Bilateral Optic Disc Swelling Research Articles

Transient binocular vision loss and pain insensitivity in Klippel–Feil syndrome: a case report

BackgroundKlippel–Feil syndrome is a rare congenital bone disorder characterized by an abnormal fusion of two or more cervical spine vertebrae. Individuals with Klippel–Feil syndrome exhibit diverse clinical manifestations, including skeletal irregularities, visual and hearing impairments, orofacial anomalies, and anomalies in various internal organs, such as the heart, kidneys, genitourinary system, and nervous system.Case presentationThis case report describes a 12-year-old Pashtun female patient who presented with acute bilateral visual loss. The patient had Klippel–Feil syndrome, with the typical clinical triad symptoms of Klippel–Feil syndrome, along with Sprengel’s deformity. She also exhibited generalized hypoalgesia, which had previously resulted in widespread burn-related injuries. Upon examination, bilateral optic disc swelling was observed, but intracranial pressure was found to be normal. Extensive investigations yielded normal results, except for hypocalcemia and low vitamin D levels, while parathyroid function remained within the normal range. Visual acuity improved following 2 months of calcium and vitamin D supplementation, suggesting that the visual loss and optic nerve swelling were attributed to hypocalcemia. Given the normal parathyroid function, it is possible that hypocalcemia resulted from low vitamin D levels, which can occur after severe burn scarring. Furthermore, the patient received a provisional diagnosis of congenital insensitivity to pain on the basis of the detailed medical history and the findings of severe and widespread loss of the ability to perceive painful stimuli, as well as impaired temperature sensation. However, due to limitations in genetic testing, confirmation of the congenital insensitivity to pain diagnosis could not be obtained.ConclusionThis case highlights a rare presentation of transient binocular vision loss and pain insensitivity in a patient with Klippel–Feil syndrome, emphasizing the importance of considering unusual associations in symptom interpretation.

Bilateral optic neuritis and encephalopathy as the atypical presentations of multiple sclerosis following severe acute respiratory syndrome coronavirus 2 infection

Abstract: Numerous evidence suggests coronavirus disease 2019 (COVID-19) potentially triggers demyelinating diseases, inclusive of multiple sclerosis (MS), and acute disseminated encephalomyelitis (ADEM), and various mechanisms have been proposed. We report a 42-year-old male presented with bilateral optic neuritis and encephalopathy, 2 weeks following COVID-19 infection. He denied any history or family history of neurological and ocular diseases. Severe bilateral visual impairment (only light perception) and pain with eye movement were reported. Fundoscopy revealed bilateral optic disc swelling. Magnetic resonance imaging showed tortuous bilateral optic nerves with optic nerve and nerve sheath enhancement. Multiple hyperintense nodules in bilateral cerebral white matter were noted on fluid-attenuated inversion recovery T2-weighted imaging without diffusion restriction or gadolinium contrast enhancement. Hypointense nodules in cerebral white matter were also noted on T1-weighted imaging, which implied some old lesions. Dissemination in space and time and cerebrospinal fluid-specific oligoclonal bands confirmed the diagnosis of MS. Both serum aquaporin-4 and myelin oligodendrocyte glycoprotein antibodies were negative. He received pulse steroid therapy for 5 days, followed by slowly tapering oral prednisolone. His vision, ocular motion pain, and encephalopathy improved gradually. However, the visual outcome was still poor (bilateral 20/400), and optic atrophy was noticed during 1-year follow-up. To our knowledge, this is the first case of MS following severe acute respiratory syndrome coronavirus 2 infection presented with bilateral optic neuritis and encephalopathy. Since these manifestations are exceedingly rare in MS, we suspect acute immune reactions induced by COVID-19 could bring about the atypical ADEM-like presentations of MS.

An analysis of the etiology composition and clinical characteristics of bilateral optic disc swelling

