scholarly journals Seronegative Neuromyelitis Optica: A Case Report of a Hispanic Male

2016 ◽  
Vol 8 (2) ◽  
pp. 102-107 ◽  
Author(s):  
Nabeel Badri ◽  
Mohamed Teleb ◽  
Saad Syed ◽  
Miraie Wardi ◽  
Mateo Porres-Aguilar ◽  
...  
Keyword(s):  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Brain Mri ◽  
Sudden Onset ◽  
Interesting Case ◽  
Imaging Studies ◽  
Thoracic Spinal Cord ◽  
Lower Extremity Weakness ◽  
Thoracic Spinal ◽  
Hispanic Male

Neuromyelitis optica (NMO) is a rare disease, common in white females and rarely reported in Hispanic males. It is usually associated with recurrent demyelinating spectrum that is autoimmune in nature. The diagnosis is usually confirmed by antibody biomarkers; however, they can be negative and lead to more dilemma in diagnosis. Furthermore, the course of disease and prognosis are different in seronegative as compared to seropositive NMO. Treatment is similar in both subgroups with new approaches under investigation for seronegative NMO patients. We present an interesting case of a 37-year-old Hispanic male who presented with sudden onset of lower extremity weakness, numbness, blurry vision, and urinary retention. Magnetic resonance imaging (MRI) of the thoracic spine showed multiphasic demyelinating process involving the thoracic spinal cord. His brain MRI also revealed changes suggesting optic neuritis. The patient met the criteria for diagnosis of NMO by having optic neuritis and myelitis by imaging studies despite having negative aquaporin-4 antibodies (AQP4-Ab). His condition improved after plasma exchange. NMO can be difficult to distinguish from acute multiple sclerosis in the early stages of the disease. Having AQP4-Ab testing is important for diagnosis with imaging studies; however, negative antibody results cannot exclude the diagnosis, but rather group it in seronegative subtype. Ongoing studies and research suggest that seronegative NMO might have a different pathophysiology, manifestation, and prognosis.

scholarly journals Atypical Multiple Sclerosis Presenting as Bilateral Optic Neuritis

2021 ◽  
Vol 20 (4) ◽  
pp. 182-188
Author(s):  
Sun Young Lee ◽  
Mi-Ra Park
Keyword(s):  
Multiple Sclerosis ◽  
Optic Neuritis ◽  
Clinical Signs ◽  
Sudden Onset ◽  
Oral Steroid ◽  
High Signal ◽  
High Dose ◽  
Color Test ◽  
Blurred Vision ◽  
Lower Extremity Weakness

Purpose: We report a case of multiple sclerosis with bilateral optic neuritis that was atypical in terms of both the clinical signs and symptoms.Case summary: A 34-year-old female visited the department of neurology with a complaint of sudden-onset, left lower extremity weakness and numbness that had developed 3 weeks prior, and bilateral blurred vision that had commenced 2 weeks prior. The patient was diagnosed with multiple sclerosis and prescribed high-dose intravenous methylprednisolone for 3 days, but the blurred vision did not improve. The patient was referred to the ophthalmology department. The initial best-corrected visual acuity was 0.04 in both eyes, and the relative afferent pupillary defect test was positive for the left eye. The Ishihara color test scores were 1/17 for both eyes. No optic disc swelling was evident on fundus examination. Brain and spine magnetic resonance imaging (T2-weighted) performed during the initial visit revealed lesions of high signal intensity in the frontal, subcortical white matter; the optic chiasm; the left retrobulbar optic segment; the medulla; and the spinal C2-5 processes. Aquaproin-4 immunogloblin G antibody (AQP4-IgG Ab) was not detected in serum. One month after prescription of the oral steroid, the vision improved to 1.0 in the right and 0.8 in the left eye. The patient was lost to follow-up after prescription of interferon-beta for 4 years, without recurrence.Conclusions: Binocular optic neuritis accompanied by severe visual loss is a rare form of multiple sclerosis. A thorough diagnosis (with a focus on exclusion) is required, as is appropriate treatment.

