. Cortisol remains elevated at 15.7mcg/dL despite high dose dexamethasone taper by the neurosurgery team for post-op development of right eye ptosis. She is currently awaiting proton beam radiation therapy.ConclusionCrooke’s cell tumors are an aggressive form of pituitary adenoma for which early diagnosis is crucial for its prognosis. Our case highlights the importance of maintaining a wide differential in evaluating young women with menstrual irregularities and to include screening for Cushing’s syndrome. Empiric treatment with OCPs can mask symptoms in the earlier course of Cushing’s disease as in our patient, causing recognition only after presentation with significant tumor growth. Earlier detection could have prevented adenoma invasion and potential neurological sequelae.

SAT-LB54 Acute Sterile Meningitis as a Primary Manifestation of Pituitary Apoplexy

Background: Pituitary apoplexy (PA) is a rare endocrinopathy that requires prompt diagnosis and treatment. Presentation with acute neutrophilic meningitis is uncommon.Clinical Case: A 67-year-old man presented to our hospital with a 2-week history of worsening bilateral frontal headache, nausea, and dry heaving. On admission, the patient was somnolent with a score of 13 on the GCS assessment (E2, V5, M6). The neurological exam was overall normal with normal ocular motion and intact cranial nerves, except for a left eye peripheral vision defect. Plain head CT revealed a well-circumscribed ovoid pituitary mass with suprasellar enlargement, consistent with a pituitary macroadenoma. Sellar MRI showed a pituitary mass, roughly 20 x 19 x 24 mm, bulging into the suprasellar cistern with optic chiasm elevation. Analysis of pituitary function revealed low ACTH concentration of 2.8 pg/mL (n = 7.2 - 63.3 pg/mL), a low random cortisol level of 1.7 ug/dL (n = 2.9 - 19.4 ug/dL), a low TSH of < 0.1 uIU/mL (n = 0.35 - 4.9 uIU/mL), a low free T4 level of 0.72 ng/dL (n = 0.77 - 1.48 ng/dL), a low LH of 0.8 IU/L (n = 1.7 - 8.6 IU/L) with a very low total testosterone level of < 3 ng/dL (n = 300 - 720 ng/dL) and normal prolactin, IGF-1 and GH levels. On hospital day 2, the patient had worsening encephalopathy with left eye ptosis and decreased vision. Repeat CT and MRI showed no interval change in the pituitary adenoma or evidence of bleeding. An immediate lumbar puncture was performed and CSF analysis revealed an increased leukocyte count of (1106/mm3) with 89% neutrophilic granulocytes, and increased total protein level of 138 mg/dL (n = 15 - 40 mg/dL), red blood cell count of 2040 without xanthochromia and glucose of 130 mg/dL (n = 40 - 70 mg/dL). Based on the laboratory results and new symptoms, empirical antibiotic (vancomycin, ceftriaxone, and ampicillin) therapy was started for suspected bacterial meningitis before the confirmation of the CSF culture study. CSF culture did not grow any organisms. Given the sudden visual impairment and neurological deterioration, the patient underwent transsphenoidal resection of the tumor with free nasal mucosal graft reconstruction. Histological examination revealed a necrotic pituitary adenoma with apoplexy and no evidence of hemorrhage. Postoperatively, his neurological exam greatly improved. His left pupil was reactive to light and the third palsy was improving.Conclusion: This case reinforces the importance of including PA in the differential diagnosis of acute headache, particularly in patients presenting with visual disturbances. Patients with PA often present with sterile meningitis due to increased debris and blood in the subarachnoid space which closely mimics acute bacterial meningitis. While MRI remains a sensitive imaging modality for the detection of PA, the latter remains a clinical diagnosis. Timely diagnosis with high clinical suspicion and treatment is essential.