Objective: To investigate the etiology composition and clinical characteristics of bilateral optic disc swelling(ODS). Methods: The medical records of all newly diagnosed bilateral ODS patients admitted to the neurology ward of Beijing Tongren Hospital from January 2017 to June 2021 were retrospectively searched to classify the etiology, obtain demographic and clinical information, and compare the differences in clinical characteristics. Results: A total of 131 patients with bilateral ODS were included, including 56 males and 75 females, aged 15-73 (39±14) years. The most common cause of the bilateral ODS was increased intracranial pressure (ICP)(56/131, 42.7%), followed by optic neuritis (ON)(40/131, 30.5%). Other causes included vascular optic neuropathy (13/131, 9.9%), pseudopilledema (9/131, 6.9%); uveitis (6/131, 4.6%), toxic optic neuropathy (3/131, 2.3%) and unknown causes (4/131, 3.1%). Idiopathic intracranial hypertension (IIH) (43/56, 76.8%) was the most common etiology for papilledema. In the comparison of the two main causes of intracranial hypertension and the clinical characteristics of ON in ODS, there was no statistically significant difference in the incidence of age, gender, complaints of ocular pain or headache, and hemorrhage of optic disc(P>0.05). Visual acuity abnormalities and low vision were more common in ON group than the increased ICP group[36/40(90%) vs 33/56(58.9%), P=0.001; 35/80(43.8%) vs 22/112(19.6%), P<0.001], while severe papilledema was more common in increased ICP group[38/112(33.9%)vs 9/80(11.3%), P<0.001]. Conclusions: The most common cause for bilateral ODS is increased ICP, but it can also be triggered by a variety of other causes. Optic neuritis(ON) is the most important differentiating disease in the study of Chinese patients.

Bilateral optic disc swelling in a man aged 32 years

Bilateral optic disc swelling in a man aged 32 years

Vogt-Koyanagi-Harada Disease after COVID-19 Vaccination

Purpose: To report a case of Vogt-Koyanagi-Harada (VKH) disease after COVID-19 vaccination.Case summary: A 53-year-old female patient with no history of systemic disease or ocular surgery presented with bilateral decreased visual acuity, hyperemia, eye pain, and headache 1 month after COVID-19 vaccination (Pfizer-BioNTech). She initially presented 1 week after symptom onset. Her best-corrected visual acuity was 0.63 in the right eye and 1.0 in the left eye, and she had bilateral grade 2 anterior chamber reactions on slit-lamp examination. Fundus photography, optical coherence tomography, and fluorescein angiography revealed bilateral optic disc swelling, choroidal folds, multiple serous retinal detachments, and fluorescein leakage at posterior poles. She was diagnosed with VKH disease and treated with topical and systemic medications, which completely resolved her symptoms over 2 months.Conclusions: VKH disease can develop after COVID-19 vaccination in patients without any underlying disease. Therefore, possible side effects and treatments should be explained to patients receiving COVID-19 vaccination.

Case Report: Bilateral optic disc swelling in a patient with tuberculoma

BACKGROUND: In children, tuberculosis of the central nervous system most usually manifests as tubercular meningitis, hydrocephalus post-tubercular meningitis, and, in rare cases, space-occupying lesions known as tuberculoma. We address the diagnostic difficulty in an adult patient with mainly ocular symptoms who was diagnosed with tuberculoma. A 19-year-old young man came with painless bilateral blurring of vision. Bilateral optic disc swelling and bilateral sixth cranial nerve palsy were present. METHODS: Brain imaging revealed ring-enhancing lesions in the cerebellar vermis, left cerebellar hemisphere and left middle cerebral peduncle with surrounding extensive perilesional edema causing effacement of fourth ventricle leading to obstructive hydrocephalus suggestive of tuberculoma. RESULTS: He had a high erythrocyte sedimentation rate and a positive Mantoux test. His Quantiferon-TB Gold test result was positive. The examination of cerebrospinal fluid suggested a high opening pressure. Following anti-tubercular medication along with systemic steroids, his vision improved due to decreased optic disc swelling. CONCLUSION: We wish to highlight that visual loss and grave consequences of tuberculoma can be averted with early recognition and treatment.

Paediatric MOG-antibody disease presenting with intracranial hypertension and unilateral vision loss without radiological evidence of optic neuritis

Intracranial hypertension (IH) is poorly described in paediatric myelin oligodendrocyte glycoprotein antibody disease (MOGAD). We describe a unique case of seropositive MOGAD in an obese 13-year-old boy who presented with an isolated IH, bilateral optic disc swelling and sudden-onset complete vision loss in one eye without radiological evidence of optic nerve involvement. Treatment with intravenous methylprednisolone combined with an emergency shunt fully restored vision and resolved the optic disc swelling. This report adds to the growing body of evidence suggesting that obese children presenting with isolated IH should be investigated for MOGAD, and the importance of managing IH during MOGAD.

