The most cases with orbital metastases have been reported in patients with a prior established diagnosis of cancer and widespread systemic involvement. However, ocular symptoms can be developed as an initial presentation of cancer in patients without cancer history. We report a case of rapid progression from trochlear nerve palsy to orbital apex syndrome as an initial presentation of advanced gastric cancer.
Diagnosis ; Diplopia ; Humans ; Neoplasm Metastasis ; Optic Nerve Diseases ; Orbit ; Stomach Neoplasms ; Trochlear Nerve Diseases ; Trochlear Nerve

PURPOSE: To present a case of orbital inflammation and optic perineuritis preceding vesicular eruption in herpes zoster ophthalmicus(HZO). CASE SUMMARY: An 84-year-old woman with a history of gall bladder cancer and hypertension complained of left periorbital erythematous edema and discomfort. On examination, visual acuity was 20/25 bilaterally; no tenderness, proptosis or ophthalmoplegia was observed. Pupils were equal, round, and reactive to light without relative afferent pupillary defects. Slit-lamp examination revealed severe conjunctival injection and chemosis without keratitis or uveitis. The remainder of the ocular examination was unremarkable. Magnetic resonance imaging confirmed left-sided preseptal swelling with an enlarged left lacrimal gland, high signal intensity of the retrobulbar fat and optic nerve sheath. Systemic antibiotic therapy with steroids was started under a presumed diagnosis of idiopathic orbital inflammatory disease, but the clinical presentation was unresolved. After 2 days, vesicular lesions confined to the first division of the trigeminal nerve and pseudodendritic keratitis developed on the left side leading to a diagnosis of HZO. Treatment with acyclovir immediately resolved anterior segment inflammation and periorbital edema. While on therapy, visual acuity deteriorated to 20/125 and the pupil became dilated and unresponsive to light over a few days. All signs and symptoms of acute orbitopathy and postherpetic neuralgia had resolved 3 months later with the exception of pupil abnormality and visual acuity. CONCLUSIONS: HZO may present with symptoms and signs of orbital inflammation and optic perineuritis even in the absence of a vesicular rash. Thus, HZO should be considered in the differential diagnosis of unexplained acute orbital syndromes.
Acyclovir ; Aged, 80 and over ; Dacryocystitis ; Diagnosis ; Diagnosis, Differential ; Edema ; Exanthema ; Exophthalmos ; Female ; Gallbladder Neoplasms ; Herpes Zoster Ophthalmicus* ; Herpes Zoster* ; Humans ; Hypertension ; Inflammation* ; Keratitis ; Lacrimal Apparatus ; Magnetic Resonance Imaging ; Neuralgia, Postherpetic ; Ophthalmoplegia ; Optic Nerve ; Orbit* ; Pupil ; Pupil Disorders ; Steroids ; Trigeminal Nerve ; Uveitis ; Visual Acuity

Pilocytic astrocytomas (PAs) are World Heath Organization Grade I tumors and are most common in children. PA calcification is not a common finding and has been reported more frequently in the optic nerve, hypothalamic/thalamus and superficially located cerebral tumors. We present a cerebellar PA in a 3-year-old male patient with cystic components and massive calcification areas. The residual tumor grew rapidly after the first operation, and the patient was operated on again. A histopathological examination revealed polar spongioblastoma-like cells. Massive calcification is not a common feature in PAs and can lead to difficulties in radiological and pathological differential diagnoses.
Astrocytoma* ; Child ; Child, Preschool ; Diagnosis, Differential ; Humans ; Male ; Neoplasm, Residual ; Neoplasms, Neuroepithelial ; Optic Nerve ; Recurrence*