SUN-286 Lymphocytic Hypophysitis Mimicking Tolosa Hunt Syndrome

Introduction:Lymphocytic hypophysitis often presents with headache, hypopituitarism and visual disturbance, the latter from optic nerve compression. Rarely, it can present with diplopia from cranial nerves III, IV and VI (3.7%) and cavernous sinus involvement (1).Clinical Case:A 40 year old woman presented with left eye pain, blurry vision, ptosis and diplopia for 2 days, preceded by headache for 2 weeks. Exam was remarkable for left eye ptosis, mild proptosis, downward and outward gaze and inability to adduct her left eye. Endocrinological exam revealed free T4 0.67 ng/dL (Nl 0.70 - 1.48), TSH 0.67 ng/dL (Nl 0.70–1.48), estradiol <10 pg/mL, LH 1.0 mIU/mL, FSH 6.9 mIU/mL, prolactin 23.3 ng/ml (Nl 5.2–26.5) and IGF-1 95 ng/mL (Nl 52–328). Cortisol was not assessed as patient was already on steroids. Work-up revealed atypical ANCA (1:320) but normal C-ANA (<1:20), P-ANCA (<1:20), and the rest of immune work-up was negative including ACE, ESR, CRP, ANA, serine protease and myeloperoxidase. No systemic manifestations were present concerning for systemic autoimmune disease. CSF exam was unrevealing including a normal ACE level. MRI revealed an enlarged pituitary gland with suprasellar extension containing a focal area of T2 hyperintensity and slight T2 hypointensity at the posterior aspect of the gland. There was a midline, thickened infundibulum, enhancement of both cavernous sinuses and narrowing of right internal carotid artery without occlusion. Endoscopic endonasal transsphenoidal biopsy of pituitary lesion confirmed diagnosis of lymphocytic hypophysitis and did not meet criteria for IgG4 hypophysitis. After 4 weeks of prednisone, she had significant symptomatic improvement and repeat MRI showed decreased pituitary size but persistent abnormal enhancement of the pituitary gland and cavernous sinuses.Conclusion:The atypical and variable clinical and radiological findings of lymphocytic hypophysitis can mimic other inflammatory, infiltrative lesions, pituitary tumor with apoplexy and Tolosa Hunt Syndrome. Tolosa Hunt syndrome is an idiopathic granulomatous inflammation of the cavernous sinus involving cranial nerves II to VI and often presenting with painful ophthalmoplegia. Pituitary involvement and carotid artery narrowing have been observed (2). Our case highlights a patient with cranial nerve III palsy and significant cavernous sinus involvement, clinically concerning for Tolosa Hunt syndrome, but confirmed by biopsy to be lymphocytic hypophysitis. There are no specific serum markers to distinguish lymphocytic hypophysitis from other entities and when uncertain, diagnosis is best established by biopsy.

Unilateral Frontal Sinus Aspergillosis: A Case Report and Review Literature

Frontal sinus aspergillosis is a rare infection that can be usually associated with immunocompromised states and life-threatening with high mortality rate. The authors report in a developing country a case of immunocompetent patient with left unilateral frontal headache, associated with left eye ptosis. The Brain CT Scan revealed frontal sinus aspergillosis. She underwent surgery followed by itraconazole cure. The outcome is successful after a period of 14 months with resolution of symptomatology. Anatomopathological examination confirmed aspergillosis. They share their experience of management of this disease in a country where neurosurgery is very young specialty and where molecules against aspergillosis are not available.