A Case of Bilateral Optic Neuropathy Following COVID-19 Infection

Purpose: To report a case of bilateral optic neuropathy after COVID-19 infection.Case summary: Thirty-four-year-old woman presented with bilateral ocular pain, visual disturbance, and decreased visual acuity after contracting COVID-19 infection 16 days ago. At the initial visit, best-corrected visual acuity was 0.8 in the right eye, and 0.7 in the left eye. Left eye had relative afferent papillary defect and visual field tests revealed enlarged blind spot in both eyes. Fundus exam showed bilateral optic disc swelling and peripapillary retinal hemorrhage along with vitreous hemorrhage in the left eye. Fluorescein angiography revealed bilateral leakage of dye from the optic disc with area of hypofluorescence corresponding to retinal hemorrhage around the optic disc in both eyes. Brain magnetic resonance imaging (MRI) showed ill-defined left optic nerve enhancement. A diagnosis of bilateral optic neuropathy was made, and the patient was treated with high dose IV steroid 1 g per day for 3 days and oral steroid tapering for 2 weeks. Disc swelling and retinal hemorrhage were improved 1 month after treatment along with enhanced visual acuity to 1.0.Conclusions: Bilateral optic neuropathy following COVID-19 infection showed improvement after steroid treatment.

The Twilight Zone Where Margins Are Blurred: A Retrospective Analysis of Unilateral and Bilateral Optic Disc Swelling in a Tertiary Center in Malaysia.

Background To analyze the etiology of optic disc swelling (ODS) and compare the clinical features between non-arteritic anterior ischemic optic neuropathy (NA-AION) and optic neuritis (ON) at our center from January 2019 to January 2020. Methodology Clinical records of all patients who presented with ODS between January 2019 and January 2020 were reviewed. The collected data were analyzed using IBM SPSS Statistics for Windows (Version 23.0,IBM Corp., Armonk, NY, USA). Results A total of 70 eyes among 47 patients were included in this review. There were 24 patients (51%) who had unilateral ODS, while 23 patients (49.0%) had bilateral ODS. The most common etiology of unilateral ODS was ON (45.7%), NA-AION (25%), and neuroretinitis (12.5%). Among bilateral ODS, the most common cause was papilledema (30.4%), hypertensive retinopathy (21.7%), diabetic papillitis (13.1%), and optic disc drusen (13.1%). Among unilateral ODS, the mean onset age was significantly older in NA-AION cases than that in ON cases (57.5 years vs. 40.3 years, P= 0.016). Ocular pain was a significant clinical finding observed in ON cases (54.5%) and none in NA-AION cases (P= 0.043). Although there was no significant difference in the initial visual acuity (VA) between NA-AION and ON, the final VA was significantly improved in ON compared to NA-AION (0.31 ± 0.34 vs. 1.14 ± 1.00, P = 0.029). Conclusions Many differential diagnoses must be considered when a patient is presented with ODS. With the increased prevalence of hypertension and diabetes in Malaysia, it is not a surprise that both hypertensive retinopathy and diabetic papillitis together accounted for one-third of the bilateral ODS patients.

The Importance of Optical Coherence Tomography in the Diagnosis of Atypical or Subclinical Optic Neuritis: A Case Series Study

Background: Optic neuritis (ON) is an inflammatory condition of the optic nerve. ON is associated with development of demyelinating diseases of the central nervous system (CNS). CNS lesions visualized by magnetic resonance imaging (MRI) and the finding of oligoclonal IgG bands (OB) in the cerebrospinal fluid (CSF) are used to stratify the risk of MS after a "first" episode of ON. However, the diagnosis of ON in absence of typical clinical manifestations can be challenging. Methods and Materials: Here we present three cases with changes in the optic nerve and ganglion cell layer in the retina over the disease course. (1) A 34-year-old female with a history of migraine and hypertension had suspect amaurosis fugax (transient vision loss) in the right eye. This patient developed MS four years later. Optical coherence tomography (OCT) showed dynamic changes of the thickness of peripapillary retinal nerve fiber layer (RNFL) and macular ganglion cell-inner plexiform layer (GCIPL) over time. (2) A 29-year-old male with spastic hemiparesis and lesions in the spinal cord and brainstem. Six years later he showed bilateral subclinical ON identified using OCT, visual evoked potentials (VEP) and MRI. The patient fulfilled diagnosis criteria of seronegative neuromyelitis optica (NMO). (3) A 23-year-old female with overweight and headache had bilateral optic disc swelling. With OCT and lumbar puncture, idiopathic intracranial hypertension (IIH) was excluded. Further investigation showed positive antibody for myelin oligodendrocyte glycoprotein (MOG). Conclusions: These three cases illustrate the importance of using OCT to facilitate quick, objective and accurate diagnosis of atypical or subclinical ON, and thus proper therapy.

