Neuromyelitis optica spectrum disorder (NMOSD) presenting as acute transverse myelitis with positive aquaporin 4 antibodies

2021 ◽  
Vol 14 (1) ◽  
pp. e238992
Author(s):  
Katherine Thornley ◽  
Carlo Canepa

An 80-year-old, previously healthy patient presents with acute transverse myelitis with sensory level at T8. The MRI scan of the spinal cord showed longitudinal extensive transverse myelitis, and she tested positive for aquaporin 4 antibodies in serum. She received treatment with intravenous and oral steroids, with no improvement and then underwent plasma exchange. She was then started on azathioprine for prevention of relapses, while continuing physiotherapy and occupational therapy. Eventually, she was transferred to a specialised spinal cord centre for long-term rehabilitation.

2020 ◽  
pp. 004947552095644
Author(s):  
Manjeet Kumar Goyal ◽  
Mahesh Lal

Transverse myelitis typically extends two or less spinal segments, whereas longitudinal extensive transverse myelitis (LETM) extends three or more spinal segments in length and may occasionally span all the segments of the spinal cord. We present a case of spinal tuberculosis presenting with LETM with true lower motor neuron-type flaccid paraplegia.


2003 ◽  
Vol 61 (2A) ◽  
pp. 265-268 ◽  
Author(s):  
Luiz Fernando Fonseca ◽  
Thelma Ribeiro Noce ◽  
Maria Letícia Gambogi Teixeira ◽  
Antônio Lúcio Teixeira Jr ◽  
Marco Aurélio Lana-Peixoto

Acute transverse myelitis is an acute inflammatory process of the spinal cord and it is a rare clinical syndrome in childhood. In this paper, we report a case of 3 years-old boy who developed acute onset tetraparesia following a viral respiratory infecction and hepatitis B vaccination. Magnetic resonance imaging of the spinal cord disclosed signal-intensity abnormalities from C4 to C3. A diagnosis of acute transverse myelitis was made and the patient was treated with IV methylprednisolone and IV immunoglobulin. The child had a fair outcome despite of the very acute course of the disease and the presence of a cervical sensory level which usually harbor a poor prognosis.


BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Wee Yong Tan ◽  
Abdul Hanif Khan Yusof Khan ◽  
Mohd Naim Mohd Yaakob ◽  
Anna Misyail Abdul Rashid ◽  
Wei Chao Loh ◽  
...  

Abstract Background Transverse myelitis (TM) is a relatively uncommon condition, and vaccine-associated myelitis is even rarer. Concern regarding neurological complications following vaccination escalated following the report of TM during the safety and efficacy trials of the COVID-19 vaccine. Case presentation We report the first case of Longitudinal Extensive Transverse Myelitis (LETM) in Malaysia following administration of the chimpanzee adenovirus-vectored (ChAdOx1 nCoV-19) vaccine. A 25-year-old female presented with bilateral lower limb weakness and inability to walk with a sensory level up to T8 with absent visual symptoms. Urgent gadolinium-enhanced magnetic resonance imaging (MRI) of the spine showed long segment TM over the thoracic region. Cerebrospinal fluid autoantibodies for anti-aquaporin-4 and anti-myelin-oligodendrocyte were negative. A diagnosis of LETM following vaccination was made, and the patient was started on a high dose of intravenous methylprednisolone. The patient eventually made a recovery following treatment. Conclusion LETM is a rare but serious adverse reaction following vaccination. Previously reported cases showed an onset of symptoms between 10 to 14 days post-vaccination, suggesting a delayed immunogenic reaction. However, the incidence of myelitis in COVID-19 is much more common, far greater than the risk associated with vaccination.


2021 ◽  
Vol 9 (06) ◽  
pp. 476-479
Author(s):  
Mohammed Ajamat ◽  
◽  
Kadira Abdi ◽  
Aziz Ahizoun ◽  
Youssouf Benmoh ◽  
...  

Spinal cord neurological disorders associated with Hashimotos disease are rare and very rarely described.Thediagnosis is based on a set of arguments with the performance of an exhaustive assessment in order to eliminate other possible causes of medullary neurological damage. In this case, we report a 21 years old man, who was admitted for a spinal cord syndrome with D12-L1 sensory level and was diagnosed with acute transverse myelitis associated with Hashimotos thyroiditis. The evolution was favorable with regression of the symptomatology after bolus of corticoids followed by oral corticotherapy.


2021 ◽  
Author(s):  
Jean-Christophe Van Cutsem ◽  
Ann-Sophie Lamon ◽  
Vincent Van Belleghem ◽  
Evelien Vancaester

Abstract IntroductionSince the COVID-19 pandemic, a growing number of central nervous system (CNS) complications in patients with COVID-19 have been reported. Isolated, longitudinally extensive transverse myelitis (LETM), is a unique presentation of CNS involvement. The limited reports, its diverse clinical manifestations and the possible long-term consequences make the reporting crucial to further our understanding of those syndromes occurring in COVID-19 positive patients.Case PresentationA 63-year old male consulted the emergency department after a sudden onset of gait ataxia, a one-week history of paresthesia progressing from the feet to the midsternal area and urinary. He tested positive on a SARS-CoV-2 RNA RT-PCR nasopharyngeal swab two days prior to the onset of his symptoms. Neurological examination showed a sensory level at T7 with symmetrically reduced fine touch, vibration, proprioception and furthermore an ataxic gait was observed. Cerebrospinal fluid on day one of admission showed pleocytosis, predominantly neutrophils, elevated protein count and normal glucose level and IgG. MRI of the spinal cord revealed a diffusely increased signal intensity involving the near-complete spinal cord, from the brainstem to level T12, fitting the diagnosis of LETM. ConclusionThe few cases of transverse myelitis in association with COVID-infection are believed to have an immune-mediated postinfectious mechanism. In this case however, parainfectious direct viral invasion of the spinal cord is far more likely because of a neutrophilic predominance in CSF and a short timespan between infection and symptoms. It could provide more clues that the SARS-CoV-2 is acutally capable of causing direct neurotoxic effects.


