059 Teratoma with glycine, NMDA & VGKC antibodies presenting with severe weakness, respiratory failure & corticospinal tract dysfunction

2018 ◽  
Vol 89 (6) ◽  
pp. A24.2-A25
Author(s):  
Andrew Swayne ◽  
Cullen O’Gorman ◽  
Nabeel Sheikh ◽  
Helen Brown ◽  
Lyndall Buck ◽  
...  
Keyword(s):  
Respiratory Failure ◽  
Muscle Weakness ◽  
Corticospinal Tract ◽  
Glycine Receptor ◽  
Cystic Teratoma ◽  
Neural Tissue ◽  
Ovarian Teratoma ◽  
Dystrophic Calcification ◽  
Lymphoid Infiltrate ◽  
Receptor Antibodies

IntroductionGlycine antibodies are associated with progressive encephalomyelitis with rigidity and myoclonus (PERM) but have been rarely reported to cause myasthenic-like symptoms.1 We present a single patient seen at a tertiary neurology centre with fatigable muscle weakness and corticospinal tract dysfunction with positive glycine, VGKC and NMDA receptor antibodies, associated with an ovarian teratoma expressing neural tissue.CaseA 32 year old female presented with fatigable muscle weakness. Physical examination was atypical for a disease of the neuromuscular junction with features of exaggerated reflexes and clonus. The weakness progressed over 1–2 weeks leading to respiratory failure. Investigations including MRI brain, repetitive stimulation and acetylcholine-receptor antibody studies were all within the normal limits. Studies for neuronal antibodies against intracellular targets were negative. Further investigation revealed positive autoantibodies against Glycine receptor, NMDA (N-Methyl-d-aspartate) receptor and VGKC (voltage-gated potassium channel) with an underlying ovarian teratoma. Complete clinical resolution was achieved with teratoma resection and 2 g/kg course of intravenous immunoglobulin. Histopathological examination of the tumour revealed a mature cystic teratoma with dystrophic calcification. The teratoma included skin, adipose tissue, intestinal type mucosa, and neuroglial tissue in which nerve fibres and ganglion cells are present. A lymphoid infiltrate was concentrated in areas of neural tissue within the teratoma.ConclusionMuscle weakness, corticospinal tract dysfunction and respiratory failure have previously been reported in the context of anti-glycine antibodies. This is the first case where anti-glycine, anti-NMDA and anti-VGKC antibodies have been found in the context of an ovarian teratoma. The autoimmune nature of this condition is emphasised by the lymphoid infiltrate around the neural tissue expressed within the teratoma.Reference1. Carvajal-Gonzalez A, Leite MI, Waters P, Woodhall M, Coutinho E, Balint B, … Vincent A. Glycine receptor antibodies in PERM and related syndromes: characteristics, clinical features and outcomes. Brain2014;137(Pt 8):2178–2192.

scholarly journals A Case of a 34-Year-Old Female with Acute Hypoxemic Respiratory Failure and Proximal Muscle Weakness

2017 ◽  
Vol 2017 ◽  
pp. 1-3
Author(s):  
Alex Diaz ◽  
Surit Sharma
Keyword(s):  
Respiratory Failure ◽  
Muscle Weakness ◽  
Length Of Hospital Stay ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Hypoxemic Respiratory Failure ◽  
Acute Hypoxemic Respiratory Failure ◽  
Wound Botulism ◽  
Life Threatening Illness ◽  
Life Threatening

Wound associated botulism is an unusual presentation. Early detection of this potentially life-threatening illness can significantly shorten length of hospital stay and improve prognosis. We present a case of a 34-year-old female with a history of heroin abuse who presented to the ED with acute respiratory failure, diplopia, and proximal muscle weakness. There was early concern for wound botulism as the instigating process. After discussion with the CDC, she was given equine serum heptavalent botulism antitoxin. Laboratory analysis later confirmed our suspicion. Symptoms improved and the patient was liberated from mechanical ventilation on day 14 and discharged from the hospital on day 23.

scholarly journals Case Report of Three Immature Cystic Teratomas in Northern Ghana

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
E. M. Der ◽  
S. Seidu
Keyword(s):  
Case Report ◽  
Histopathological Examination ◽  
Germ Cell Tumour ◽  
Cystic Teratoma ◽  
Abdominal Ultrasound ◽  
Northern Ghana ◽  
Ovarian Teratoma ◽  
Emergency Laparotomy ◽  
Abdominal Masses ◽  
High Sense

Background. Preoperative diagnosis of immature cystic teratoma can be challenging for clinicians. In this report, we present three cases.Methods. We describe three women aged 10, 20, and 23 years, respectively, who presented with abdominal masses which were diagnosed by abdominal ultrasound as mature cystic teratomas. All women had emergency laparotomy and oophorectomy.Results. Histopathological examination reported these ovarian tumours to be immature cystic teratomas. This case report also provided a brief summary of the clinicopathological features of all ovarian teratomas diagnosed in two centres during the period of review.Conclusion. Immature ovarian teratoma affects primarily younger patients; it is important for clinicians to have a high sense of suspicion whenever the diagnosis of a germ cell tumour is entertained.