Clinical Profile of Patients with Bilateral Optic Disc Swelling Attending in an Apex Eye Hospital in Bangladesh

Background: Patient with optic disc swelling demands thorough ophthalmic as well as systemic evaluation as it may reflect intraocular as well as systemic diseases. Early diagnosis and proper management of bilateral disc swelling is important, as it can save vision as well as life. This study is aimed to determine the socio-demographic and clinical profile of patients with bilateral optic disc swelling in a tertiary eye hospital of Bangladesh.&#x0D; Methods: This cross sectional study had been conducted over 102 patients with bilateral disc swelling at the Neuroophthalmology clinic of National institute of ophthalmology and Hospital, Dhaka between April 2019 and March 2020. Patients were selected purposively based on specific selection criteria. Detail history was taken, ocular examinations like visual acuity, color vision, field of vision, pupillary light reaction, fundus examination were done and relevant investigations included color fundus photograph, visual field analysis, Computed tomography scans and Magnetic Resonance Imaging were performed. All the information’s were recorded in a pre-designed data collection sheet.&#x0D; Results: The mean age of the patients was 30.2±10.8 years where 37.3% patients were from 18-27 year age group. Females (67.7%) were commonly presented with bilateral disc swelling than male (33.3%). Most of the patients commonly presented with the symptoms like headache (71.6%), dimness of vision (62.8%), nausea &amp; vomiting (42.2%), ocular pain (21.6%) etc. Best corrected visual acuity of the study population was within 0-0.3 log MAR unit in the right eye in 55.9% cases and in the left eye in 59.8% cases. Majority of the patients (Right eye: 60.8%, Left eye: 63.7%) could read all plates while color vision assessment. About 20% patient had RAPD. About 17% patients had field defect in their right eyes whereas about 15% had visual filed defect in left eyes. Other than disc swelling, 14 patients had diplopia (13.7%), 12 patients had uveitis (11.8%). Out of 102 patients, 36 patients were clinically diagnosed as IIH (35.3%). Others were diagnosed as ICSOL (24.5%), bilateral optic neuritis (12.7%), VKH (12.7%), grade-IV hypertensive retinopathy (8.8%). The analysis of neuro imaging findings among the patients showed 72 patients (70.6%) had no space occupying lesions, 9 patients (8.8%) had meningioma, 6 patients had CP angle tumor (5.9%), 5 patients had empty sella (4.9%).&#x0D; Conclusions: Most of the Bangladeshi patients with bilateral disc swelling report to neuro-ophthalmology clinic in NIO&amp;H are female and belong to 18-37 years age group. Among them headache is the most common presenting complaint and IIH is the most common clinical diagnosis.&#x0D; J. Natl. Inst. Ophthalmol. 2022;5(1):39-45

The Clinical Profile of Unilateral and Bilateral Optic Disc Swelling in a Tertiary Center in Northern Malaysia.

Background Optic disc swelling (ODS) is a pathological condition with a variety of causes, including optic neuritis (ON), anterior ischemic optic neuropathy, and papilledema. Determining the causes of ODS is critical due to the possibilities of vision- or life-threatening diseases, such as space-occupying lesions. This study aimed to investigate the clinical profile of unilateral and bilateral ODS in Penang Hospital, Malaysia. Methodology This retrospective, descriptive study was conducted in Penang Hospital. Medical records of patients who were diagnosed with ODS from June 2018 until June 2020 in Penang Hospital Eye Emergency Clinic were reviewed. We excluded patients who defaulted on subsequent three months of follow up and those with pseudo-ODS. Results ODS was diagnosed in 43 patients who were all included in the study. Majority were females 55.8% (n = 24), with age ranging from 16 to 78 years. ON contributed most (41.9%, n = 18), followed by non-arteritic anterior ischemic optic neuropathy (NA-AION) (34.9%, n = 15), and papilledema (9.3%, n = 4). Other causes (14%, n = 6) included diabetic papillitis (n = 1), hypertensive retinopathy (n = 1), and central retinal vein occlusion (n = 4). Poor mean initial visual acuity was seen in patients with ON (1.07 ± 0.68) and NA-AION (1.33 ± 0.67). ON showed better final visual outcomes compared to NA-AION at the one-year follow-up. Conclusions ON and NA-AION were identified as the two most common causes of ODS in Penang Hospital for both unilateral and bilateral presentations. Most cases presented with poor initial visual acuity. After one year of follow-up, good visual recovery was seen in ON cases compared to other cases.These results were comparable with studies conducted in other Asian counties.