Rare Case of Simultaneous Diabetic Oculomotor and Trochlear Nerve Neuropathy: Case Report

INTRODUCTION: Diabetic cranial neuropathy is a spectrum of diabetic neuropathy. Isolated cranial nerve involvement is common as the manifestation of diabetic cranial neuropathy. The cranial nerve that frequently involved are abducens and oculomotor nerve, trochlear nerve is the least involved. Simultaneous cranial nerve involvement is not common and need further investigation for other possible etiology. CASE REPORT: We reported a 52-year-old, diabetic and hypertensive male, presented with acute onset, first ever, severe temporal headache with left eye ptosis, diplopia and opthalmoplegia. Neurologic examination revealed left oculomotor and trochlear nerve palsy with sparring of pupillary function. Examination of blood D-dimer and C-reactive were normal. Head contrast MRI and MR Angiography revealed no lesion. He was diagnosed with diabetic cranial neuropathy after exclusion of other possible etiology. CONCLUSION: Simultaneous involvement of two or more cranial nerve neuropathy in diabetic patient is not common and need further investigation. Exclusion of the other possible diagnosis is important to diagnose diabetic cranial neuropathy.

A CASE OF PANCOAST SYNDROME IN TB

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Tuberculosis is one of the most common systemic infectious disease, and a major public health problem all over the world. It has several forms of presentation and clinical manifestations. Here we present an interesting clinical manifestation of Tuberculosis as Pancoast Syndrome. CASE PRESENTATION: A 55 year old male, smoker for past 30 years, presented with cough with expectoration, left shoulder pain and weakness of left upper limb of 6 months duration. He had irregular treatment for pulmonary tuberculosis two years ago. On examination he had partial ptosis of left eye, enophthalmos, miosis, ipsilateral anhydrosis, and tenderness in left supraclavicular region, wasting of left upper limb muscles. Sputum examination was positive for Acid fast bacilli. Cartridge based Nucleic Acid Amplification Test (CBNAAT) of sputum confirmed Rifampicin sensitive Mycobacterium Tuberculosis. Chest radiograph and CT Chest showed fibrocavitary changes, Cavitary consolidations predominantly involving right lung and mass like lesion in left apical lobe with erosion of left first rib. Histopathological examination with CT guided biopsy of the mass like lesion was negative for malignancy but showed caseous granulomatous inflammation. DISCUSSION: Pancoast syndrome1 refers to a constellation of symptoms of Brachial Neuralgia, Horner’s syndrome and atrophy of muscles of ipsilateral upper limb. Pancoast syndrome is commonly associated with Pancoast tumor of Squamous cell carcinoma , recently adenocarcinoma has emerged as commonest. Other conditions that may result in Pancoast syndrome are (1) Other tumors - primary chest wall and metastatic tumors, pleural mesothelioma, hematologic malignancies, (2) infectious and inflammatory lung diseases – tuberculosis, aspergillosis, echinococcosis, actinomyces and lung abscesses, inflammatory pseudotumor, amyloid nodules and (3) vascular lesions - carotid pseudoaneurysm, hemangioma.These conditions can invade and destruct the apical bony skeleton of the thorax, producing similar clinical and radiographic picture. CONCLUSIONS: We conclude that clinicians need to be aware of the variable manifestations of Tuberculosis. Reference #1: Pancoast HK. Superior pulmonary sulcus tumor: tumor characterized by pain Horner’s syndrome, destruction of bone and atrophy of hand muscles. JAMA 1932;19:1391-6. Reference #2: White HD, White BA, Boethel C, et al. Pancoast’s syndrome secondary to infectious etiologies: a not so uncommon occurrence. Am J Med Sci 2011;341:333-6 Reference #3: Beshay M, Roth T, Stein RM, et al. Tuberculosis presenting as Pancoast tumor. Ann Thorac Surg 2003;76:1733-5 DISCLOSURES: No relevant relationships by ALLWYN VIJAY GOWRI SHANKAR, source=Web Response No relevant relationships by Sagindar Samaraj, source=Web Response

Meatal Anterior Inferior Cerebellar Artery Aneurysm with AICA-PICA Complex: Endovascular Coiling and Parent Artery Preservation

