Facial nerve palsy in neurosarcoidosis: clinical course, neuroinflammatory accompaniments, ancillary investigations, and response to treatment

Nwebube, Chineze; Bou, Gabriela; Castilho, Alexander Jordan; Hutto, Spencer K.

doi:10.1007/s00415-022-11189-6

Cited by 9 publications

(10 citation statements)

References 25 publications

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“…Autoimmune- and tumor-related PFP can both be misdiagnosed as BP . Regarding autoimmune diseases, 2 existing studies also support the routine use of MRI in patients with suspected BP. Di Stadio et al observed that 13 of their 18 patients with MS (72.2%) had a history of PFP with no other associated clinical sign, with PFP being the first symptom of MS in 3 of the 13 cases (23.1%).…”

Section: Discussionmentioning

confidence: 96%

“…Therefore, PFP can be caused by central lesions and be misdiagnosed as BP in early stages of the disease . Similarly, Nwebube et al found that 23 of 218 patients with neurosarcoidosis (10.6%) in their cohort had PFP, with PFP being the first and only symptom of the disease in 12 of 23 cases (52.2%). Regarding tumors as the underlying cause, 50% of patients with facial nerve schwannoma have PFP, and Li and Dai found that 11 of their 32 patients with facial nerve schwannoma (34.4%) were initially diagnosed with BP before MRI.…”

Section: Discussionmentioning

confidence: 97%

“…There was no significant difference in symptom improvement at 1 month between the 2 groups. This may be because several causes of nonidiopathic PFP are responsive to corticosteroid therapy (ie, MS, neurosarcoidosis, cerebral lymphoma), such that facial function may improve at 1 month . Corticosteroids are recommended for patients in whom there is any suspicion of BP, and they improve the prospect for recovery .…”

Section: Discussionmentioning

confidence: 99%

“…Autoimmune-and tumor-related PFP can both be misdiagnosed as BP. 14 Regarding autoimmune diseases, 2 existing studies 15,16 21 Finally, isolated PFP can also be caused by ischemic or hemorrhagic lesions in the pons involving the nucleus of the facial nerve, 22 but these lesions rarely manifest as PFP alone. 23 One of the benefits of MRI in cases of presumed BP is the elimination of causative diseases with clinical presentations that mimic those of BP.…”

Section: Discussionmentioning

confidence: 99%

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Incidence of Underlying Abnormal Findings on Routine Magnetic Resonance Imaging for Bell Palsy

et al. 2023

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ImportanceThere is no consensus on the benefits of routine magnetic resonance imaging (MRI) of the facial nerve in patients with suspected idiopathic peripheral facial palsy (PFP) (ie, Bell palsy [BP]).ObjectivesTo estimate the proportion of adult patients in whom MRI led to correction of an initial clinical diagnosis of BP; to determine the proportion of patients with confirmed BP who had MRI evidence of facial nerve neuritis without secondary lesions; and to identify factors associated with secondary (nonidiopathic) PFP at initial presentation and 1 month later.Design, Setting, and ParticipantsThis retrospective multicenter cohort study analyzed the clinical and radiological data of 120 patients initially diagnosed with suspected BP from January 1, 2018, to April 30, 2022, at the emergency department of 3 tertiary referral centers in France.InterventionsAll patients screened for clinically suspected BP underwent an MRI of the entire facial nerve with a double-blind reading of all images.Main Outcomes and MeasuresThe proportion of patients in whom MRI led to a correction of the initial diagnosis of BP (any condition other than BP, including potentially life-threating conditions) and results of contrast enhancement of the facial nerve were described.ResultsAmong the 120 patients initially diagnosed with suspected BP, 64 (53.3%) were men, and the mean (SD) age was 51 (18) years. Magnetic resonance imaging of the facial nerve led to a correction of the diagnosis in 8 patients (6.7%); among them, potentially life-threatening conditions that required changes in treatment were identified in 3 (37.5%). The MRI confirmed the diagnosis of BP in 112 patients (93.3%), among whom 106 (94.6%) showed evidence of facial nerve neuritis on the affected side (hypersignal on gadolinium-enhanced T1-weighted images). This was the only objective sign confirming the idiopathic nature of PFP.Conclusions and RelevanceThese preliminary results suggest the added value of the routine use of facial nerve MRI in suspected cases of BP. Multicentered international prospective studies should be organized to confirm these results.