PURPOSE: Diencephalic syndrome is an uncommon cause of failure to thrive in early childhood that is associated with central nervous system neoplasms in the hypothalamic-optic chiasmatic region. It is characterized by complex signs and symptoms related to hypothalamic dysfunction; such nonspecific clinical features may delay diagnosis of the brain tumor. In this study, we analyzed a series of cases in order to define characteristic features of diencephalic syndrome. METHODS: We performed a retrospective study of 8 patients with diencephalic syndrome (age, 5-38 months). All cases had presented to Seoul National University Children's Hospital between 1995 and 2013, with the chief complaint of poor weight gain. RESULTS: Diencephalic syndrome with central nervous system (CNS) neoplasm was identified in 8 patients. The mean age at which symptoms were noted was 18+/-10.5 months, and diagnosis after symptom onset was made at the mean age of 11+/-9.7 months. The mean z score was -3.15+/-1.14 for weight, -0.12+/-1.05 for height, 1.01+/-1.58 for head circumference, and -1.76+/-1.97 for weight-for-height. Clinical features included failure to thrive (n=8), hydrocephalus (n=5), recurrent vomiting (n=5), strabismus (n=2), developmental delay (n=2), hyperactivity (n=1), nystagmus (n=1), and diarrhea (n=1). On follow-up evaluation, 3 patients showed improvement and remained in stable remission, 2 patients were still receiving chemotherapy, and 3 patients were discharged for palliative care. CONCLUSION: Diencephalic syndrome is a rare cause of failure to thrive, and diagnosis is frequently delayed. Thus, it is important to consider the possibility of a CNS neoplasm as a cause of failure to thrive and to ensure early diagnosis.
Astrocytoma ; Brain Neoplasms ; Central Nervous System ; Central Nervous System Neoplasms ; Diagnosis ; Diarrhea ; Drug Therapy ; Early Diagnosis ; Failure to Thrive* ; Follow-Up Studies ; Head ; Humans ; Hydrocephalus ; Hypothalamic Diseases ; Infant* ; Optic Nerve Glioma ; Palliative Care ; Retrospective Studies ; Seoul ; Strabismus ; Vomiting ; Weight Gain

No abstract available.
Diagnosis, Differential ; Female ; Humans ; Middle Aged ; Optic Disk Drusen/*diagnosis ; Optic Nerve Neoplasms/*diagnosis ; Tomography, Optical Coherence

No abstract available.
Diagnosis, Differential ; Female ; Humans ; Middle Aged ; Optic Disk Drusen/*diagnosis ; Optic Nerve Neoplasms/*diagnosis ; Tomography, Optical Coherence

PURPOSE: To report a case of subacute optic neuropathy caused by thiamine (vitamin B1) deficiency. CASE SUMMARY: A 44-year-old woman was referred to the ophthalmology department due to decreased vision which began 10 days prior to presentation. The patient history indicated that she had undergone chemotherapy for ovarian cancer and she had been dependent on total parenteral nutrition for 3 weeks due to nausea and vomiting. Her best corrected vision of the right and the left eyes were 0.15 and 0.2, respectively. Color vision was severely impaired in both eyes without retrobulbar pain. There was marginal blurring on the temporal side of the optic discs of both eyes. The optic nerves were unremarkable on orbital and brain magnetic resonance imaging (MRI). There was high signal intensities in both the mammillary body and periaqueductal gray matter on T2-weighted imaging. In addition, the patient exhibited ataxia along with short-term memory loss. She was diagnosed with Wernicke's encephalopathy. Thiamine was administrated based on the diagnosis, and after 2 days of administration, the patient's vision and neurologic symptoms began to improve. Two weeks later, the patient recovered her vision. CONCLUSIONS: Thiamine deficiency may cause optic neuropathy and can be recovered with early thiamine supplementation. This condition could occur due to deficient oral thiamine administration. We should keep this point in mind and try to prevent or diagnose early.
Adult ; Ataxia ; Brain ; Color Vision ; Diagnosis ; Drug Therapy ; Female ; Humans ; Magnetic Resonance Imaging ; Mamillary Bodies ; Memory, Short-Term ; Nausea ; Neurologic Manifestations ; Ophthalmology ; Optic Nerve ; Optic Nerve Diseases* ; Orbit ; Ovarian Neoplasms ; Parenteral Nutrition, Total ; Periaqueductal Gray ; Thiamine Deficiency ; Thiamine* ; Vomiting ; Wernicke Encephalopathy*

