[Skip to Content]

Quiz

Clinical Challenge

Encephalomyelitis and Lymphadenopathy in a Man in His Early 40s

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.

1 Credit CME

JAMA Neurology

Published Online: June 15, 2020

JAMA Neurology Clinical Challenge

Article Information and Disclosures

Christos Bakirtzis, MD, MSc, PhD¹;

Styliani-Aggeliki Syntila, MD¹;

Nikolaos Grigoriadis, MD, PhD¹

Author Affiliations

¹Department of Neurology, AHEPA University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece

Read article on JAMA Network

Read Case Take Quiz Answer

A white man in his early 40s presented with dizziness and fatigue followed by tingling of both lower extremities as well as gait disturbances. The patient’s medical history was unremarkable besides mild intermittent whole-body itching starting 9 months prior. Physical examination revealed mild proximal pyramidal weakness of the left leg (Medical Research Council grade 4/5), with brisk reflexes and extensor plantar response as well as hypoesthesia below the T2 level without sphincter disturbances. In addition, mild decrement in position sense and marked decrement of vibration sense were observed in both lower extremities. No skin lesions were detected, while cervical and supraclavicular lymph nodes were enlarged. Multiple enhancing lesions were observed on brain and cervical spine magnetic resonance imaging (MRI) (Figure). Cerebrospinal fluid (CSF) was acellular, and eosinophils and oligoclonal bands were not detected, while proteins, glucose, and angiotensin-converting enzyme (ACE) levels were within normal range. Test results for B-cell clones, viral, and fungal infections were all negative. Routine blood test results were unremarkable besides an elevated eosinophilic count (2.76 K/μL;17.6%). Antibodies for AQP4, MOG, and paraneoplastic syndromes were not detected. Whole-body fluorodeoxyglucose positron emission tomography demonstrated generalized lymphadenopathy without any other pathological findings. Whole-body gallium scan, chest high-resolution computed tomography, and bone marrow biopsy results were normal, while axillary and inguinal lymph node biopsies revealed nonspecific reactive lymphadenopathy. Parasitic infections were excluded by plasma and CSF antibodies testing with enzyme-linked immunosorbent assay, stool, and lymph node microscopy. Extensive diagnostic workup for malignancies and systemic autoimmune diseases was also inconclusive.

Take Quiz

Please finish quiz first before checking answer.

A. Encephalomyelitis with hyperIgEaemia

Although no central nervous system (CNS) biopsy was performed, whole-body fluorodeoxyglucose positron emission tomography scan did not demonstrate hypermetabolism of the lesions. In addition, bone marrow and lymph node biopsies did not present any evidence of lymphoma. This patchy enhancement in the spinal cord has been previously described in sarcoidosis.¹ However, gallium scan, chest computed tomography, ACE levels both in serum and CSF, and glucose levels in CSF were all normal, while no granulomatous infiltration was observed in lymph node biopsies. The patient did not present with any skin lesions, asthma, or sinusitis; in fact, no other organ was involved. No signs of vasculitis were observed in the biopsied tissues, therefore criteria for the diagnosis of Churg-Strauss syndrome were not fullfiled.²

Sign in to take quiz and track your certificates

Buy This Activity

Article Information

Corresponding Author: Christos Bakirtzis, MD, MSc, PhD, Department of Neurology, AHEPA University Hospital, Aristotle University of Thessaloniki, 1 Stilp Kyriakidi Str, GR 54636 Thessaloniki, Greece (bakirtzischristos@yahoo.gr).

Published Online: June 15, 2020. doi:10.1001/jamaneurol.2020.1634

Conflict Interest Disclosures: Dr Bakirtzis reported personal fees from Novartis, Biogen, Specifar, Genesis, Roche, and Sanofi outside the submitted work. Dr Grigoriadis reported grants and personal fees from Genesis Pharma, Biogen, and Specifar Teva and Novartis, Sanofi, Roche, and Merck outside the submitted work. No other disclosures were reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References

Soni N , Bathla G , Pillenahalli Maheshwarappa R . Imaging findings in spinal sarcoidosis: a report of 18 cases and review of the current literature. Neuroradiol J. 2019;32(1):17-28. doi:10.1177/1971400918806634 PubMed Google Scholar Crossref

Fries JF , Hunder GG , Bloch DA , et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis: summary. Arthritis Rheum. 1990;33(8):1135-1136. doi:10.1002/art.1780330812 PubMed Google Scholar Crossref

Gregoire SM , Mormont E , Laloux P , Godfraind C , Gilliard C . Atopic myelitis: a clinical, biological, radiological and histopathological diagnosis. J Neurol Sci. 2006;247(2):231-235. doi:10.1016/j.jns.2006.05.045 PubMed Google Scholar Crossref

Kira J , Kawano Y , Horiuchi I , et al. Clinical, immunological and MRI features of myelitis with atopic dermatitis (atopic myelitis). J Neurol Sci. 1999;162(1):56-61. doi:10.1016/S0022-510X(98)00291-3 PubMed Google Scholar Crossref

Osoegawa M , Ochi H , Minohara M , et al. Myelitis with atopic diathesis: a nationwide survey of 79 cases in Japan. J Neurol Sci. 2003;209(1-2):5-11. doi:10.1016/S0022-510X(02)00441-0 PubMed Google Scholar Crossref

Kikuchi H , Osoegawa M , Ochi H , et al. Spinal cord lesions of myelitis with hyperIgEemia and mite antigen specific IgE (atopic myelitis) manifest eosinophilic inflammation. J Neurol Sci. 2001;183(1):73-78. doi:10.1016/S0022-510X(00)00475-5 PubMed Google Scholar Crossref

Osoegawa M , Ochi H , Kikuchi H , et al. Eosinophilic myelitis associated with atopic diathesis: a combined neuroimaging and histopathological study. Acta Neuropathol. 2003;105(3):289-295. doi:10.1007/s00401-002-0645-2 PubMed Google Scholar Crossref

Isobe N , Kanamori Y , Yonekawa T , et al. First diagnostic criteria for atopic myelitis with special reference to discrimination from myelitis-onset multiple sclerosis. J Neurol Sci. 2012;316(1-2):30-35. doi:10.1016/j.jns.2012.02.007 PubMed Google Scholar Crossref

Park CW , Choe WJ , Chun YI . Eosinophilic myelitis in the cervical cord mimicking intramedullary cord tumor. J Korean Neurosurg Soc. 2012;52(4):410-413. doi:10.3340/jkns.2012.52.4.410 PubMed Google Scholar Crossref

Itoh K , Umehara F , Osame M . Multifocal relapsing-remitting myelitis in a patient with atopic dermatitis: multiple sclerosis or atopic myelitis? Intern Med. 2002;41(6):495-497. doi:10.2169/internalmedicine.41.495 PubMed Google Scholar Crossref

AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.