Anti-IgLON5 disease with distinctive brain MRI findings responding to immunotherapy: A case report. 2021

Yan Pi, and Li-Li Zhang, and Jing-Cheng Li
Department of Neurology, Institute of Surgery Research, Daping Hospital, Third Military Medical University (Army Medical University), 10 Changjiangzhilu, Yuzhong District, Chongqing, China.

BACKGROUND Anti-IgLON5 disease was first described as a progressive antibody-associated encephalopathy, with multiple non-specific clinical symptoms including sleep dysfunction, bulbar symptoms, progressive supranuclear palsy-like syndrome, cognitive impairment, and a variety of movement disorders. This newly discovered disease presents with unremarkable or unspecific brain magnetic resonance imagings (MRI), and have poor responsiveness to immunotherapy. METHODS In this case, a 37-year-old man presented with 4-day history of gait instability, dysarthria, and oculomotor abnormalities. The initial neurologic examination revealed mild unsteady gait, subtle dysarthria, and left abducent paralysis. METHODS The patient was diagnosed with anti-IgLON5 disease, based on clinical features and positive anti-IgLON5 antibodies in serum. METHODS Initially, the patient was treated with high dosages of methylprednisolone and immunoglobulins.Outcomes: The symptoms of patient rapidly improved after high-dose intravenous methylprednisolone and immunoglobulins. CONCLUSIONS In this paper, we report a new case of anti-IgLON5 disease with major symptoms of gait instability, dysarthria, and oculomotor abnormalities, with distinctive brain MRI findings, and responsive to immunotherapy.

UI MeSH Term Description Entries
D008279 Magnetic Resonance Imaging Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques. Chemical Shift Imaging,MR Tomography,MRI Scans,MRI, Functional,Magnetic Resonance Image,Magnetic Resonance Imaging, Functional,Magnetization Transfer Contrast Imaging,NMR Imaging,NMR Tomography,Tomography, NMR,Tomography, Proton Spin,fMRI,Functional Magnetic Resonance Imaging,Imaging, Chemical Shift,Proton Spin Tomography,Spin Echo Imaging,Steady-State Free Precession MRI,Tomography, MR,Zeugmatography,Chemical Shift Imagings,Echo Imaging, Spin,Echo Imagings, Spin,Functional MRI,Functional MRIs,Image, Magnetic Resonance,Imaging, Magnetic Resonance,Imaging, NMR,Imaging, Spin Echo,Imagings, Chemical Shift,Imagings, Spin Echo,MRI Scan,MRIs, Functional,Magnetic Resonance Images,Resonance Image, Magnetic,Scan, MRI,Scans, MRI,Shift Imaging, Chemical,Shift Imagings, Chemical,Spin Echo Imagings,Steady State Free Precession MRI
D008297 Male Males
D001921 Brain The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM. Encephalon
D004401 Dysarthria Disorders of speech articulation caused by imperfect coordination of pharynx, larynx, tongue, or face muscles. This may result from CRANIAL NERVE DISEASES; NEUROMUSCULAR DISEASES; CEREBELLAR DISEASES; BASAL GANGLIA DISEASES; BRAIN STEM diseases; or diseases of the corticobulbar tracts (see PYRAMIDAL TRACTS). The cortical language centers are intact in this condition. (From Adams et al., Principles of Neurology, 6th ed, p489) Hyperkinetic Dysarthria,Hypokinetic Dysarthria,Scanning Speech,Dysarthosis,Dysarthria, Flaccid,Dysarthria, Guttural,Dysarthria, Mixed,Dysarthria, Scanning,Dysarthria, Spastic,Dysarthoses,Dysarthria, Hyperkinetic,Dysarthria, Hypokinetic,Dysarthrias,Dysarthrias, Flaccid,Dysarthrias, Guttural,Dysarthrias, Hyperkinetic,Dysarthrias, Hypokinetic,Dysarthrias, Mixed,Dysarthrias, Scanning,Dysarthrias, Spastic,Flaccid Dysarthria,Flaccid Dysarthrias,Guttural Dysarthria,Guttural Dysarthrias,Hyperkinetic Dysarthrias,Hypokinetic Dysarthrias,Mixed Dysarthria,Mixed Dysarthrias,Scanning Dysarthria,Scanning Dysarthrias,Scanning Speechs,Spastic Dysarthria,Spastic Dysarthrias,Speechs, Scanning
D004660 Encephalitis Inflammation of the BRAIN due to infection, autoimmune processes, toxins, and other conditions. Viral infections (see ENCEPHALITIS, VIRAL) are a relatively frequent cause of this condition. Inflammation, Brain,Rasmussen Syndrome,Brain Inflammation,Encephalitis, Rasmussen,Rasmussen Encephalitis,Rasmussen's Syndrome,Brain Inflammations
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D001323 Autoantibodies Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them. Autoantibody
D015816 Cell Adhesion Molecules, Neuronal Surface ligands that mediate cell-to-cell adhesion and function in the assembly and interconnection of the vertebrate nervous system. These molecules promote cell adhesion via a homophilic mechanism. These are not to be confused with NEURAL CELL ADHESION MOLECULES, now known to be expressed in a variety of tissues and cell types in addition to nervous tissue. Axon-Associated Adhesion Molecules,Neuronal Cell Adhesion Molecules,Adhesion Molecules, Axon-Associated,Axon Associated Adhesion Molecules,Molecules, Axon-Associated Adhesion
D015840 Oculomotor Nerve Diseases Diseases of the oculomotor nerve or nucleus that result in weakness or paralysis of the superior rectus, inferior rectus, medial rectus, inferior oblique, or levator palpebrae muscles, or impaired parasympathetic innervation to the pupil. With a complete oculomotor palsy, the eyelid will be paralyzed, the eye will be in an abducted and inferior position, and the pupil will be markedly dilated. Commonly associated conditions include neoplasms, CRANIOCEREBRAL TRAUMA, ischemia (especially in association with DIABETES MELLITUS), and aneurysmal compression. (From Adams et al., Principles of Neurology, 6th ed, p270) Cranial Nerve III Diseases,Third-Nerve Palsy,Oculomotor Nerve Disorders,Oculomotor Nerve Palsy,Oculomotor Nerve Paralysis,Oculomotor Neuropathy,Partial Third-Nerve Palsy,Third Cranial Nerve Diseases,Third-Nerve Paralysis,Total Third-Nerve Palsy,Nerve Disease, Oculomotor,Nerve Disorder, Oculomotor,Nerve Palsy, Oculomotor,Nerve Paralysis, Oculomotor,Neuropathy, Oculomotor,Oculomotor Nerve Disease,Oculomotor Nerve Disorder,Oculomotor Nerve Palsies,Oculomotor Nerve Paralyses,Oculomotor Neuropathies,Palsy, Oculomotor Nerve,Palsy, Partial Third-Nerve,Palsy, Third-Nerve,Palsy, Total Third-Nerve,Paralysis, Oculomotor Nerve,Paralysis, Third-Nerve,Partial Third Nerve Palsy,Partial Third-Nerve Palsies,Third Nerve Palsy,Third Nerve Paralysis,Third-Nerve Palsies,Third-Nerve Palsies, Partial,Third-Nerve Palsy, Partial,Third-Nerve Palsy, Total,Third-Nerve Paralyses,Total Third Nerve Palsy,Total Third-Nerve Palsies

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