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Otol Neurotol 2019 Apr;40(4):517-528

Department of Otorhinolaryngology.

Purpose: To examine the etiology, clinical course, and management of recurrent peripheral facial nerve paralysis.

Methods: Retrospective review at a single tertiary academic center and systematic review of the literature. Clinical presentation, laboratory and imaging findings, treatment and outcome for all cases of recurrent ipsilateral, recurrent contralateral, and bilateral simultaneous cases of facial paralysis are reviewed. Read More

Curr Opin Neurol 2019 Mar 6. Epub 2019 Mar 6.

Department of Medicine, University of Cincinnati Medical Center, Cincinnati, Ohio, USA.

Purpose Of Review: Sarcoidosis is a complex disease with many faces, and the clinical manifestation and course of neurosarcoidosis are particularly variable. Although neurosarcoidosis occurs in up to 10% of sarcoidosis patients, it can lead to significant morbidity and some mortality.

Recent Findings: Three criteria are usually required for a diagnosis of (neuro)sarcoidosis: clinical and radiologic manifestations, noncaseating granulomas, and no evidence of alternative disease. Read More

J Neurol 2019 Mar 11. Epub 2019 Mar 11.

Centre for Neurosarcoidosis, Neuroimmunology Unit, Institute of Immunity and Transplantation, University College London, London, NW3 2PF, UK.

Two cases in which a neurological disorder was identified pathologically to be due to a granulomatous infiltration were found after diagnosis to have an associated testicular seminoma with pathologically proven lymphatic metastasis. We present the clinical and imaging features, and pathological appearances of the lymphatic tissue and the brain. We summarise the literature to date and discuss the pathogenesis of the disorder and its treatment. Read More

Am J Med 2019 Mar 7. Epub 2019 Mar 7.

Department of Medicine, McMaster University, Health Sciences Centre, 4V33, 1200 Main Street West, Hamilton, Ontario, Canada L8N 3Z5.

Otol Neurotol 2019 Feb 27. Epub 2019 Feb 27.

Department of Otorhinolaryngology.

Purpose: To examine the etiology, clinical course, and management of recurrent peripheral facial nerve paralysis.

Methods: Retrospective review at a single tertiary academic center and systematic review of the literature. Clinical presentation, laboratory and imaging findings, treatment and outcome for all cases of recurrent ipsilateral, recurrent contralateral, and bilateral simultaneous cases of facial paralysis are reviewed. Read More

Neurosurgery 2019 Mar 4. Epub 2019 Mar 4.

Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota.

Background: Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis. Up to 50% of patients develop central nervous system involvement, and a subset of these patients can present with isolated tumor-like masses.

Objective: To describe the skull base manifestations of ECD with an emphasis on aspects most pertinent to surgeons who may be referred such patients for primary evaluation. Read More

Neuroradiol J 2019 Feb 21:1971400919830173. Epub 2019 Feb 21.

4 Institute of Nuclear Medicine, University College London, Institute of Nuclear Medicine, UK.

Purpose: Neurosarcoidosis and primary central nervous system lymphomas, although distinct disease entities, can both have overlapping neuroimaging findings. The purpose of our preliminary study was to assess if magnetic resonance texture analysis can differentiate parenchymal mass-like neurosarcoidosis granulomas from primary central nervous system lymphomas.

Methods: A total of nine patients was evaluated, four with parenchymal neurosarcoidosis granulomas and five with primary central nervous system lymphomas. Read More

Case Rep Cardiol 2019 15;2019:5845839. Epub 2019 Jan 15.

Department of Medicine, Division of Advanced Heart Failure and Transplant Cardiology, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.

Osborn waves, or J waves, initially described by John Osborn in 1953 in hypothermic dog experiments, are highly sensitive and specific for hypothermia. Initially thought to be secondary to a hypothermia-induced "injury current," they have more recently been attributed to a voltage differential between epicardial and endocardial potassium (I) currents. While the exact conditions required to induce such waves have been debated, numerous clinical scenarios of environmental and iatrogenic hypothermia have been described. Read More

J Neurol 2019 Feb 9. Epub 2019 Feb 9.

Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Service de Médecine Interne 2, Centre National de Référence Maladies Systémiques Rares, Hôpital Pitié-Salpêtrière, 47-83 Boulevard de l'hôpital, 75651, Paris Cedex 13, France.

Objectives: Infliximab is increasingly used to treat neurosarcoidosis. We aimed to determine the efficacy and tolerance of an infliximab biosimilar for treating neurosarcoidosis.

Methods: We conducted a retrospective single-center study to describe the efficacy, safety and immunogenicity of an infliximab biosimilar in neurosarcoidosis patients. Read More

Pract Neurol 2019 Jan 30. Epub 2019 Jan 30.

Wessex Neurological Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK.

A 52-year-old man experienced a relapse of neurosarcoidosis, characterised by obstructive hydrocephalus and multiple posterior circulation infarcts. He was taking methotrexate, but his prednisolone was being weaned because of adverse effects. Stroke is rare in neurosarcoidosis and typically relates to granulomatous inflammation with a predilection for the perforator arteries. Read More

Ann Indian Acad Neurol 2019 Jan-Mar;22(1):100-101

Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.

AJNR Am J Neuroradiol 2019 Mar 24;40(3):E14-E15. Epub 2019 Jan 24.

Division of Neuroradiology, Michigan Radiology University of Michigan, Ann Arbor, Michigan.

Case Rep Med 2018 11;2018:3162403. Epub 2018 Dec 11.

Department of Emergency Medicine, University of Michigan, Ann Arbor, MI, USA.

Introduction: Neurosarcoidosis, either isolated or as part of systemic sarcoidosis, is an uncommon entity and has diagnostic uncertainty. Treatment for neurosarcoidosis can increase the risk of infections, including fungal infections such as disseminated histoplasmosis. Neurosarcoidosis may further predispose patients to infections of the central nervous system. Read More

Nervenarzt 2019 Jan 7. Epub 2019 Jan 7.

Klinik für Innere Medizin, Spital Linth, Gasterstraße 25, 8730, Uznach, Schweiz.

J Community Hosp Intern Med Perspect 2018 11;8(6):363-367. Epub 2018 Dec 11.

HCA-West Florida Division, Tampa, FL, USA.

Sarcoidosis is an inflammatory granulomatous multisystem disease with an unknown etiology. Neurosarcoidosis is a cryptogenic neuroinflammatory manifestation of sarcoidosis. We describe a case of neurosarcoidosis with initial presentation as confusion. Read More

Medicine (Baltimore) 2018 Dec;97(50):e13667

Rationale: Sarcoidosis is a multisystem disorder characterized by noncaseating granulomas. The nervous system is involved in 5 to 16% of the patients. However, neurosarcoidosis in the medulla oblongata presenting as hiccough is remarkably rare. Read More

Cesk Slov Oftalmol 2018 ;73(5-6):189-197

Aim: To introduce the range of eye changes in sarcoidosis in the individual casuistics.

Materials: At the Ophthalmic Clinic of Teaching Hospital Královské Vinohrady in Prague were examined and treated six patients with ocular forms of sarcoidosis in the years from 1998 to 2015. Three patients were unilateral lesions of the lacrimal gland without systemic symptoms. Read More

OTO Open 2017 Oct-Dec;1(4):2473974X17742633. Epub 2017 Nov 15.

Department of Otolaryngology, New York University Langone Medical Center, New York, New York, USA.

Neurology 2018 Nov;91(22):1020-1021

From the Department of Neurology, University of Washington, Seattle.

eNeurologicalSci 2018 Dec 2;13:21-23. Epub 2018 Nov 2.

Dartmouth-Hitchcock Medical Center, Department of Neurology, 1 Medical Center Drive, NH 03756, Lebanon.