Bilateral Optic Disc Swelling in a Patient With Atypical Hemolytic Uremic Syndrome.

Bilateral Optic Disc Swelling in a Patient With Atypical Hemolytic Uremic Syndrome.

A 10-year-old girl with red eyes and hypothyroidism

Objective: Hypothyroidism has many causes and manifestations in children. One of the causes is autoimmunity, which is known as autoimmune thyroiditis or Hashimoto thyroiditis. Pseudotumor cerebri is a rare manifestation of Hashimoto thyroiditis. Here we report a 10-year-old girl with asymptomatic papillary edema who was treated with levothyroxine and acetazolamide. Case Presentation: A 10-year-old girl suffered from left eye trauma while playing volleyball and went to an ophthalmology center due to redness in the same eye. During the examination, they noticed a bilateral optic disc swelling without ocular inflammation and the other eye examination was within normal limits. Encephalopathy may rarely occur during autoimmune thyroiditis, which is known as Hashimoto encephalopathy, and it is stated that the autoimmune disorder is not related to the thyroid dysfunction characterized by the symptoms of decreased level of consciousness and seizures. Conclusion: The diagnosis of hypothyroidism in children is usually made by examining the thyroid-stimulating hormone (TSH), and T4 in which TSH is elevated and T4 is decreased as in our patient.

Malignant hypertension in a child - What lies beneath?

<br>We report a case of a 7-year-old girl presenting to the Neuro-Ophthalmology OPD with only complaints of chronic headache who was found to have malignant (grade 4) hypertensive retinopathy. Visual acuity was 20/400 in the right eye and 20/80 in the left eye. Blood pressure was 220/120 mm Hg. The patient was not a known hypertensive. Fundoscopy revealed bilateral optic disc swelling, macular stars, and serous retinal detachment in both eyes. The patient was urgently referred to pediatrician for detailed evaluation and systemic control of hypertension. This emphasizes the need for checking blood pressure in children presenting with chronic headache.<br>

Idiopathic intracranial hypertension presenting as iron deficiency anemia: a case report

BackgroundThe presentation of idiopathic intracranial hypertension (IIH) in association with iron deficiency anemia (IDA) is rare.Case presentationThis case report depicts the unusual case of a 31-year-old woman of mixed Jamaican and English heritage with IIH who presented initially as IDA in the context of menorrhagia. Subsequent ophthalmic review, lumbar puncture, cerebrospinal fluid analysis and neuroimaging studies revealed severe bilateral optic disc swelling and raised intracranial pressure in keeping with IIH. Prompt treatment of IDA with blood transfusion and orally administered iron supplements, in addition to medical treatment for IIH, contributed to significant improvement of symptoms and prevented long-term visual deficits.ConclusionThe possibility of IDA, albeit rare, should always be considered and investigated appropriately in all patients with IIH, as the treatment of the anemia alone may be sight-saving.

Elevated Intracranial Pressure Associated With Exogenous Hormonal Therapy Used for Gender Affirmation.

Addison disease, corticosteroid withdrawal, and taking synthetic growth hormone have been linked with development of intracranial hypertension, but there is still debate on whether administration of other exogenous hormones plays a role in precipitating elevated pressure. The growing use of hormonal therapy for gender affirmation provides an opportunity to explore this possibility. All transgender patients taking exogenous hormones for female-to-male (FTM) and male-to-female (MTF) transitions who were diagnosed with intracranial hypertension at Massachusetts Eye and Ear Infirmary, Massachusetts General Hospital and Beth Israel Deaconess Medical Center between August 2014 and November 2018 were included in a retrospective review. Visual acuity, type, and dose of exogenous hormone, visual field testing, clinical exam, results of neuroimaging and lumbar puncture, and treatment modalities were catalogued and analyzed. Six transgender individuals were identified. Five were FTM, with an average hormone treatment time of 18.4 months, and one was MTF who had been treated with hormones for 4 years. The average age of all patients was 23.5 years. The average time between onset of symptoms and presentation was 5 months. Fifty percent of the patients reported pulse-synchronous tinnitus, 83% reported positional headache, 33% reported transient visual obscurations, and 16% reported diplopia. Lumbar punctures performed on 4 of the patients revealed elevated opening pressures and normal cerebrospinal fluid constituents. MRI findings consistent with elevated intracranial pressure (ICP) were present in the other 2 patients in whom lumbar puncture was unsuccessful. Four patients were treated with acetazolamide and one was treated with topiramate, with an average follow-up time of 15.7 months. All patients demonstrated bilateral optic disc swelling, and all maintained normal acuity and color vision. Performance on visual field testing was not significantly affected in any patient. This is the largest reported series to date of gender-transitioning patients with intracranial hypertension, including one novel MTF conversion. These observations warrant further investigation into the possible link of exogenous hormonal therapy and elevated ICP and any mechanisms or confounders underlying this potential association.