AbstractSecondary stroke prevention requires early initiation of antiplatelet; therefore, stroke mimics need to be ruled out particularly in circumstances when antiplatelet therapy can be of disastrous consequences. A 54-year-old female patient presented to the emergency department with symptoms of sudden-onset deviation of angle of mouth to the right side, left eye ptosis, and occipital headache for past 4-hour duration. Neurologic examination revealed right-sided gaze-dependent torsional nystagmus and left lower motor neuron facial weakness. An embolic posterior circulation stroke secondary to vertebral artery dissection was suspected. Diffusion-weighted imaging (DWI) did not show any acute infarcts, and careful review of susceptibility-weighted imaging (SWI) scans showed hemorrhage in the fourth ventricle. Subsequent digital subtraction angiography (DSA) was done, which showed left anterior inferior cerebellar artery (AICA) aneurysm involving its intrameatal segment with AICA-posterior inferior cerebellar artery (PICA) complex. Retrospective review of computed tomographic (CT) angiography images showed small aneurysm in the internal auditory meatus, which is difficult to discern secondary to adjacent bony structure and smaller size of the aneurysm. The patient underwent endovascular coiling of the aneurysm with preservation of the parent artery. Our experience concluded that these clinical features suggest remote subarachnoid hemorrhage secondary to the ruptured of AICA intrameatal segment aneurysm with left facial nerve paralysis and peripheral cochlear vestibular changes secondary to either compression (mechanical or pulsations of the aneurysm sac) or ischemia of vestibular apparatus. The neurointerventionist should consider the possibility of aneurysmal rupture, especially in cases of atypical location of hemorrhage and no signs of infarct on neuroimaging of posterior circulation stroke.

SUN-432 A Case of Sellar Plasmacytoma Masquerading as Pituitary Carcinoma with Hypopituitarism

Background: Sellar plasmacytomas are rare tumors arising from clonal plasma cells localized to the sellar/parasellar region. They may mimic other pituitary pathology such as pituitary adenomas, chordomas, meningiomas or metastatic carcinomas in both clinical manifestations and radiographic evidence. Certain features such as predominance of cranial nerve palsies and absence of hypopituitarism appear to be more common with sellar plasmacytomas. We present an interesting case of a sellar plasmacytoma, initially mistaken for pituitary carcinoma, with rapid development of hypopituitarism and systemic multiple myeloma. Case: A 75 year old healthy male presented with sudden onset left eye diplopia and retro-orbital pain. Left eye ptosis and diplopia with left lateral and upward gaze were noted on exam. Initial MRI brain revealed a 17mm soft tissue mass involving pituitary and clivus, extending into the left superior orbital fissure, concerning for pituitary carcinoma. With the exception of low LH (1.2) and testosterone (25), baseline pituitary hormone labs, complete blood count and metabolic panel were within normal limits. Within 1 month, he acutely presented with fever, confusion, weakness, and bony pains. Repeat pituitary labs showed low ACTH < 5, AM cortisol 4.3, TSH 0.07, FT4 0.5. New findings of acute renal injury, hypercalcemia, anemia with elevated Mspike, Kappa light chain 39.7 (0.3-2.0mg/dl), kappa/lamda ratio 66.2 (0.3-1.6) and beta2microglobulin 4484 were found. Repeat MRI brain noted multifocal osseous metastatic disease with left sellar and cavernous sinus lesion contiguous with clival osseous lesion displacing pituitary gland, concerning for plasmacytoma. PET/CT showed focal lytic lesions in clivus and posterior left iliac bone. Bone marrow biopsy confirmed 70% myeloma involvement with kappa monotypic plasma cell population. He was started on hydrocortisone, levothyroxine, chemotherapy and XRT. Conclusion: The rarity of plasmacytomas, combined with their clinical presentation and imaging characteristics similar to other sellar tumors, can often result in misdiagnosis. Accuracy of diagnosis is critical, as sellar plasmacytomas and underlying multiple myeloma (present in 50% of cases) require XRT and systemic chemotherapy respectively rather than surgery. Although pituitary function is usually preserved in plasmacytomas, our case shows that acute anterior pituitary dysfunction can be an early presenting sign.