Is Devic's neuromyelitis optica a separate disease? A comparative study with multiple sclerosis

2003 ◽  
Vol 9 (5) ◽  
pp. 521-525 ◽  
Author(s):  
J de Seze ◽  
C Lebrun ◽  
T Stojkovic ◽  
D Ferriby ◽  
M Chatel ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Brain Mri ◽  
Oligoclonal Bands ◽  
Time And Space ◽  
Laboratory Findings ◽  
Therapeutic Trials ◽  
The Difference ◽  
Devic’S Neuromyelitis Optica

Devic's neuromyelitis optica (NMO) associates optic neuritis and myelopathy without other neurological signs. Many patients with NMO may be diagnosed as having multiple sclerosis (MS). However, there have been no previous studies comparing these two patho logies and it is still unclear if NMO is a separate entity or a subtype of MS. In the present study, we compared a series of NMO patients with a series of MS patients for whom optic neuritis or myelopathy was the presenting symptom, in order to determine the place of NMO in the spectrum of MS. We retrospectively studied 30 patients diagnosed with NMO and we compared these patients with 50 consecutive MS cases revealed by optic neuritis or acute myelopathy. MS patients were only included if a relapse occurred demonstrating time and space dissemination. We compared the two groups in terms of clinical presentatio n, laboratory findings (MRI and C SF) and clinical outcome. NMO patients were older and more frequently women than MS patients but the difference was not significant. C SF and MRI data were clearly different: oligoclonal bands (O C B) were found in 23% of NMO cases and 88% of MS (P B/0.001), abnormal brain MRI data were observed in 10% of NMO cases and 66% of MS (P B/0.001) and a large spinal cord lesion was observed in 67% of NMO cases and 7.4% of MS cases (P B/0.001). C linical outcome was evaluated as more severe in the NMO group (P B/0.001). O n the basis of clinical data, all NMO patients but three had dissemination in time and space. When we included MRI parameters, only two of the NMO patients met criteria for MS and one of the MS patients met criteria for NMO. O ur study demonstrates that NMO and MS should be considered as two different entities. The respective criteria for NMO and MS were able to distinguish these two patho logies but only when MRI data were applied. This finding could have implications for future therapeutic trials.

scholarly journals Spinal Cord Diffuse Midline Glioma in a 4-Year-Old Boy

2019 ◽  
Vol 6 ◽  
pp. 2329048X1984245 ◽  
Author(s):  
Ashutosh Kumar ◽  
Salman Rashid ◽  
Sumit Singh ◽  
Rong Li ◽  
Leon S. Dure
Keyword(s):  
Spinal Cord ◽  
Cerebrospinal Fluid ◽  
World Health ◽  
Thoracic Spinal Cord ◽  
Lower Extremity Weakness ◽  
Thoracic Spinal ◽  
History Of ◽  
Health Organization ◽  
Cerebrospinal Fluid Pleocytosis ◽  
Diffuse Midline Glioma

Objective: We report a child presenting with spinal myelopathy secondary to H3K27M mutant diffuse midline glioma. Case Report: A 4-year-old boy presented with a 3-week history of progressive gait difficulty. Examination revealed bilateral hand and lower extremity weakness, left leg hypertonia with ankle clonus, and a right hemisensory deficit. Magnetic resonance imaging of neuroaxis showed cervical and thoracic spinal cord with expansion and irregular areas of enhancement. Serum and cerebrospinal fluid studies were unremarkable for infectious, autoimmune, inflammatory, and neoplastic causes but showed mild cerebrospinal fluid pleocytosis, hypoglycorrhachia, and high protein level. A thoracic cord biopsy revealed a diffuse midline glioma (World Health Organization grade IV). Consequently, the tumor involved intracranial structures and patient died within 4 months after diagnosis. Conclusion: High-grade spinal cord gliomas are very rare but should be considered in the differential diagnosis of pediatric myelopathy. Tissue biopsy is recommended in indeterminate cases to facilitate diagnosis and to guide management.