In this case report we compare two patients presenting with similar symptoms of a brainstem syndrome including ataxia, dysarthria, and diplopia. Their MRIs showed hyperintense FLAIR signal changes with patchy areas of contrast enhancement within the brainstem particularly the pons and cerebellum. The broad differential diagnosis of this brainstem pathology included rhomboencephalitis, neurosarcoidosis, lymphoma, vasculitis, infection, and paraneoplastic or autoimmune process. Read More

Int J Neurosci 2018 Nov 15:1-4. Epub 2018 Nov 15.

a Department of Neurology and Rehabilitation Medicine , University of Cincinnati , Cincinnati , OH , USA.

Introduction: CNS involvement in sarcoidosis is seen in 5-10% of cases. Long term treatment involves steroids and other immunomodulatory agents, including infliximab. Chronic immunosuppression can result in increased patient susceptibility to opportunistic infections. Read More

J Stroke Cerebrovasc Dis 2019 Feb 2;28(2):369-370. Epub 2018 Nov 2.

Department of Radiology, University of Iowa Hospitals and Clinics, Iowa City, Iowa. Electronic address:

Introduction: Venous phlebitis in Neurosarcoidosis (NS) is rare but is often associated with intracranial hemorrhage (ICH). Imaging findings in such cases have been recently described on susceptibility weighted imaging (SWI).

Case Presentation And Outcome: We report a patient who presented with ICH. Read More

Ther Adv Neurol Disord 2018 26;11:1756286418805732. Epub 2018 Oct 26.

Department of Neurology at Katholische Kliniken Ruhrhalbinsel Essen, Essen, Germany.

Background: Neurosarcoidosis occurs in about 5-15% of patients with sarcoidosis. Therapy with corticosteroids is generally accepted as the first-line medication, followed by various immunomodulating and cytotoxic agents or combined therapy. However, some patients show an unsatisfactory outcome or have adverse events and require novel treatment strategies. Read More

Dtsch Arztebl Int 2018 Sep;115(38):635

Neurol Clin 2018 Nov 20;36(4):831-849. Epub 2018 Sep 20.

Department of Neurology, Geisinger Commonwealth School of Medicine, Geisinger Medical Center, 100 North Academy Avenue, Danville, PA 17822, USA.

Chronic meningitis is defined as cerebrospinal fluid pleocytosis that persists for at least 4 weeks without spontaneous resolution. The differential diagnosis of chronic meningitis is broad, encompassing 4 main categories, including infectious, autoimmune, neoplastic, and idiopathic. Up to one-third of cases have no discernible cause, making chronic meningitis a diagnostic dilemma for many clinicians. Read More

Ann Otol Rhinol Laryngol 2019 Feb 26;128(2):157-161. Epub 2018 Oct 26.

2 Department of Otolaryngology-Head and Neck Surgery, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

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. Read More

J Med Case Rep 2018 Oct 23;12(1):307. Epub 2018 Oct 23.

Department of Ophthalmology and Visual science, Yokohama City University School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, Kanagawa, 236-0004, Japan.

Background: Neurosarcoidosis is a rare complication, and cranial neuropathy is the most frequent manifestation of this disease. However, few cohesive reports have discussed multiple cranial neuropathies in Japanese patients with sarcoidosis. The present report discusses the case of a patient with sarcoid uveitis and multiple neurological findings. Read More

Neurol Neuroimmunol Neuroinflamm 2018 Nov 26;5(6):e506. Epub 2018 Sep 26.

Department of Neurology (Y.W., A.W.), University of Washington; Division of Rheumatology (J.A.), Department of Internal Medicine, University of Washington; and Department of Pathology (P.J.C.), University of Washington, Seattle.

Pan Afr Med J 2018 28;30:67. Epub 2018 May 28.

Service de Neurologie, Hôpital Militaire Moulay Ismail, Meknès, Maroc.