SUN-596 A Rare Case of Metastatic Medullary Thyroid Carcinoma to Pituitary Gland

Background: Metastasis to the hypothalamus & pituitary gland account for 1-2% of sellar masses. Pituitary metastasis of medullary thyroid carcinoma (MTC) accounts for only 6 published cases in the literature. Imaging alone cannot diagnose metastasis & requires resection for histochemical identification. We describe a case of metastatic MTC presenting with 3rd nerve palsy & pituitary lesion. Case Presentation: 63 y.o. man with 1-month history of bilateral retro-orbital headache, diplopia & left eye ptosis presented to the hospital for second evaluation. He had history of MTC (5 x 3 x 2.5 cm), h/o thyroidectomy 5 years prior & known metastatic non-progressive lung lesions on CT. Medications included levothyroxine & citalopram. He had no personal or family history of MEN syndromes. 2 weeks prior he went to another hospital for same complaints. Initial MRI of head found a parasellar 1.6 cm mass extending to sella. Neurosurgery did not recommend intervention at that time. At our hospital, dedicated pituitary MRI this time showed a primary intrasellar lesion displacing normal pituitary gland, without bony erosion, favoring a pituitary macroadenoma with bilateral cavernous sinus invasion. No optic optic chiasm involvement was noted. Calcitonin level was 2582 pg/mL (1580 pg/mL 10 months ago), CEA 17.0 ng/mL [<3.0 ng/mL]. Hormonal workup did not indicate functional adenoma - instead showed partial hypopituitarism, with LH 1.0 mIU/mL [1.24-8.62 mIU/mL], FSH 2.90 mIU/mL, am free testosterone 7.9 pg/mL [35 - 155 pg/mL], total testosterone 522 ng/dL [250-1100 ng/dL], TSH 0.059 [ 0.28-3.89 uIU/mL], free T4 0.72 ng/dL [0.58-1.64 ng/dL], IGF-1 230 [41-279 ng/mL], am cortisol of 8.4 mcg/dL [6.7-22.7 mcg/dL]. Ophthalmology consult revealed CN III palsy with left ptosis, without visual field deficits, indicating mass compression of CN III. Due to persistent headache, 2nd neurosurgical opinion was sought & transphenoidal resection performed. Initial frozen section suggested pituitary adenoma. Gross pathology demonstrated calcitonin, chromogranin A & synaptophysin immunoreactive cells consistent with metastatic MTC. Post operative MRI demonstrated only small residual right sellar & cavernous component. Daily local conventional fractionated radiation was started with Rad/Onc. Tyrosine kinase inhibitor therapy will be considered after post-radiation MRI & genetic testing for RET mutations. Discussion: Our patient’s clinical presentation of severe headache & rapid onset of diplopia was not consistent with pituitary adenoma, and the MRI result was a confounder. Conclusion: Although pituitary metastasis is rare, it should be considered in the differential in the appropriate clinical context. For MTC, active surveillance with imaging and biochemical markers is critical for management. Doubling time of calcitonin <1 year and rising CEA levels should prompt suspicion for disease progression.

A TYPICAL CASE OF CHILDHOOD OCULAR MYASTHENIA GRAVIS: A LITERATURE REVIEW FROM OPHTHALMOLOGY PERSPECTIVE

ABSTRACT Background: Childhood ocular myasthenia gravis (MG) is a rare autoimmune disease that has a benign course and provide a good overall prognosis. Diagnosis of ocular MG especially in children is often difficult especially in younger age group as the symptoms may be subtle and may mimic other pathology. There are many articles discussing MG in general, but only a handful elaborating on childhood ocular MG. This article is discussing a typical case of childhood MG and a review of current clinical approach particularly from ophthalmology point of view. Case Report: A one-year-old Malay girl who was previously well, presented with 2 weeks history of left eye ptosis and intermittent exotropia following a brief episode of fever. A provisional diagnosis of ocular MG was made. Unfortunately, the patient defaulted pediatric neurology follow up for confirmatory investigations. She came back 2 years later with bilateral ptosis. Upon her second presentation, blood investigation for acetylcholine receptor antibody showed positive result and finally she was diagnosed to have ocular MG. She was co-managed with a pediatric neurologist and was treated with oral pyridostigmine. Satisfactory improvement of ptosis was achieved with no amblyopia. There was no features of systemic MG during 2 years follow up. Conclusion: Although this is a typical presentation of childhood ocular MG, there are many interesting and useful learning points particularly from an ophthalmology point of view.