Glycine receptor antibodies are detected in progressive encephalomyelitis with rigidity and myoclonus (PERM) but not in saccadic oscillations

2012 ◽  
Vol 259 (8) ◽  
pp. 1566-1573 ◽  
Author(s):  
Takahiro Iizuka ◽  
Maria I. Leite ◽  
Bethan Lang ◽  
Patrick Waters ◽  
Yoshiaki Urano ◽  
...  
Keyword(s):  
Glycine Receptor ◽  
Receptor Antibodies

scholarly journals Muscle-Specific Tyrosine Kinase-Associated Myasthenia Gravis: A Neuromuscular Surprise

2021 ◽  
Vol 2021 ◽  
pp. 1-3
Author(s):  
Hassam Ali ◽  
Rahul Pamarthy ◽  
Nayab Ahsan ◽  
WashmaAwan ◽  
Shiza Sarfraz
Keyword(s):  
Neuromuscular Junction ◽  
Tyrosine Kinase ◽  
Myasthenia Gravis ◽  
Muscle Weakness ◽  
Acetylcholine Receptors ◽  
Muscular Contraction ◽  
Skeletal Muscle Weakness ◽  
Hypoxic Respiratory Failure ◽  
Receptor Antibodies

Myasthenia gravis is a neuromuscular autoimmune disease that results in skeletal muscle weakness that worsens after periods of activity and improves after rest. Myasthenia gravis means “grave (serious), muscle weakness.” Although not completely curable, it can be managed well with a relatively high quality of life and expectancy. In myasthenia gravis, antibodies against the acetylcholine receptors at the neuromuscular junction interfere with regular muscular contraction. Although most commonly caused by antibodies to the acetylcholine receptor, antibodies against MuSK (muscle-specific kinase) protein can also weaken transmission at the neuromuscular junction. Muscle-specific tyrosine kinase myasthenia gravis (MuSK-Ab MG) is a rare subtype of myasthenia gravis with distinct pathogenesis and unique clinical features. Diagnosis can be challenging due to its atypical presentation as compared to seropositive myasthenia gravis. It responds inconsistently to steroids, but plasma exchange and immunosuppressive therapies have shown promising results. We report a case of a 49-year-old female who presented with acute hypoxic respiratory failure. Our patient experienced progressive, undiagnosed MuSK-Ab MG for years without a diagnosis.

scholarly journals Paraneoplastic AQP4-IgG–Seropositive Neuromyelitis Optica Spectrum Disorder Associated With Teratoma

2021 ◽  
Vol 8 (5) ◽  
pp. e1045
Author(s):  
Ryotaro Ikeguchi ◽  
Yuko Shimizu ◽  
Ayato Shimomura ◽  
Miki Suzuki ◽  
Kanoko Shimoji ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Neuromyelitis Optica ◽  
Neural Tissue ◽  
Spectrum Disorder ◽  
Lower Limbs ◽  
Ovarian Teratoma ◽  
Cerebral Peduncle ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Lymphocyte Infiltration ◽  
Sensory Dysfunction

ObjectivesTo assess a case of paraneoplastic aquaporin-4 (AQP4)-immunoglobulin G (IgG)–seropositive neuromyelitis optica spectrum disorder (NMOSD) associated with teratoma and determine whether it is a paraneoplastic neurologic disorder.MethodsA single case study and literature review of 5 cases.ResultsA 27-year-old woman presented with diplopia, facial nerve palsy, paraplegia, sensory dysfunction of lower limbs, dysuria, nausea, and vomiting. Spinal cord MRI detected an extensive longitudinal lesion in the spinal cord, and brain MRI detected abnormal lesions in the right cerebral peduncle and tegmentum of the pons. CSF analysis revealed positive oligoclonal IgG bands (OCBs). The patient tested positive for AQP4-IgG, confirming a diagnosis of NMOSD. An abdominal CT scan detected an ovarian tumor. After steroid therapy and tumor removal, the patient progressively improved, with only mild sensory dysfunction. Histopathologic analysis of the tumor revealed a teratoma and the presence of glial fibrillary acidic protein (GFAP)+ neural tissue with AQP4 immunoreactivity, accompanied by lymphocyte infiltration. Including the present case, there have been 6 reported cases of AQP4-IgG–seropositive NMOSD associated with ovarian teratoma (mean onset age, 32.7 years). Of these patients, 5 (83%) presented with nausea and/or vomiting, positive OCB, and dorsal brainstem involvement. Pathologic analyses of the teratoma were available in 5 cases, including the present case, revealing neural tissue with AQP4 immunoreactivity and lymphocyte infiltration in all cases.ConclusionsThis study suggests that ovarian teratoma may trigger the development of AQP4-IgG–seropositive NMOSD. Further studies are needed to elucidate the pathogenesis of teratoma-associated NMOSD.

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