We report the case of a 37-year old patient with right optic neuropathy. Magnetic resonance imaging (MRI) showed T2 hypersignal in the midline, enhanced after gadolinium injection (figure 1). Cerebrospinal fluid (CSF) analysis revealed lymphocytic meningitis with 64 white blood cells associated with hyperproteinorachy. Read More

Ophthalmic Plast Reconstr Surg 2018 Nov/Dec;34(6):e197-e201

Ruiz Department of Ophthalmology and Visual Science.

A 61-year-old man with well-controlled diabetes mellitus type 2, cirrhosis from hepatitis C, alcohol abuse, and portal hypertension presented with painful vision loss and left orbital swelling. Imaging showed diffuse orbital, perineural, and pachymeningeal inflammation. He was initially diagnosed with neurosarcoidosis. Read More

Neuroradiol J 2019 Feb 12;32(1):17-28. Epub 2018 Oct 12.

Neuroradiology Department, University of Iowa Hospitals and Clinics, USA.

Purpose: Spinal sarcoidosis, referring to involvement of the spine in sarcoidosis, is relatively rare and may mimic other neurological disease affecting the spine. The authors present a clinic radiological review of 18 spinal sarcoidosis patients who presented to a tertiary hospital, with emphasis on initial imaging and radiological response to treatment.

Materials And Methods: We retrospectively reviewed our departmental imaging archives over a 15-year period and found 49 cases of neurosarcoidosis out of which 18 patients had spinal magnetic resonance imaging. Read More

Br J Neurosurg 2018 Oct 8:1-5. Epub 2018 Oct 8.

d Department of Otorhinolaryngology, Head and Neck Surgery , University of Bonn , Bonn , Germany.

Objective: Sarcoidosis is a multisystemic granulomatous disease of unknown cause which affects the lung or bilateral hilar lymphadenopathy in over 90% of the cases. Neurosarcoidosis (NS) is rare and accounts for approximately 5 - 15% of the cases. Involvement of all parts of the central and peripheral nervous system is possible with various clinical symptoms, e. Read More

J Clin Rheumatol 2018 Sep 21. Epub 2018 Sep 21.

Emory University Atlanta, GA

AJNR Am J Neuroradiol 2018 Nov 20;39(11):2045-2050. Epub 2018 Sep 20.

From the Division of Neuroradiology, Department of Radiology, University of North Carolina School of Medicine, Chapel Hill, North Carolina.

We describe the prevalence and potential significance of deep medullary vein engorgement on SWI in patients with neurosarcoidosis, a finding that has not been described previously. Engorgement was evaluated for possible associations with meningeal or perivascular disease, intracranial hemorrhage, and venous thrombosis, as well as with modified Rankin Scale scores at the time of MR imaging and at follow-up. Deep medullary vein engorgement was seen in 7 of 21 patients and was more common in men. Read More

Nervenarzt 2018 Oct;89(10):1095-1105

Institut für klinische Neuroimmunologie, Klinikum Großhadern, Ludwig-Maximilians-Universität München, Marchioninistr. 15, 81377, München, Deutschland.

Multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) are chronic inflammatory diseases of the central nervous system (CNS). They may cause inflammation in the brain, spinal cord and optic nerve. Both conditions must be differentiated from CNS manifestations of other systemic autoimmune diseases such as systemic lupus erythematosus (SLE), Sjögren's syndrome, autoinflammtory diseases and sarcoidosis, since amongst others myelitis and optic nerve inflammation may also occur in these conditions. Read More

Clin Case Rep 2018 Sep 10;6(9):1718-1722. Epub 2018 Jul 10.

Department of Neurology Royal Free Hospital London UK.

Neurosarcoidosis represents a significant diagnostic challenge, as clinical features overlap with other neuroinflammatory conditions, and biopsy of affected neuronal tissue is often high risk or not feasible. Here we highlight application of the modified Zajicek criteria to diagnose probable spinal neurosarcoidosis in the absence of histology from affected neuronal tissue. Read More

Turk J Ophthalmol 2018 Aug 4;48(4):202-205. Epub 2018 Sep 4.