Sepsis + ptosis = an unusual diagnosis

The authors present the case of a 19-year old man presenting to the emergency department with a 2-day history of left eye pain and ptosis, facial pain around the maxillae,...

Rare Coexistence of Myasthenia Gravis and Lambert-Eaton Myasthenic Syndrome with Adenocarcinoma of the Bladder: A Case Report

Abstract Introduction: The coexistence of myasthenia gravis and Lambert-Eaton myasthenic syndrome (LEMS) is very rare.Case report: Herein we report an 82 year old man a known case of adenocarcinoma of bladder who came with ptosis in left eye, diplopia, and dysphagia, weakness of both lower extremities and autonomic dysfunction. According to history, physical examination and para clinical work ups coexisting of myasthenia gravis and LEMS was diagnosed.Discussion: our patient is the first patient with Overlap myasthenic syndrome on the base of adenocarcinoma of bladder. The purpose of this case report is to emphasize on this fact that a patient with LEMS should be evaluate carefully for detecting underlying malignancies. Although in the most cases the origin is lung, extra pulmonary origin such as urinary system should be evaluate carefully too.

Reversible and unilateral corticospinal tract disease secondary to autoimmune free-T3-thyrotoxicosis

68-year-old female patient with no significant medical history presents with a 3-month history of progressive neurological symptoms, which began with left eye ptosis, blurred vision and non-painful jaw discomfort, followed...

Isolated third nerve palsy: a rare neurological presentation of Burkitt's lymphoma

Lymphoma patient presenting with isolated third nerve palsy is relatively rare, and diagnosis of underlying disease may be challenging. Until this date, less than 20 cases have been described in...

P.025 Magnetic resonance imaging in pediatric recurrent ophthalmoplegic cranial neuropathy

Background: Recurrent ophthalmoplegic cranial neuropathy (ROCN), previously called ophthalmoplegic migraine, is characterized by recurrent episodes of headache and ophthalmoplegia of unclear etiology. Characteristic neuroimaging findings can support the diagnosis. Methods: A case report and review of the literature. Results: We present a 6-year-old girl with a past history of migraine headaches associated with retroorbital pain since 4 years of age. Family history is positive for migraine. She presented with a half a day history of left eye ptosis, 10 days post a resolved gastroenteritis which was associated with headaches. Examination showed only a left eye ptosis, pupil-sparing with no exotropia or diplopia. There was no headache. The rest of the neurologic examination was normal. Investigations showed normal blood tests and lumbar puncture. MRI head showed on thin cuts asymmetric nodular thickening (4mm) of the origin of the cisternal segment of the left oculomotor nerve, with corresponding homogeneous enhancement post gadolinium infusion. Clinical resolution occurred spontaneously within 48 hours. A review of the literature highlights focal thickening and enhancement of the affected cranial nerve, with resolution of enhancement post-acute phase. Conclusions: Focal thickening and enhancement of affected cranial nerve is seen in the majority of pediatric ROCN. These findings are best seen with thin MRI cuts and gadolinium infusion.

Viral mononeurities causing partial oculomotor nerve palsy in an 8 month old child.