scholarly journals The expanded spectrum of neuromyelitis optica: evidences for a new definition

2012 ◽  
Vol 70 (10) ◽  
pp. 807-813 ◽  
Author(s):  
Marco A Lana-Peixoto ◽  
Dagoberto Callegaro
Keyword(s):  
Optic Nerve ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Brain Mri ◽  
Transverse Myelitis ◽  
Aquaporin 4 ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Index Event ◽  
Aquaporin 4 Antibody ◽  
Extensive Transverse Myelitis

Neuromyelitis optica (NMO) has been traditionally described as the association of recurrent or bilateral optic neuritis and longitudinally extensive transverse myelitis (LETM). Identification of aquaporin-4 antibody (AQP4-IgG) has deeply changed the concept of NMO. A spectrum of NMO disorders (NMOSD) has been formulated comprising conditions which include both AQP4-IgG seropositivity and one of the index events of the disease (recurrent or bilateral optic neuritis and LETM). Most NMO patients harbor asymptomatic brain MRI lesions, some of them considered as typical of NMO. Some patients with aquaporin-4 autoimmunity present brainstem, hypothalamic or encephalopathy symptoms either preceding an index event or occurring isolatedly with no evidence of optic nerve or spinal involvement. On the opposite way, other patients have optic neuritis or LETM in association with typical lesions of NMO on brain MRI and yet are AQP4-IgG seronegative. An expanded spectrum of NMO disorders is proposed to include these cases.

scholarly journals NMO in pediatric patients: brain involvement and clinical expression

2011 ◽  
Vol 69 (1) ◽  
pp. 34-38 ◽  
Author(s):  
Joaquín A. Peña ◽  
María Elena Ravelo ◽  
Eduardo Mora-La Cruz ◽  
Cecilia Montiel-Nava
Keyword(s):  
Multiple Sclerosis ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Optic Neuropathy ◽  
Pediatric Patients ◽  
Brain Mri ◽  
Water Channel ◽  
Suggested Method ◽  
Clinical Expression ◽  
Brain Involvement

OBJECTIVE: To analyze the clinical, neuroimaging characteristics and positivity of the acquaporin water channel (NMO-IgG) in pediatric patients with neuromyelitis optica (NMO). This disorder could have a variable clinical expression. To address such variability, the term NMO spectrum has been suggested. METHOD: We evaluated six pediatric patients, with a median age of 11 years at the time of the study, with the diagnosis of NMO by the Wingerchuck criteria. RESULTS: All the cases exhibited bilateral optic neuritis (ON). Four patients had abnormalities on brain MRI from the onset,although only three of them developed symptoms correlated to those lesions during the course of their disorder. NMO-IgG was positive in 80%. CONCLUSION: Optic neuropathy is the most impaired feature in NMO patients. Brain MRI lesions are not compatible with multiple sclerosis and positivity of the NMO-IgG are also present in NMO pediatric patients, confirming the heterogeneity in the expression of this disorder.

Brain abnormalities in Sjogren syndrome with recurrent CNS manifestations: association with neuromyelitis optica

2009 ◽  
Vol 15 (9) ◽  
pp. 1069-1076 ◽  
Author(s):  
JH Min ◽  
HJ Kim ◽  
BJ Kim ◽  
KW Lee ◽  
IN Sunwoo ◽  
...  
Keyword(s):  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Vasogenic Edema ◽  
Brain Mri ◽  
Internal Capsule ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Brain Abnormalities ◽  
Posterior Limb ◽  
Brain Involvement