University of Health Sciences, Kartal Dr. Lütfi Kırdar Training and Research Hospital, Ophthalmology Clinic, İstanbul, Turkey.

In this study, we present a case of bilateral optic neuropathy and macular ischemia in the right eye associated with neurosarcoidosis. A 26-year-old woman presented to our clinic with complaints of bilateral blurred vision. Bilateral granulomatous anterior uveitis, vitritis, optic neuropathy, and macular ischemia were detected in the right eye in slit-lamp examination. Read More

Case Rep Oncol 2018 May-Aug;11(2):521-526. Epub 2018 Jul 27.

Department of Neurology, Duke University, Durham, North Carolina, USA.

Recently, immune checkpoint inhibitors have revolutionized cancer care by enhancing anti-tumor immunity. However, by virtue of stimulating the immune system, they can lead to immune-related adverse events (irAEs). Neurologic irAEs are uncommon but are becoming increasingly recognized and can be quite serious or even fatal. Read More

Clin Nucl Med 2018 Nov;43(11):840-841

Institute of Clinical Neuroimmunology, and.

We present a 45-year-old man with newly generalized tonic-clonic seizures due to a contrast-enhancing frontal lesion with perifocal edema suggestive for high-grade glioma (HGG). For further evaluation, a dynamic F-FET PET scan was performed, which showed high F-FET-uptake with early peak and constantly decreasing time-activity curves, a characteristic feature of HGG. Stereotactic biopsy and histological evaluation excluded a neoplastic lesion but confirmed a manifestation of neurosarcoidosis with strong expression of the L-amino-acid-transporter considered responsible for F-FET-uptake. Read More

Mult Scler Relat Disord 2018 10 24;25:297-299. Epub 2018 Aug 24.

Alabama College of Osteopathic Medicine (ACOM), United States.

JAMA Neurol 2018 Dec;75(12):1546-1553

Department of Medicine, University of Cincinnati, Cincinnati, Ohio.

Importance: The Neurosarcoidosis Consortium Consensus Group, an expert panel of physicians experienced in the management of patients with sarcoidosis and neurosarcoidosis, engaged in an iterative process to define neurosarcoidosis and develop a practical diagnostic approach to patients with suspected neurosarcoidosis. This panel aimed to develop a consensus clinical definition of neurosarcoidosis to enhance the clinical care of patients with suspected neurosarcoidosis and to encourage standardization of research initiatives that address this disease.

Observations: The work of this collaboration included a review of the manifestations of neurosarcoidosis and the establishment of an approach to the diagnosis of this disorder. Read More

Case Rep Med 2018 6;2018:9496149. Epub 2018 Aug 6.

Department of Neurosurgery, Saitama Sekishinkai Hospital, Saitama, Japan.

We treated a patient with neurosarcoidosis, which caused the syndrome of inappropriate secretion of antidiuretic hormone (SIADH), in whom diagnosis was performed using neuroendoscopy. The patient was a 56-year-old female who was hospitalized for hyponatremia and diagnosed with SIADH based on a detailed examination. During the course, she developed impaired consciousness due to acute hydrocephalus, which improved after ventricular drainage. Read More

Intern Med 2019 Jan 24;58(1):105-108. Epub 2018 Aug 24.

Department of Pathology, Shizuoka General Hospital, Japan.

A 58-year-old Japanese woman complained of unstable gait and dizziness lasting for a month. She had been diagnosed histologically with pulmonary and cutaneous sarcoidosis and attended outpatient clinics for routine checkups. Head computed tomography and magnetic resonance imaging (MRI) indicated obstructive hydrocephalus caused by a contrast-enhanced lesion in the cerebral aqueduct. Read More

J Neurol 2018 Oct 14;265(10):2333-2341. Epub 2018 Aug 14.