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Section: Discussionmentioning

confidence: 96%

Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Incidence of Underlying Abnormal Findings on Routine Magnetic Resonance Imaging for Bell Palsy

et al. 2023

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“… 4 The neurologic manifestations of sarcoidosis are protean and have the potential to affect any subcomponent of the nervous system, but particular clinical scenarios should prompt clinicians to consider neurosarcoidosis in the differential diagnosis, including meningitis (leptomeningitis or pachymeningitis), cranial neuropathies (facial and optic especially), and myelitis. 3 , 5 , 6 Meningeal inflammation is one of its most common forms, occurring in 16%–69% of affected patients. 3 , 7 - 9 Leptomeningitis (inflammation of the pia and arachnoid mater) occurs much more commonly in neurosarcoidosis than does pachymeningitis (inflammation of the dura mater).…”

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confidence: 99%

Pachymeningitis in Biopsy-Proven Sarcoidosis

Chakales

Herman

Chien

et al. 2022

Neurol Neuroimmunol Neuroinflamm

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Background and ObjectivesMeningeal inflammation is one of the most common manifestations of neurosarcoidosis, occurring in 16%–69% of affected patients. While the clinical and radiographic features of leptomeningitis in neurosarcoidosis are well known, those of pachymeningitis are far less clear. Our primary aim was to study the clinicoradiographic features of pachymeningeal involvement in neurosarcoidosis and its evolution over time in response to treatment.MethodsPatients with a diagnosis of neurosarcoidosis seen at Emory University (January 2011–August 2021) were included if pachymeningeal involvement was evident by MRI and the patient's sarcoidosis was pathologically confirmed (from a CNS or non-CNS site).ResultsTwenty-six of 215 (12.1%) patients with neurosarcoidosis qualified for inclusion. Pathologic confirmation came from CNS tissue in 50%. The median age of onset was 43.5 years; most were male (16/26, 61.5%). Symptoms were primarily related to pachymeningitis in 20/26 (76.9%). Headache (19/26, 73.1%), visual dysfunction (12/26, 46.2%), and seizures (7/26, 26.9%) were the most common symptoms. All patients had cranial pachymeningitis; only a single patient undergoing spinal imaging (1/11, 9.1%) had spinal pachymeningitis. The falx cerebri (16/26, 61.5%) was the most commonly affected dural structure, but the anterior and middle cranial fossae and tentorium cerebelli were frequently involved (12/26 each, 46.2%). The pachymeningeal lesions were unifocal (11/26, 42.3%) or multifocal (15/26, 57.7%) in distribution, nodular morphologically (23/25, 92.0%), and homogeneously enhancing (24/25, 96.0%). Symptomatic improvement occurred with steroids initially in 22/25 (88.0%). Ultimately, 23/26 (88.5%) required initiation of steroid-sparing immunosuppressants, including 8/26 (30.8%) eventually undergoing TNF inhibition. Pachymeningeal relapses occurred in 7/26 (26.9%). The median clinical follow-up was 48 months. The median modified Rankin scale score at last follow-up improved to 1.0 from 2.0 at presentation.DiscussionPachymeningitis due to sarcoidosis often presents with headaches, visual dysfunction, and seizures; it usually affects the dura of the falx cerebri, anterior and middle cranial fossae, and tentorium cerebelli and tends to require steroid-sparing immunosuppressants. It has the potential to relapse, but the prospect for recovery is good.

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