BACKGROUND:Palsy of the oculomotor nerve is rarely seen in children, and comprehensive guidelines for management and outcome are difficult to find.CASE:Here we describe a 8 month old boy with left sided ptosis and infero-leteral deviation of left eye and normally reacting pupil and CSF antimeasles antibody titre negative.OUTCOME:The ptosis improved within 3 weeks with no residual neurodeficit.MESSAGE:Viral mononeuritis leading to partial 3rd nerve palsy is a rare entity in children till date.

Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type Involving Skin Masquerading as Eczema

Dear Editor: Extranodal natural killer (NK)/T-cell lymphoma, nasal type (ENKTL) is an uncommon type of lymphoma that is endemic to East Asia and parts of Central and South America1. Most of the patients clinically present with nasal obstruction, sinusitis, ulcer, and epistaxis due to a destructive mass involving the midline facial tissues and skin is the second most commonly affected organ after nasal area1. Descriptions of cutaneous manifestations of ENKTL are of well-circumscribed lesions such as nodules/tumors, plaques and ulcers. Other atypical, various clinical morphologies have been reported including papules, cysts, ulcers, and cellulitis2. ENKTL presenting in the skin is highly aggressive with a mean survival of less than 12 months3. We report a case of extranodal NK/T-cell lymphoma, nasal type who presented with lesions clinically similar to eczema. A 63-year-old woman visited with pruritic erythematous papules and plaques with areas of postinflammatory hyperpigmentation on right upper back, left breast, and left thigh which persisted for 6 weeks (Fig. 1A~C). Under the clinical impression of eczema and urticarial dermatitis, she was treated with oral antihistamines and topical steroids ointment for 2 weeks. Despite treatment the lesions persisted and therefore skin biopsy was done on erythematous plaque of right upper back. Histopathologic findings revealed mixed atypical lymphoid cells and histiocytic cells along superficial and deep perivascular area (Fig. 2A). Lymphocytic infiltration showed perivascular pattern, with pale cytoplasm and dense chromatin with irregularly shaped nuclei (Fig. 2B). Immunohistochemical study showed CD3 and CD4 positivity in majority of lymphoid cells (Fig. 2C), focal positivity in CD8, CD30, CD56 (Fig. 2D) strong and profuse positivity in in situ hybridization for Epstein-Barr virus (EBV) (Fig. 2E). The patient was informed to visit immediately, however, did not visit within 2 weeks of notification. Five weeks after the initial visit the patient presented with left eye ptosis and swelling of left eyelid and mandibular area. Under the impression of extranodal NK/T-cell lymphoma, nasal type, the patient was transferred to hemato-oncology department. Fig. 1 Erythematous papules and plaques with areas of brownish hyperpigmentation on (A), (B) right upper back and (C) left thigh. Fig. 2 (A) Mixed atypical lymphoid cells and histiocytic cells along superficial and deep perivascular area (H&E, ×40). (B) Lymphocytic infiltration in perivascular pattern, with pale cytoplasm and dense chromatin with irregularly shaped nuclei ... The NK/T-cell lymphomas are classified into 2 subtypes, nasal and non-nasal NK/T-cell lymphomas. The non-nasal group can be further subdivided into primary cutaneous and 4 types of secondary cutaneous lymphomas: nasal-type, aggressive, blastoid, and other specific lymphoma types4,5. Nasal-type NK/T-cell lymphoma is the most common subtype among the secondary cutaneous non-nasal NK/T-cell lymphomas5. The skin is the most common extranodal site of involvement followed by the soft tissues, and could be either primary or secondary feature of the disease3. The new sites of involvement are also mostly extranodal, and are similar to the predilection sites at presentation4. Extracutaneous involvement at the time of presentation is associated with worse prognosis3. In a patient with known ENKTL, a skin biopsy should be obtained from any suspicious clinical lesion to assess for possible cutaneous involvement. Furthermore, a simple erythematous patch may be the initial presenting manifestation of the disease. In conclusion, we report a case which stresses the importance of awareness of malignancy and prompt skin biopsy in patients with erythematous papules and plaques that fail to respond to traditional management.