Seropositive Neuromyelitis Optica imitating an Intramedullary Cervical Spinal Cord Tumor: Case Report and Brief Review of the Literature

Abstract

A 44-year-old woman with progressive cervical myelopathy and central cord syndrome was noted to have an extensive cervical intramedullary contrast-enhancing lesion on magnetic resonance imaging (MRI). The lesion resembled a spinal astrocytoma or ependymoma that required surgical intervention. She was subsequently diagnosed to have neuromyelitis optica (NMO), a rare idiopathic inflammatory demyelinating disorder, when the clinical examination revealed left optic atrophy. This was confirmed by a test showing seropositivity for NMO-immunoglobulin (IgG). Disease control was achieved with corticosteroids and immunosuppressive therapy. We report a rare case of a patient with NMO who had MRI features that could have easily led to the condition being misdiagnosed as a spinal cord tumor. The importance of careful history taking, awareness of typical radiological findings and the usefulness of serum NMO-IgG as a diagnostic tool are emphasized.

The Clinical and Treatment Characteristics of Neuromyelitis Optica. Summary of the Doctoral Thesis

“…Simultaneous involvement of the optic nerves and spinal cord, similar to the earlier reports, was more common among patients tested negative for AQP4-IgG (29% vs. 5%) (Jarius, Ruprecht et al 2012). In a single case report the patient tested positive for AQP4-IgG with symptoms of the index event (myelitis) evolving gradually over several months has been described (Woo, Chiu et al 2014); no other similar reports can be found in the current literature. A frequency of progressive onset attack in NMO is unknown.…”

“…To date only a single report (8 years ago) has described secondary progressive disease course in NMO, which occurred in only 2% of cases (Wingerchuk, Pittock et al 2007). A progressive onset of the disease (symptoms of the index event evolving over several months) has been also reported only in a single case (Woo, Chiu et al 2014).…”

longitudinally extensive myelitis as first presentation of renal cell carcinoma

“…However, development of progressive myelopathy in the absence of antecedent neurological symptoms and without signs indicating dissemination beyond the spinal cord is a diagnostic challenge, and rule out of other causes of myelitis should be done. 1 There have been many reports of patients with demyelinating disorders mimicking spinal cord tumors, [2][3][4][5] and on the contrary, we report a rare case of renal cell carcinoma (RCC) presenting by LETM without manifestation of primary tumor.…”

“…On the other hand, tumors can cause LETM as paraneoplastic lesions before detection of malignancy, particularly RCC. 4 RCC is unique among the genitourinary malignancies in that close to one third of affected patients show symptoms of a paraneoplastic syndrome in addition to central nervous system metastasis. Such as neuromyopathies have been described in cases of nonmetastatic RCC.…”

Optiskā neiromielīta klīniskās norises un ārstēšanas raksturojums. Promocijas darba kopsavilkums

“…Līdz šim ir tikai viens pētījums (pirms 8 gadiem) par sekundāri progresējošas norises biežumu NMO slimniekiem; tā novērota tikai 2% gadījumu (Wingerchuk, Pittock et al 2007). Arī progresējošs saslimšanas sākums (pirmā paasinājuma simptomu attīstība vairāku mēnešu garumā) līdz šim ir aprakstīts tikai vienā klīniskā gadījumā (Woo, Chiu et al 2014). MS slimnieku slimības aktivitātē vērojamas sezonālas svārstības, nereti ar stacionēto slimnieku skaita pieaugumu tieši gada "siltajos" mēnešos (Bamford, Sibley et al 1983, Jin, de Pedro-Cuesta et al 2000, Koziol and Feng 2004, Ogawa, Mochizuki et al 2004, Abella-Corral, Prieto et al 2005, Fonseca, Costa et al 2009, Balashov, Pal et al 2010, Meier, Balashov et al 2010, Salvi, Bartolomei et al 2010, Handel, Disanto et al 2011, Damasceno, Von Glehn et al 2012, Iuliano 2012, Hart and Gorman 2013, Muto, Mori et al 2013, Spelman, Gray et al 2014.…”