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Multiphasic disseminated encephalomyelitis followed by optic neuritis in a child with gluten sensitivity

Ching BH, Mohamed AR, Khoo TB, Ismail HI

Mult Scler, 2015 Aug;21(9):1209-11.
PMID: 26199345 DOI: 10.1177/1352458515593404

Multiphasic disseminated encephalomyelitis (MDEM) followed by optic neuritis (ON) has been described as a new entity in recent years. Gluten encephalopathy has also been recognized as a neurological manifestation of celiac disease. Accurate diagnosis of both is important due to the therapeutic implications. We report a girl presenting with recurrent encephalopathic polyfocal demyelinating episodes followed by optic neuritis, and a clinical history suggestive of gluten sensitivity. She had persistently high ESR, neutrophilia, and tested positive for anti-MOG (myelin oligodendrocyte glycoprotein) antibody. She responded well to methylprednisolone in each relapse, and achieved remission for a year after azathioprine was added.

Matched MeSH terms: Optic Neuritis/complications*
Fulltext Encephalitis followed by optic neuritis: a case report and review of literature

Hasan S, B Basri H, P Hin L, Stanslas J

Pak J Med Sci, 2013 May;29(3):859-62.
PMID: 24353644

Encephalitis has been included in the causes of optic neuritis, but post encephalitic optic neuritis has been rarely reported. Majority of the cases of optic neuritis are either idiopathic or associated with multiple sclerosis, especially in western countries. This is very important in the Asian population where the incidence and prevalence of multiple sclerosis is not as high as in the Western countries. Although post infectious optic neuritis is more common in children, it can also be found in adults and is usually seen one to three weeks after a symptomatic infective prodrome. Here, we present a case of a 48 year-old-male who developed optic neuritis following viral encephalitis. His first presentation was with severe headache of two weeks duration. Viral encephalitis was diagnosed and treated. The patient presented again three weeks later with right eye pain and other features typical of optic neuritis. Corticosteroid therapy facilitated prompt recovery. Optic neuritis is an uncommon manifestation of encephalitis. It is important that both doctors and patients remain aware of post infectious cause of optic neuritis, which would enable a timely diagnosis and treatment of this reversible cause of vision loss.

Matched MeSH terms: Optic Neuritis
Fulltext Clinical presentation, severity and progression of primary angle closure in malay and chinese patients

Liza-Sharmini AT, Ng GF, Nor-Sharina Y, Khairil Anuar MI, Nik Azlan Z, Azhany Y

Med J Malaysia, 2014 Dec;69(6):245-51.
PMID: 25934953 MyJurnal

OBJECTIVE: To compare the clinical presentation, severity and progression of primary angle closure between Chinese and Malays residing in Malaysia.

METHODS: A comparative retrospective record review study was conducted involving one hundred (200 eyes) Malay and fifty eight (116 eyes) Chinese patients. They were selected from medical records of Hospital Universiti Sains Malaysia, Kelantan and Hospital Pulau Pinang, Penang, Malaysia. The selected patients were re-diagnosed based on the International Society of Geographical and Epidemiological classification. The clinical data on presentation including the presence of systemic diseases were documented. Progression of the disease was based on available reliable visual fields and optic disc changes of patients who have been on follow-up for at least five years.

RESULTS: Malay patients presented at older age (61.4 years SD 8.4) compared to Chinese (60.6 years SD 8.3). There was significant higher baseline Intraocular Pressure (IOP) among Malays (34.7 SD 18.5mmHg) compared to Chinese (30.3 SD 16.7mmHg) (p=0.032). The Chinese patients presented with significantly better visual acuity (p<0.001) and less advanced cup to disc changes (p=0.001) compared to Malays. Malay patients progressed faster than the Chinese. Majority progressed within 1 year of diagnosis. Malays without laser peripheral iridotomy (LPI) have a 4 fold (95% CI 1.4, 10.9) risk of progression. Higher baseline IOP, more advanced visual field defect and absence of LPI was identified as significant predictors associated with progression.

CONCLUSION: The Malays presented with more advanced angle closure glaucoma as compared to the Chinese in Malaysia. Aggressive disease progression was observed in Malays with the onset of optic neuropathy. Effective public awareness and aggressive management is important to prevent blindness in the Malaysian population.

Matched MeSH terms: Optic Neuritis
Fulltext The natural history of optic neuritis in Asian patients: an observational cohort study

Seah, B.H.A., Tow, S.L.C., Ong, K.C.B. Ong, Yang, C.C., Tsai, C.P., Lee, K.H., et al.

