Neurosarcoidosis Presenting Initially as Idiopathic Vocal Cord Paralysis

Introduction: The presentation, course, and management of a rare laryngeal manifestation of neurosarcoidosis due to central nervous system (as opposed to peripheral nervous system) injury are described. Methods: The authors present 3 cases of vocal cord paralysis as the initial symptom of isolated neurosarcoidosis at a tertiary care laryngology clinic. Results: Laryngoscopy diagnosed unilateral vocal cord paralysis. Laryngeal electromyography revealed high vagal injury, prompting workup on brain magnetic resonance imaging. On magnetic resonance imaging, 2 cases showed basilar leptomeningeal inflammation and 1 case showed a brainstem mass. Patients were found at follow-up to have severe, progressive vagal injury, with patients developing severe quality of life impairments and medical complications. Conclusions: Neurosarcoidosis is not usually considered in the differential diagnosis of vocal cord paralysis. At initial presentation, all patients lacked other cranial neuropathies and systemic sarcoidosis manifestations, making diagnosis difficult. Otolaryngologists should be aware of this rare presentation, as prompt diagnosis by brain magnetic resonance imaging with or without central nervous system biopsy, as opposed to traditional chest radiography or computed tomography for the workup of peripheral nerve injury, is necessary. Serial laryngeal examinations are recommended for close monitoring of progressive disease and recommending treatment. Injection or medialization laryngoplasty can provide improvements in voicing but not swallow.

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Characteristic brain magnetic resonance imaging abnormalities in central nervous system aquaporin-4 autoimmunity

Multiple Sclerosis Journal ◽

10.1177/1352458510376640 ◽

2010 ◽

Vol 16 (10) ◽

pp. 1229-1236 ◽

Cited By ~ 121

Author(s):

Woojun Kim ◽

Min Su Park ◽

Sang Hyun Lee ◽

Su-Hyun Kim ◽

In Ja Jung ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

Central Nervous System ◽

Nervous System ◽

Magnetic Resonance ◽

Neuromyelitis Optica ◽

Brain Mri ◽

Brain Magnetic Resonance Imaging ◽

Aquaporin 4 ◽

Resonance Imaging ◽

Brain Involvement

Background: Although neuromyelitis optica has been traditionally regarded as a disease without brain involvement, brain abnormalities are not uncommon in patients with neuromyelitis optica-related disorders. Methods: We aimed to characterize the brain magnetic resonance imaging (MRI) abnormalities in neuromyelitis optica spectrum disorder patients who are seropositive for anti-aquaporin-4 autoantibody (AQP4 Ab). Of 236 consecutive patients with inflammatory demyelinating central nervous system diseases, we retrospectively analyzed MRI characteristics of 78 patients who were seropositive for AQP4 Ab. Results: For an average observational period of 6.3 years, 62 patients (79%) had brain lesions on MRI. Twenty-four patients (31%) had brain MRI abnormalities at the onset of disease, and 35 (45%) had symptomatic brain involvement. Characteristic brain MRI abnormalities were classified into five categories: (1) lesions involving corticospinal tracts (e.g. posterior limb of internal capsule and cerebral peduncle (44%); (2) extensive hemispheric lesions likely due to vasogenic edema (29%); (3) periependymal lesions surrounding aqueduct and the third and fourth ventricles (22%); (4) periependymal lesions surrounding lateral ventricles (40%); and (5) medullary lesions, often contiguous with cervical lesions (31%). Fifty-four patients (69%) showed at least one kind of brain abnormality among the five characteristic MRI lesions. Ten patients showed gadolinium-enhancing lesions, which were characterized by multiple patchy enhancing patterns with blurred margins. Conclusions: In central nervous system AQP4 autoimmunity, brain MRI abnormalities were more common than is generally appreciated and were characterized by their unique localization and configuration.

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Headache and Hemiparesis in Middle Age

10.1093/med/9780197583425.003.0061 ◽

2021 ◽

pp. 189-191

Author(s):

Catalina Sanchez Alvarez ◽

Kenneth J. Warrington

Keyword(s):

Magnetic Resonance Imaging ◽

Central Nervous System ◽

Nervous System ◽

Magnetic Resonance ◽

Cerebral Artery ◽

Brain Magnetic Resonance Imaging ◽

Resonance Imaging ◽

Primary Angiitis ◽

The Central Nervous System ◽

The Brain

A 53-year-old man with hypertension, hyperlipidemia, and a remote, cryptogenic, multifocal, posterior circulation ischemic stroke, came to the emergency department with 1 day of vertigo, ataxic gait, nausea, occipital headache, and painless binocular diplopia. Symptoms were present upon awakening on the day of presentation and progressed throughout the day. The erythrocyte sedimentation rate was 10 mm/h and C-reactive protein level was less than 3 mg/L. Cerebrospinal fluid examination indicated mild lymphocytic pleocytosis with 9 cells/µL, protein value of 45 mg/dL, and glucose level within normal limits. Brain magnetic resonance imaging and magnetic resonance angiography with contrast demonstrated a left caudate head infarction and leptomeningeal and perivascular enhancement involving bilateral temporal lobes, basal ganglia, and frontal lobes. Right middle cerebral artery wall enhancement was also noted. Conventional cerebral angiography showed diffuse dilatation and mural irregularity of the right middle cerebral artery M1 segment, as well as dilatation of the first 2 mm of the left anterior cerebral artery A1 segment. These findings were associated with vessel wall gadolinium enhancement on magnetic resonance imaging, which raised concern for vasculitis. The patient was diagnosed with primary angiitis of the central nervous system. The diagnosis was based on the presence of multiple ischemic infarcts, without cardioembolic source, abnormal brain magnetic resonance imaging and magnetic resonance angiography findings consistent with vasculitis, and the absence of systemic vasculitis, infection, and cancer. After diagnosis, the patient was started on intravenous methylprednisolone, followed by oral prednisone and intravenous cyclophosphamide. Because of some new areas of enhancement on magnetic resonance imaging, the patient was subsequently treated with rituximab. Clinical and radiologic remission was achieved, although the patient had permanent residual gait difficulties. Central nervous system vasculitis is an inflammatory process of the blood vessels in the brain, meninges, and spinal cord. It is called primary angiitis of the central nervous system when the process is limited to the brain and, rarely, the spinal cord. In other circumstances, central nervous system vasculitis can be secondary to a systemic inflammatory syndrome or infectious process.

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