Service de Médecine Interne 2, Institut e3m, Centre National de Référence Maladies Systémiques Rares, Sorbonne Université, Faculté de Médecine, Assistance Publique Hôpitaux de Paris, Hôpital de la Pitié-Salpêtrière, 47-83 Boulevard de l'hôpital, 75651, Paris Cedex 13, France.

Objectives: Clinical involvement of the nervous system is uncommon during sarcoidosis. Cerebrovascular events are rarely reported during sarcoidosis and may be confused with primary angiitis of the central nervous system. The characteristics and outcomes of cerebrovascular events during sarcoidosis have not been well-evaluated. Read More

Clin Neurol Neurosurg 2018 Oct 4;173:101-104. Epub 2018 Aug 4.

Department of Neurology, Aomori Prefectural Central Hospital, Aomori, Japan.

Neurosarcoidosis is difficult to diagnose, because definite diagnosis requires detailed histology of the central nervous system. Three-dimensional contrast-enhanced fluid-attenuated inversion recovery (CE-FLAIR) is more useful for detecting leptomeningeal lesions compared with 3D CE-T1 weighted imaging. However, the clinical diagnostic utility of 3D CE-FLAIR for neurosarcoidosis is unclear. Read More

Neurol Sci 2018 Oct 7;39(10):1725-1733. Epub 2018 Aug 7.

Department of Neurology, Xuan Wu Hospital, Capital Medical University, Changchun Street 45, Xicheng District, Beijing, 100053, People's Republic of China.

Neurosarcoidosis is relatively rare and has diverse manifestations. The clinical characteristics, diagnosis, treatment, and outcome for neurosarcoidosis in China are poorly understood. We retrospectively analyzed the clinical features, laboratory and imaging results, treatment, and outcomes in patients who met the criteria for definite or probable neurosarcoidosis in Xuan Wu Hospital of Capital Medical University from 2000 to 2015. Read More

J Immunother Cancer 2018 Jul 31;6(1):77. Epub 2018 Jul 31.

Division of Medical Oncology, Duke University Medical Center, 2301 Erwin Rd, Durham, NC, 27701, USA.

Background: Immune checkpoint inhibitors have transformed the treatment landscape for many cancers, including metastatic melanoma, but have also opened the door for a diverse variety of immune-related adverse effects.

Case Presentation: We describe the first reported case of presumed neurosarcoidosis as an immune-related adverse effect that developed nearly a year after discontinuation of treatment with combination ipilimumab and nivolumab for recurrent metastatic melanoma. The patient was noted to develop clinical signs consistent with systemic sarcoidosis shortly after the initiation of treatment and underwent a biopsy of hilar lymphadenopathy that confirmed sarcoidosis and after which immunotherapy was discontinued. Read More

J Stroke Cerebrovasc Dis 2018 Oct 25;27(10):e230-e232. Epub 2018 Jul 25.

Neurological Institute's Mellen Center for Multiple Sclerosis, Cleveland Clinic Foundation, Cleveland, Ohio. Electronic address:

Sarcoidosis is a multisystem granulomatous disease that can cause a wide range of neurologic symptoms. Leptomeningeal enhancement is frequently described but reports of stroke-like symptoms or the appearance of ischemia on magnetic resonance imaging are rare. We present a case of a patient with both leptomeningeal enhancement and multifocal restricted diffusion in a patient with sarcoidosis. Read More

Clin Radiol 2018 Oct 30;73(10):907.e15-907.e23. Epub 2018 Jun 30.

Division of Neuroradiology, Michigan Radiology, University of Michigan, Ann Arbor, MI, USA.

Aim: To determine the occurrence of ischaemic and haemorrhagic events in patients with neurosarcoidosis at presentation and follow-up and to evaluate its association with perivascular enhancement.

Materials And Methods: The MRI findings in patients with neurosarcoidosis who presented to our institute from 2002-2017 were retrospectively reviewed, with emphasis on cerebrovascular events. A chi-squared test was used to evaluate the statistical association with presence of perivascular enhancement. Read More