Neurology Asia, 2017;22(4):341-348.
MyJurnal

Optic neuritis, which may be a precursor to multiple sclerosis (MS), is an uncommon disease in
Asian patients. The Asian Collaborative Longitudinal Optic Neuritis Epidemiology (ACLONE) is
an observational cohort study that assessed the risk of recurrent optic neuritis and/or progression
of further neurologic events, either MS or neuromyelitis optica (NMO) in Asian patients with firstever
optic neuritis. Secondary aims were to study the presenting characteristics and visual outcome,
and to identify risk factors for development of either MS or NMO. A total of 112 patients (25 men
and 87 women) aged from 12 to 61 years were recruited from Singapore, Taiwan, South Korea and
Malaysia. Of these, 94 (84%) had unilateral optic neuritis, with the right eye involved in 45 patients
and the left eye in 49 patients and the remaining 18 (16%) had bilateral optic neuritis. Follow up
data was available for 104 patients, and patients were followed for a median duration of 25.9 months.
Of these patients, 6 patients were adjudicated to have reached the primary endpoint (composite of
MS/NMO and optic neuritis): 3 patients with recurrent optic neuritis also subsequently experienced
neurologic symptoms, and 3 patients without recurrent eye involvement had neurologic symptoms.
Only one patient was considered to have prototypical MS, the other 5 were diagnosed with NMO,
all with subsequent antibody confirmation. Optic neuritis in Asian patients has significantly different
presenting characteristics from the classic description. However, in the majority of the patients it is
usually a benign disease, with good visual outcome and no further events.

Matched MeSH terms: Optic Neuritis
Fulltext Caecocentral Scotoma: A Rare Presentation of Optic Perineuritis

Mohamad SA, Zunaina E, Wan Hitam WH

Cureus, 2019 Nov 08;11(11):e6101.
PMID: 31886042 DOI: 10.7759/cureus.6101

Optic perineuritis (OPN) is a subtype of optic neuritis (ON) in which the inflammatory process involves meningeal sheath surrounding the optic nerve. Clinically, OPN simulates ON. However, in contrast to ON, patient with OPN shows sparing of central vision, improves dramatically with high-dose corticosteroid, are more likely to experience recurrence after stopping treatment. We report a rare case of caecocentral scotoma observed in a female with typical ON symptoms. Her magnetic resonance imaging showed features in line with OPN. She was treated with intravenous methylprednisolone 1 g/day for five days followed by slow tapering dose of oral prednisolone for one month. Her vision improved dramatically with a resolution of visual field defect. No relapses seen within two years of follow-up.

Matched MeSH terms: Optic Neuritis
Unusual case of cerebral demyelination and bilateral optic neuritis in an infant with suppurative BCG lymphadenitis

Anandakrishnan P, Khoo TB

BMJ Case Rep, 2018 May 30;2018.
PMID: 29848532 DOI: 10.1136/bcr-2018-224496

Cerebral demyelination and optic neuritis are often seen in children with acute disseminated encephalomyelitis following various infections and immunisations. An eight month old girl presented with a left axillary lymph node swelling and an erythematous lace-like rash over her cheeks and trunk. She then developed acute encephalopathy, bilateral nystagmus, right hemiparesis and left facial nerve palsy. Her electroencephalogram showed an encephalopathic process and visual evoked response study were grossly abnormal. Her MRI brain showed hyperintensities in the midbrain, pons and bilateral cerebellar peduncles. She was treated as postinfectious cerebral demyelination with intravenous antibiotics, methylprednisolone and immunoglobulin. Left axillary lymph node excision biopsy and GeneXpert test detected Mycobacterium tuberculosis complex that prompted initiation of antituberculous therapy. Her chest X-ray and cerebrospinal fluid examinations for tuberculosis were normal. She showed significant recovery after 2 weeks. This case illustrates a rare presentation of cerebral demyelination and bilateral optic neuritis following suppurative BCG lymphadenitis.

Matched MeSH terms: Optic Neuritis/chemically induced*
Fulltext Posterior intraorbital metallic foreign body: a case discussion