PROLIFERATIVE LEUKEMIC RETINOPATHY WITH BILATERAL EXUDATIVE RETINAL DETACHMENTS AND OPTIC DISC SWELLING IN A PATIENT WITH ACUTE LYMPHOBLASTIC LEUKEMIA

Introduction: To report a patient with acute lymphoblastic leukemia (ALL) who developed proliferative leukemic retinopathy with bilateral exudative retinal detachments and optic disc swelling. &#x0D; Case Report: A 11-year-old girl diagnosed with ALL-L2 on maintenance chemotherapy presented with severe blurred vision in both eyes for the past 1 year. Her visual acuity was 0,5/60 for both eyes. Slit-lamp examination of the anterior segment was found normal. Dilated fundus examination revealed tortuous retinal vessels, retinal haemorrhage, retinal and optic disc neovascularizations, optic disc with indistinct margins, and total retinal detachments in both eyes, as well as vitreous haemorrhage in the left eye. The ultrasonography examination revealed bilateral subretinal fluid.&#x0D; Discussion: The patient was diagnosed with bilateral proliferative leukemic retinopathy with exudative retinal detachments and infiltrative optic neuropathy, and left eye vitreous haemorrhage due to ALL. Conservative treatment was given. She continued the maintenance chemotherapy from pediatric department. After 2 months follow up, the visual acuity did not improve although retinal detachments were reduced. There were atrophic changes at the retina of her both eyes.&#x0D; Conclusion: It was reported a rare case of proliferative leukemic retinopathy with bilateral exudative retinal detachments and optic disc swelling. Early recognition and treatment is crucial to improve prognosis.

Enlarged and Enhancing Optic Nerves in Advanced Glial Fibrillary Acidic Protein Meningoencephalomyelitis.

A 36-year-old woman presented with intermittent fever, nausea and vomiting, generalized polyarthralgias, and bilateral optic disc swelling. She had a history of difficult-to-control myasthenia gravis since the age of 18 years. Lumbar puncture demonstrated a normal opening pressure; cerebrospinal fluid (CSF) was remarkable for high protein, low glucose, and a mononuclear pleocytosis. Although initial MRI of the brain was normal, a repeat study 8 weeks later revealed enlarged and enhancing bilateral intraorbital and intracranial optic nerves. After a nondiagnostic brain biopsy, a CSF sample tested positive for antibodies to glial fibrillary acidic protein (GFAP). Findings in this case indicate that optic nerve swelling encountered in GFAP meningoencephalomyelitis is more likely due to optic nerve inflammation rather than elevated intracranial pressure.

Progressive Limb Weakness in A Young Man: A Case Report of POEMS Syndrome

Polyneuropathy is an initial presentation and essential feature of POEMS ( polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome. Neuropathy is typically distal, symmetric and slowly progressive with demyelinating changes. After a gradual proximal spread, it usually results in severe muscle weakness and functional disabilities. In the present report, we describe a 40-year-old diabetic male presented with gradually progressive weakness of both lower limbs for 1 year followed by the involvement of both upper limbs for the last 3 months. On examination hyperpigmentation, lymphadenopathy, gynecomastia, anasarca, hepatomegaly, bilateral optic disc swelling, sensory-motor polyneuropathy was found. Laboratory findings showed IgG lambda monoclonal gammopathy, raised VEGF, sensory-motor demyelinating and axonal polyneuropathy. All findings were consistent with POEMS syndrome. The patient was treated with lenalidomide and dexamethasone cyclical therapy with some clinical improvement.&#x0D; Bangladesh Journal of Neuroscience 2019; Vol. 35 (1): 51-56