Background and objectives Optic neuritis or longitudinally extensive myelitis in Sjogren syndrome (SS) suggests a neuromyelitis optica spectrum disorder (NMOSD). However, brain abnormalities of SS remain to be elucidated for the association with neuromyelitis optica (NMO). Methods Twelve primary SS patients (all women, 42 ± 13.2 years) who had recurrent central nervous system (CNS) manifestations with brain involvement were retrospectively identified. Brain MRI, and neurologic and serologic findings were analyzed with the measurement of anti-aquaporin-4 antibody (AQP4-Ab). Results All patients showed brain lesions characteristic of NMO as follows: 1) the involved sites adjacent to the third and fourth ventricles and in the posterior limb of the internal capsule, 2) unique configurations, such as the longitudinal course from the internal capsule to the midbrain, large cerebral or cerebellar lesions over 3 cm, and cavity-like formations. AQP4-Ab was positive in six of eight patients tested, and all the seropositive patients showed lesions with increased diffusion, suggestive of vasogenic edema. Four patients met the revised criteria of NMO, and nine had features of NMOSDs. Of the remaining three patients showing only brain involvement, one had AQP4-Ab. Conclusions This study demonstrates that SS patients with recurrent CNS involvement have brain abnormalities characteristic of NMO and AQP4-Ab in Korea. The presence of AQP4-Ab in one SS patient with only brain involvement may suggest that the coexistence of NMO should be explored in SS patients with recurrent CNS manifestations, even without optic neuritis or myelitis.

Relapsing neuromyelitis optica in an adolescent girl

2021 ◽  
Vol 14 (8) ◽  
pp. e242402
Author(s):  
Vinita Gupta ◽  
Saurabh Luthra ◽  
Shrey Maheshwari ◽  
Shrutanjoy M Das
Keyword(s):  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Adolescent Girl ◽  
Brain Mri ◽  
Young Patients ◽  
Transverse Myelitis ◽  
Normal Brain ◽  
Antibody Testing ◽  
Visual Disability ◽  
Delay In Diagnosis

Early differentiation of neuromyelitis optica spectrum disorder (NMO-SD) from multiple sclerosis (MS) is of paramount importance as NMO-SD (especially relapsing variant) has more severe morbidity than MS. We describe a case of an adolescent girl who presented with repeated episodes of optic neuritis over a period of 4 years with normal brain MRI scans. She was treated initially as relapsing remitting MS, before showing clinical evidence of transverse myelitis (TM), and eventually being diagnosed as NMO-SD. Pulse intravenous methyl prednisolone along with immunosuppressive therapy led to remission of her disease. However, delay in diagnosis as NMO-SD led to visual disability in the left eye. Therefore, in young patients with recurrent optic neuritis and normal brain MRI, it may be prudent to get spinal MRI done to look for TM, even when asymptomatic. In addition, we should keep a low threshold for requesting aquaporin-4 antibody testing in these patients.

scholarly journals Role of Multimodality Intraoperative Neurophysiological Monitoring during Embolisation of a Spinal Cord Arteriovenous Malformation

2000 ◽  
Vol 6 (3) ◽  
pp. 223-234 ◽  
Author(s):  
F. Sala ◽  
Y. Niimi ◽  
A. Berenstein ◽  
V. Deletis
Keyword(s):  
Spinal Cord ◽  
Mr Imaging ◽  
General Anesthesia ◽  
Endovascular Procedures ◽  
Motor Evoked Potentials ◽  
Neurological Deficits ◽  
Neurophysiological Monitoring ◽  
Thoracic Spinal Cord ◽  
Lower Extremity Weakness ◽  
Thoracic Spinal