Vinodh VP, Sellamuthu P, Harun RH, Zenian MS

Med J Malaysia, 2014 Apr;69(2):89-91.
PMID: 25241819 MyJurnal

Intraorbital foreign body (IOFB) has been a rare phenomenon in the cases of gunshot wounds and always represents a dilemma in medical management. In Sabah, this scenario is becoming common as there is still certain population in the interiors who owns self made guns for hunting. They either present with self inflicted gunshot injuries or after being mistakenly shot while hunting. There are very few articles on this topic especially when it is located posteriorly in the orbit and occurs without visual impairment. This case reports the challenges faced in the management of the patient with a posteriorly located metallic IOFB. The appropriate management of the patient is discussed based on several international literatures. Author describes a 37 years old male farmer, who was referred from a nearby district hospital after he sustained gunshot injuries to his face and scalp. Urgent computed tomography (CT) scan showed that bullet pellets were at the left orbital floor, the left mandible and the left frontal bone. All bullet pellets were located extracranially only without intracranial involvement. Patient clinically does not have any neurologic deficit and without any visual impairment, thus he refused any surgical intervention. As there is no proper guideline in managing such cases, decision was made based on evidences from international literatures. It was concluded that metallic IOFB located in the posterior orbit may be conservatively managed with observation and regular follow-ups as they are well-tolerated and does not cause much impact on visual deterioration unless inflammation, infection, optic neuropathy or functional deficit occurs. This avoids unnecessary surgery and prevents risk of iatrogenic injury to the eye.

Matched MeSH terms: Optic Neuritis
Fulltext Evaluation of retinal nerve fiber layer thickness and optic nerve functions in fellow eye of neuromyelitis optica with unilateral optic neuritis

Ong Chin Feng W, Wan Hitam WH

Taiwan J Ophthalmol, 2020 06 20;10(3):189-196.
PMID: 33110750 DOI: 10.4103/tjo.tjo_22_20

Purpose: Peripapillary retinal nerve fibre layer (RNFL) thickness might be useful in monitoring ongoing subclinical structural damage especially in eyes with no history of optic neuritis (ON) in neuromyelitis optica (NMO).
Objective: To evaluate the peripapillary RNFL thickness and optic nerve functions in fellow eye of NMO with unilateral optic neuritis.
Materials and Methods: A comparative cross-sectional study was conducted in 2 tertiary hospitals from August 2017 to May 2019. RNFL thickness and optic nerve functions were evaluated. Statistical analysis was performed using Statistical Package for Social Science version 24.
Results: A total of 26 NMO patients and 26 controls were involved in this study. The median age (IQR) of NMO patients was 32.5 (12) years old. The RNFL thickness was significantly reduced in NMO patients with non-ON eyes as compared to control group. Best corrected visual acuity between the 2 groups were comparable (0.20 vs 0.00, p=0.071). Contrast sensitivity was also reduced in NMO patients (non-ON eyes) at all 5 spatial frequencies. In NMO group, 34.6% have normal colour vision. The mean deviation (MD) of Humphrey visual field (HVF) was higher in NMO group (p<0.001). There was a moderate correlation between RNFL thickness and contrast sensitivity. Weak correlation was found between the RNFL thickness with visual acuity and mean deviation of visual field test.
Conclusion: Our study showed that the fellow eye of NMO patients with unilateral ON revealed a significant reduction in RNFL thickness and all the optic nerve functions have subtle early changes that signify a subclinical retinal damage.

Matched MeSH terms: Optic Neuritis
Fulltext Myelin Oligodendrocyte Glycoprotein (MOG) Optic Neuritis: A Case Series

Nurul-Ain M, Khairul Kamal ZN, Wan Hitam WH, Abd Munaaim M, Mohd Zaki F

Cureus, 2021 Apr 13;13(4):e14452.
PMID: 33996312 DOI: 10.7759/cureus.14452

Myelin oligodendrocyte glycoprotein (MOG) antibody disease has been recognised as a distinct demyelinating disorder. Optic neuritis has been reported as the most common presentation and manifestation of this spectrum disorder. This is a case series of three MOG optic neuritis patients. Patients involved are female with disease onset ranging between 7- and 37-year-old. Most of these patients experienced symptoms of profound reduced visual acuity with eye pain. All three patients had optic disc swelling upon first presentation and they experienced at least one episode of bilateral simultaneous optic neuritis. Only one patient had demonstrable optic nerve enhancement on magnetic resonance imaging (MRI). Disease was confirmed through positive MOG antibody. Patients typically responded well to intravenous methylprednisolone (IVMP) during acute attack of optic neuritis. However, one patient had suboptimal response to IVMP after multiple relapses. We noted multiple relapses of optic neuritis are common in MOG patients. MOG optic neuritis is a devastating, but treatable condition. Aggressive treatment during acute optic neuritis attack and relapse prevention may favour a good visual prognosis in MOG antibody disease.

Matched MeSH terms: Optic Neuritis
Fulltext Retinitis punctata albescens presenting with tunnel vision: diagnostic dilemmas

Norhayaty S., Nurul Dalila M.S., Tai, Evelyn L.M., Liza Sharmini A.T., Zunaina E., Azhany Y.

