ANCA-associated vasculitis presenting with isolated neurological manifestations in a patient with cocaine abuse: a case report and literature review.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of rare conditions predominantly affecting small vessels of skin, musculoskeletal, pulmonary, renal, and rarely central and peripheral nervous systems. Isolated neurological manifestations of AAV are uncommon and challenging to diagnose. Cocaine has been reported as a potential trigger for the development of AAV. There are only a few case reports of isolated neurological involvement in cocaine-induced AAV with poorly characterized histopathological features. We present a unique case of AAV with isolated neurological manifestations presenting with multiple cranial neuropathies, leptomeningeal enhancement on imaging and histopathologic evidence of small-vessel vasculitis in the leptomeninges and brain and extensive dural fibrosis in a patient with cocaine abuse. The patient's progressive neurological deficits were controlled after starting immunosuppression with rituximab and prednisone. We also reviewed the literature to provide the diagnostic overview of AAV and evaluate intervention options. To our knowledge, this is the first case of AAV with isolated neurological manifestations and histopathologic evidence of small-vessel vasculitis in a patient with cocaine abuse. Patients with multiple cranial neuropathies and meningeal involvement should be screened for AAV, especially if they have a history of cocaine abuse.

Multiple cranial neuropathy: Clinical findings in a case series of 142 patients.

INTRODUCTION: Multiple cranial neuropathies (MCN) is an entity frequently seen in clinical practice but there is a lack of studies published about this entity, with most of them based on case reports and small case series. OBJECTIVE: The aim of this study is to describe the clinical involvement of different cranial nerves, the location within the central or peripheral nervous system and the diagnosis in a group of patients with MCN managed in one hospital in Bogotá-Colombia. METHODOLOGY: A case series study was conducted using the electronic clinical records of a teaching hospital in Bogota-Colombia. Clinical data were collected from patients aged ≥18 with a clinical diagnosis of MCN between 2015 and July 2021. RESULTS: The cranial nerves most commonly affected were III and VII, with the most prevalent combinations being III-IV, III-VI, and V-VII. Among etiologies, the most frequently found were autoimmune, vascular and neoplastic and most common locations included peripheral nerves, neuromuscular junction, cavernous sinus and lateral medulla. CONCLUSIONS: The differential diagnosis of MCN is broad, but clinical clues may aid in identifying the underlying etiology. According to our results, MG was the most frequent etiology, so it should be considered in any patient with a clinical diagnosis of MCN associated with fatigability.

Multiple cranial nerve palsies in malignant external otitis: A rare presentation of a rare condition.

Malignant external otitis (MEO) is a rare inflammatory and infectious condition, typically caused by Pseudomonas aeruginosa, that mainly affects diabetic or immunocompromised elderly patients and is associated with severe morbidity and mortality. It begins in the external auditory canal and rapidly progresses through the skull base, leading to osteomyelitis and may result in cranial neuropathy, especially of the facial nerve. Here we describe a rare neurological presentation of MEO in a 65-year old diabetic man, who presented with an 8-month progressing left otitis externa and evolved with ipsilateral proptosis, ophthalmoplegia, blindness, facial palsy, hearing loss and contralateral evolvement of the temporal bone with hearing impairment. He was initially treated with oral ciprofloxacin and after one week was transferred to our tertiary hospital, where antibiotic therapy was switched to meropenem and vancomycin due to the severity of the case and to the hospital's microbiological profile. The patient underwent left canal wall-up mastoidectomy with insertion of ear ventilation tube bilaterally, with good recovery of right ear hearing capacity, but with no improvements of neurological deficits nor left hearing function. All microbiological tests performed were negative, and this was interpreted as a possible consequence of the early use of antibiotics. Unfortunately, the patient was infected by Sars-CoV-2 during hospitalization and passed away after ten days of COVID-19 intensive care unit internment.

[Atypical skull base osteomyelitis suspected of spreading inflammation from the ear canal with unilateral multiple cranial neuropathy and cerebral infarctions].

A 76-year-old man, who had undergone surgery for esophageal cancer in 2010, presented to our hospital in April 2017 complaining of prolonged slight fever, loss of appetite, and dysphagia. Initial evaluation revealed a paralyzed left vocal cord, slight muscle weakness of the extremities, left facial paralysis, hoarseness, left sternocleidomastoid and trapezius muscle weakness, tongue deviation to the left, and left hypacusia-suggesting a diagnosis of Garcin's syndrome. Laboratory tests revealed increased white blood cells and C-reactive protein. Cerebrospinal fluid (CSF) analysis showed mild pleocytosis (predominantly polymorphonuclear cells), elevated protein, and low CSF/plasma glucose ratio. CT showed mild clival erosion, with no evidence of carcinoma recurrence. Brain contrast-enhanced MRI showed abnormal clival marrow, enhanced soft tissue and dura matter from the clivus to the atlantoaxial joint, enhanced soft tissue around the left ear canal, multiple cerebral infarctions in the left watershed zones, and left internal carotid stenosis. There was excessive ear wax and inflammation of the left external acoustic meatus but no otorrhea or otalgia. On the basis of his overall presentation, he was diagnosed with atypical skull base osteomyelitis due to external otitis. He was treated with antibiotic treatment that included ceftazidime for the Pseudomonas aeruginosa detected on bacterial cultures. He did not respond to treatment and died approximately 4 months later. Skull base osteomyelitis is thus an important differential diagnosis candidate after finding unilateral, multiple cranial neuropathy, underscoring the importance of prompt treatment when suspected.