2013 ◽  
Vol 20 (7) ◽  
pp. 882-888 ◽  
Author(s):  
M Ringelstein ◽  
I Metz ◽  
K Ruprecht ◽  
A Koch ◽  
J Rappold ◽  
...  

Longitudinally extensive transverse myelitis is characteristic but not pathognomonic for neuromyelitis optica spectrum disorders (NMOSDs) and may mimic local tumors. In this retrospective study based on a cohort of 175 NMOSD patients we identified seven patients who initially presented with a longitudinally extensive spinal cord lesion and underwent spinal cord biopsy due to magnetic resonance imaging (MRI)-suspected malignancies. Remarkably, routine neuropathology was inconclusive and did not guide the diagnostic process to anti-aquaporin-4 (AQP4)-seropositive NMOSD. Serious postoperative complications occurred in 5/7 patients and persisted during follow-up in 2/7 patients (29%). Considering these sequelae, AQP4-antibody testing should be mandatory in patients with inconclusive longitudinally extensive spinal cord lesions prior to biopsy.


2021 ◽  
Vol 12 ◽  
Author(s):  
Gustavo C. Román ◽  
Fernando Gracia ◽  
Antonio Torres ◽  
Alexis Palacios ◽  
Karla Gracia ◽  
...  

IntroductionAlthough acute transverse myelitis (ATM) is a rare neurological condition (1.34-4.6 cases per million/year) COVID-19-associated ATM cases have occurred during the pandemic.Case-finding methodsWe report a patient from Panama with SARS-CoV-2 infection complicated by ATM and present a comprehensive clinical review of 43 patients with COVID-19-associated ATM from 21 countries published from March 2020 to January 2021. In addition, 3 cases of ATM were reported as serious adverse events during the clinical trials of the COVID-19 vaccine ChAdOx1 nCoV-19 (AZD1222).ResultsAll patients had typical features of ATM with acute onset of paralysis, sensory level and sphincter deficits due to spinal cord lesions demonstrated by imaging. There were 23 males (53%) and 20 females (47%) ranging from ages 21- to 73- years-old (mean age, 49 years), with two peaks at 29 and 58 years, excluding 3 pediatric cases. The main clinical manifestations were quadriplegia (58%) and paraplegia (42%). MRI reports were available in 40 patients; localized ATM lesions affected ≤3 cord segments (12 cases, 30%) at cervical (5 cases) and thoracic cord levels (7 cases); 28 cases (70%) had longitudinally-extensive ATM (LEATM) involving ≥4 spinal cord segments (cervicothoracic in 18 cases and thoracolumbar-sacral in 10 patients). Acute disseminated encephalomyelitis (ADEM) occurred in 8 patients, mainly women (67%) ranging from 27- to 64-years-old. Three ATM patients also had blindness from myeloneuritis optica (MNO) and two more also had acute motor axonal neuropathy (AMAN).ConclusionsWe found ATM to be an unexpectedly frequent neurological complication of COVID-19. Most cases (68%) had a latency of 10 days to 6 weeks that may indicate post-infectious neurological complications mediated by the host’s response to the virus. In 32% a brief latency (15 hours to 5 days) suggested a direct neurotropic effect of SARS-CoV-2. The occurrence of 3 reported ATM adverse effects among 11,636 participants in the AZD1222 vaccine trials is extremely high considering a worldwide incidence of 0.5/million COVID-19-associated ATM cases found in this report. The pathogenesis of ATM remains unknown, but it is conceivable that SARS-CoV-2 antigens –perhaps also present in the AZD1222 COVID-19 vaccine or its chimpanzee adenovirus adjuvant– may induce immune mechanisms leading to the myelitis.


2016 ◽  
Vol 47 (S 01) ◽  
Author(s):  
V. Kraus ◽  
C. Makowski ◽  
H. Jünger ◽  
A. Wawer ◽  
P. Strotmann ◽  
...  

2017 ◽  
Vol 23 (14) ◽  
pp. 1950-1954 ◽  
Author(s):  
Jinhua Zhang ◽  
Fang Liu ◽  
Yiqi Wang ◽  
Ying Yang ◽  
Yuehong Huang ◽  
...  

Understanding the characteristics of neuromyelitis optica spectrum disorder (NMOSD) with recurrent short partial transverse myelitis (SPTM), which is very rare, contributes to the differential diagnosis of multiple sclerosis (MS). We present two Chinese aquaporin-4 immunoglobulin G (AQP4-IgG)-seropositive NMOSD cases who had at least twice SPTM during 4 and 6 years of follow-up, respectively. Their SPTMs have been mild and responded well to corticosteroids just like in the case of MS. The findings highlight the need of searching for serum AQP4-IgG (cell-based assay strongly recommended) in patients with recurrent SPTM and suggest that those patients may have a mild acute attack phase and favorable long-term prognosis.


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