A 13-year-old girl with a right intraorbital optic nerve glioma (ONG) was referred to our glaucoma clinic because of uncontrolled intraocular pressure (IOP) in her right eye. The IOP reached as high as 80 mmHg. Several months earlier, she had undergone stereotactic image-guided robotic radiosurgery using the CyberKnife for her ONG; the mass had become smaller after treatment. Her visual acuity was no light perception. Slit lamp examination revealed rubeosis iridis, a swollen pale optic disc, and vitreous hemorrhage. After medical treatment, the IOP decreased to 34 mmHg, and no pain was reported. Although the mass effect of an ONG can cause neovascular glaucoma (NVG), this case shows that stereotactic radiosurgery may also cause NVG, even after reducing the mass of the tumor. Patients who undergo radiosurgery targeting the periocular area should be followed carefully for complications.
Adolescent ; Diagnosis, Differential ; Female ; Fluorescein Angiography ; Follow-Up Studies ; Fundus Oculi ; Glaucoma, Neovascular/diagnosis/*etiology/physiopathology ; Glioma/diagnosis/*surgery ; Gonioscopy ; Humans ; Intraocular Pressure ; Magnetic Resonance Imaging ; Optic Nerve Neoplasms/diagnosis/*surgery ; Radiosurgery/*adverse effects

Pituitary apoplexy is a clinical syndrome caused by an acute ischemic or hemorrhagic vascular accident involving a pituitary adenoma or an adjacent pituitary gland. Pituitary apoplexy may be associated with a variety of neurological and endocrinological signs and symptoms. However, isolated third cranial nerve palsy with ptosis as the presenting sign of pituitary apoplexy is very rare. We describe two cases of pituitary apoplexy presenting as sudden-onset unilateral ptosis and diplopia. In one case, brain magnetic resonance imaging (MRI) revealed a mass in the pituitary fossa with signs of hemorrhage, upward displacement of the optic chiasm, erosion of the sellar floor and invasion of the right cavernous sinus. In the other case, MRI showed a large area of insufficient enhancement in the anterior pituitary consistent with pituitary infarction or Sheehan's syndrome. We performed neurosurgical decompression via a transsphenoidal approach. Both patients showed an uneventful recovery. Both cases of isolated third cranial nerve palsy with ptosis completely resolved during the early postoperative period. We suggest that pituitary apoplexy should be included in the differential diagnosis of patients presenting with isolated third cranial nerve palsy with ptosis and that prompt neurosurgical decompression should be considered for the preservation of third cranial nerve function.
Brain ; Cavernous Sinus ; Decompression ; Diagnosis, Differential ; Diplopia ; Displacement (Psychology) ; Floors and Floorcoverings ; Hemorrhage ; Humans ; Hypopituitarism ; Infarction ; Magnetic Resonance Imaging ; Oculomotor Nerve ; Optic Chiasm ; Paralysis ; Pituitary Apoplexy ; Pituitary Gland ; Pituitary Neoplasms ; Postoperative Period

OBJECTIVETo study the clinicopathologic features of pilomyxoid astrocytoma (PmA).

METHODSThe clinical and pathologic features in 3 cases of PmA were analyzed. Immunohistochemical study for glial fibrillary acidic protein (GFAP), CD34 and Ki-67 was performed on paraffin-embedded sections by standard EnVision method.

RESULTSAll the 3 cases occurred in female patients at the age of 10 months, 10 years and 19 years respectively. Two cases were located in the third ventricle, while the remaining case was located at the optic pathway. Histologically, the tumor was composed of bipolar spindle cells setting in a strikingly mucinous background. There was a marked proliferation of vessels within the tumor. In some areas, the tumor cells exhibited an angiocentric growth pattern. The biphasic pattern noted in a classic pilocytic astrocytoma was not found in PmA. Rosenthal fibers and eosinophilic granular bodies were also not identified. Immunohistochemcal study showed that the tumor cells were diffusely positive for GFAP. The Ki-67 labeling index measured less than 1%. CD34 highlighted mainly the vascular networks.

CONCLUSIONSPmA is a distinctive variant of pilocytic astrocytoma with subtle histologic differences. Compared with conventional pilocytic astrocytoma, PmA behaves more aggressively. Some cases tend to occur in older children and adolescents. Immunohistochemical study for GFAP is helpful in differential diagnosis.

Antigens, CD34 ; metabolism ; Astrocytoma ; metabolism ; pathology ; surgery ; Cerebral Ventricle Neoplasms ; metabolism ; pathology ; surgery ; Child ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Immunohistochemistry ; Infant ; Ki-67 Antigen ; metabolism ; Magnetic Resonance Imaging ; Optic Nerve Glioma ; metabolism ; pathology ; surgery ; Third Ventricle ; metabolism ; pathology ; Tomography, X-Ray Computed ; Young Adult