The decision whether or not to embolise during endovascular procedures for arteriovenous malformations (AVMs) of the spinal cord under general anesthesia, relies primarily on neurophysiological results of provocative tests with Lidocaine and short-acting barbiturates. Because of the complex haemodynamics of spinal AVMs, when either sensory (CSEPs) or muscle motor evoked potentials (mMEPs) are used independently, they can mislead the interpretation of provocative tests. This report illustrates the specific but complementary role played by provocative tests using CSEPs and mMEPs during embolisation of a low thoracic spinal cord AVM. We present the case of a 46 year old male with six year history of right lower extremity weakness. At that time, Magnetic Resonance (MR) imaging of the spine disclosed an intramedullary AVM at T11. He remained neurologically stable up to seven months before admission, when he developed sudden onset of low back pain, followed by progressive paraparesis, numbness in lower extremities, urinary retention and fecal incontinence. A new MR imaging study indicated venous thrombosis of the AVM. A two-stage embolisation was performed. During the first procedure, after provocative tests did not affect either CSEPs or mMEPs, an embolisation was performed through a sulco-commisure feeder from the anterior spinal artery (ASA) at T9. Conversely, provocative tests with Lidocaine performed from a right posterior spinal artery (PSA) feeder to the AVM nidus resulted in a significant (>50%) decrease of CSEPs, while mMEPs remained unchanged. The repeatedly positive tests warranted further investigation of the vascular anatomy which disclosed a normal right PSA distal to the nidus; the distal normal PSA was protected with coils. A repeated Lidocaine test was negative and the posterior feeder was embolised with no subsequent changes in CSEPs or mMEPs. After the procedure, the patient experienced only a mild transitory increase in right leg numbness, but no additional motor deficits. Five days later, the embolisation through the ASA feeder at T9 was completed on the basis of negative provocative tests. No additional neurological deficits were observed. Favoring either CSEPs or MEPs during endovascular procedures in the spinal cord is not justified by a solid scientific background. This case report illustrates that monitoring both CSEPs and mMEPs combined with provocative tests allows the safest and most effective embolisation of spinal cord AVMs under general anesthesia.

Regression of Recurrent Spinal Cord High-Grade Glioma After Convection-Enhanced Delivery of Nimustine Hydrochloride: Case Reports and Literature Review

2019 ◽  
Author(s):  
Toshiki Endo ◽  
Tomoo Inoue ◽  
Shinichiro Sugiyama ◽  
Ryuta Saito ◽  
Teiji Tominaga
Keyword(s):  
Spinal Cord ◽  
Case Reports ◽  
High Grade Glioma ◽  
Chemotherapeutic Agents ◽  
High Grade ◽  
Convection Enhanced Delivery ◽  
Thoracic Spinal Cord ◽  
Lower Extremity Weakness ◽  
Thoracic Spinal ◽  
Nimustine Hydrochloride

Abstract BACKGROUND Spinal cord high-grade glioma has poor prognosis. Especially, no treatment protocols have been established for recurrent cases. OBJECTIVE To apply a novel treatment method, convection-enhanced delivery (CED), for recurrent high-grade glioma. CED can deliver chemotherapeutic agents directly into the intramedullary lesion and possibly lead to remarkable regression of enlarging tumors that are, otherwise, difficult to control. METHODS Two patients developed high-grade glioma in the thoracic spinal cord. Partial resection and chemotherapy and radiotherapy induced remission of the disease. However, following the initial treatment, recurrence was noted in the spinal cord at 6 and 12 mo, respectively. No effective treatment was available for these recurrent lesions. Therefore, the authors decided to use CED to infuse nimustine hydrochloride (ACNU) directly into the spinal cord. During the procedure, the infusion cannula was inserted into the spinal cord lesion under intraoperative computed tomography scan. RESULTS After ACNU CED, successive magnetic resonance imaging confirmed remarkable shrinkages of the tumors in both cases. However, the patient's preinfusion symptoms, including bilateral lower extremity weakness, did not change after the treatment. Importantly, overall survivals of the 2 patients were as long as 67 and 33 mo. CONCLUSION The authors report the first 2 cases of recurrent spinal cord high-grade glioma. ACNU CED dramatically regressed enhanced mass lesions and provided local tumor controls in the spinal cord.

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