Sarcoid uveitis in a patient with multiple neurological lesions: a case report and review of the literature.

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. We further review relevant literature regarding Japanese patients with multiple cranial nerve palsies published within the past 34 years (from January 1982 to December 2016). CASE PRESENTATION: We report findings associated with the case of a 56-year-old Japanese woman with granulomatous pan-uveitis who was later diagnosed as having sarcoidosis by skin and transbronchial lung biopsies. She presented right-sided Bell's palsy and was treated with orally administered prednisolone. However, while prednisolone was tapered, she developed facial (VII) and vagus (X) nerve palsies, followed by brain parenchyma lesions, which were not associated with any additional neurological symptoms. Furthermore, she exhibited increased intraocular pressure in her right eye, and she underwent trabeculectomy. Our review of the literature revealed that 64 Japanese patients with sarcoidosis experienced multiple cranial nerve palsies between 1982 and 2016. The most commonly affected cranial nerves were the facial (VII) (73.4%) and glossopharyngeal/vagus (IX/X) nerves (48.4%). Palsies of two distinct cranial nerves were found in 40.6% of the patients, followed by palsies of three (23.4%) and four (18.8%) nerves. Almost all patients (98.3%) received systemic steroid therapy, and total or partial remission was achieved in almost all patients (96.5%). CONCLUSIONS: According to the literature, patients with multiple cranial nerve palsies associated with sarcoidosis respond well to orally administered steroid therapy. However, our findings suggest that careful follow-up is necessary for patients with neurosarcoidosis due to potential aggravation of neuropathy.

Multiple cranial neuropathy (a teaching case).

There are few reports of the multiple cranial neuropathy variant of Guillain-Barré Syndrome (GBS). Patients usually present with facial diplegia, lower cranial nerve involvement and hypo or areflexia. It is crucial to identify promptly this unusual cranial variant but the clinical characteristics remain poorly defined. This GBS variant usually has a rapid progressive course with respiratory muscle paralysis. Most of the patients recover well, although the process is slow. We report a 54 year old man presenting with facial diplegia, progressive ophthalmoplegia, lower cranial nerve involvement, sensory ataxia and generalized areflexia. This GBS variant is very unusual and seldom described in the literature; it is oftenly misdiagnosed. The clinical features and nerve conduction studies (absent F-waves, motor conduction block) provide evidence to support a diagnosis of an acute demyelinating polyneuropathy consistent with a regional cranial variant of GBS.

Hepatic Lymphoma Preceded by Recurrent Multiple Cranial Neuropathy

No abstract available.

Neurosyphilis Presenting with Multiple Cranial Neuropathy

Neurosyphilis can present with various clinical symptoms. We report a patient with multiple cranial neuropathy that, mimicked tuberculosis, but was finally diagnosed as neurosyphilis. A 38-year-old man was admitted due to subacutely progressive hearing loss. Brain MRI revealed multiple dural enhanced masses on contrast-enhanced T1 weighted images. Brain biopsy of the dural enhanced lesion revealed chronic granulomatous inflammation with coagulation necrosis, compatible with syphilitic gumma. Serum venereal disease research laboratory and fluorescent treponemal antibody absorption test results were positive. Treatment with penicillin resolved the patient's symptom.

A Case of Central Skull Base Osteomyelitis Concurrent with Multiple Lower Cranial Nerve Palsies / 대한이비인후과학회지

In most cases, skull base osteomyelitis arises from infections of external auditory canal, middle ear or paranasal sinuses. On the other hand, atypical or central skull base osteomyelitis has no evidence of external otitis or other infection sources. It is often found located on clivus and involves lower cranial nerves and brainstem through the foramina of skull base. The overall rate of mortality reaches 10% and long-term neurologic sequelae are seen in additional 31% of cases regardless of aggressive treatment. We recently encountered a 68-year-old man with right facial pain and generalized headache, and we achieved good outcome with the recovery of vocal cord palsy and disappearance of headache by surgical debridement and long-term antibiotics. We report it with a review of literature.

Multiple Cranial Neuropathy with and Polyradiculoneuropathy as a Delayed Sequelae after of Ethylene Glycol Intoxication / 대한임상신경생리학회지

Multiple cranial and peripheral neuropathies as a delayed sequellae of ethylene glycol poisoning is a less well known clinical entity and its information about long-term electrophysiological and clinical outcomes is limited. We report a 45-year-old male who presented with acute renal failure and subsequently developed multiple cranial neuropathy, respiratory failure, and flaccid tetraparesis. Through sequential electrophysiological studies, we would like suggest that the main pathophysiology of ethylene glycol-related neuropathy is a demyelinating polyradiculoneuropathy with secondary axonal degeneration.