Journal of Biomedical and Clinical Sciences, 2017;2(1):20-22.
MyJurnal

Tunnel vision is a classic sign among patients with advanced glaucoma. However, other conditions such as retinitis pigmentosa, optic neuritis and rod-cone dystrophy may be characterized by similar visual field defects. A 52-year-old lady with a family history of glaucoma presented with bilateral gradual loss of peripheral vision for two years. She claimed to have poor night vision about 20 years prior to this presentation. Her visual acuity was 6/7.5 in both eyes. The anterior chamber depth was moderate bilaterally, with Schaffer grading on gonioscopy of grade I to II. The intraocular pressure was 14 mmHg in both eyes. The optic discs appeared normal. Fundus examination showed scattered hypopigmented changes sparing the fovea. Humphrey visual field test revealed bilateral constricted visual fields. She was diagnosed with retinitis punctata albescens (RPA) based on her symptom of poor night vision, supported by the diffuse hypopigmented changes in her fundi. The management of this condition involves careful counselling regarding the genetic nature of the disease and its progressive course. We discuss this case to illustrate the importance of a thorough history taking and careful fundus examination in the workup of patients presenting with tunnel vision.

Matched MeSH terms: Optic Neuritis
Fulltext Recurrent optic neuritis as early presentation of idiopathic hypertrophic cranial pachymeningitis

Norhayaty Samsudin, Tai, Evelyn Li Min, Chui, Yain Chen, Kumar, Lakana, Azhany Yaakub, Adil Hussein, et al.

Journal of Biomedical and Clinical Sciences, 2017;2(1):23-26.
MyJurnal

44-year-old Malay lady presented with drooping of the right eyelid and worsening of left eye vision for one week duration. There was associated headache, periorbital discomfort and diplopia on left gaze. She previously had a history of recurrent optic neuritis affecting both eyes over a period of 12 years. On examination, there was right-sided partial ptosis and left exotropia. The adduction, abduction, elevation and depression of the right eye was limited. Left eye extraocular movements were full. The right eye visual acuity was 6/9, while the left eye visual acuity was perception to light, with a positive relative afferent papillary defect and a pale optic disc. The right optic disc was normal. There was reduced sensation in the trigeminal nerve distribution over the right side of the face. Neurological examination was otherwise normal. Magnetic resonance imaging of the brain and orbit revealed meningeal thickening with involvement of the right orbital apex and cavernous sinus. Blood investigations for infectious and autoimmune causes were unremarkable. She was diagnosed to have idiopathic hypertrophic cranial pachymeningitis and treated with systemic corticosteroids. The right eye extraocular motility improved, while the left eye visual acuity improved to counting finger. This case demonstrates that idiopathic hypertrophic cranial pachymeningitis may present as recurrent optic neuritis in the early phase, before radiological evidence of the disease is present. A high index of suspicion for the underlying cause is essential to prevent irreversible optic nerve damage due to recurrent optic neuritis.

Matched MeSH terms: Optic Neuritis
Ocular tuberculosis in Hospital Universiti Sains Malaysia - A case series

Shahidatul-Adha M, Zunaina E, Liza-Sharmini AT, Wan-Hazabbah WH, Shatriah I, Mohtar I, et al.

Ann Med Surg (Lond), 2017 Dec;24:25-30.
PMID: 29062482 DOI: 10.1016/j.amsu.2017.10.003

INTRODUCTION: Ocular tuberculosis (TB) encompasses a broad spectrum of clinical manifestations affecting different structures of the eye. It is caused by Mycobacterium tuberculosis, a great ancient organism that induces various types of diseases and unfavorable outcomes if unrecognized and not well treated.
PURPOSE: To report the clinical profile of 34 ocular TB cases observed during 6 years period in Hospital Universiti Sains Malaysia (HUSM).
METHOD: A retrospective review of medical records from 34 patients diagnosed with ocular TB in HUSM from January 2011 until December 2016.
RESULTS: The mean age was 43 ± 14.6 years old. Both male and female affected in about 1:1 ratio. The majority of subjects were local Malays (91.2%). Risk factors included previous contact with pulmonary TB patients (38.2%), and patients with underlying diabetes mellitus (26.5%). Most patients showed normal chest radiography (79.4%). However they had positive Mantoux test (94.1%) and raised erythrocyte sedimentation rate (ESR) value (58.8%). Uveitis was the most common ocular manifestation of ocular TB (70.6%) while the rare ocular manifestations included optic perineuritis and optic neuritis, orbital apex syndrome, orbital cellulitis, sclerokeratitis, corneal ulcer and conjunctival abscess. All patients responded well to anti-TB treatment, but visual outcome was variable.
CONCLUSIONS: This review shows the diverse entity of ocular TB spectrum in an endemic area. Good clinical response to anti-tuberculous therapy supported the presumed diagnosis of ocular TB in majority of the cases.

Matched MeSH terms: Optic Neuritis

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