B-Cell Lymphoma Presenting With Seventh Cranial Nerve Palsy and Mononeuritis Multiplex: A Case Report and Comprehensive Literature Review.

Diagnosing B-cell lymphoma-associated mononeuritis multiplex is challenging due to its rarity and the potential co-existence of other causes of mononeuritis multiplex. Here, we report a case of a 74-year-old male who initially presented with left cranial neuropathies followed by right-sided extremity weakness with hyporeflexia, right facial involvement, and subsequently asymmetric weakness and multifocal muscle wasting. Minor improvements were observed with multiple rounds of steroid treatment. The diffuse large B-cell lymphoma diagnosis was eventually established six months later upon a repeat mediastinal lymph node biopsy and cerebrospinal fluid cytology. A nerve biopsy demonstrated severe axonal neuropathy with loss of axons in all fascicles without evidence of vasculitis. A muscle biopsy showed atrophy in both type 1 and type 2 fibers. A presentation of mononeuritis multiplex warrants concern for B-cell lymphoma, mainly when other mechanisms of peripheral neuropathy are less likely.

Painful Small Fiber Neuropathy Associated With Teriflunomide: A Case Series and Literature Review Related to Teriflunomide and Leflunomide.

Teriflunomide and its prodrug, leflunomide, are disease-modifying medications used to treat relapsing-remitting multiple sclerosis (RRMS) and rheumatoid arthritis (RA), respectively. Peripheral neuropathy is a rare side effect associated with both medications, although the incidence rate and exact pathological mechanism remain unknown. We present a retrospective case series of three patients with RRMS, who developed painful small fiber neuropathy at various timeframes (<6 months, one year, and four years, respectively) while on teriflunomide treatment (14 mg/day); we also engage in a literature review of small and large fiber neuropathy associated with teriflunomide and leflunomide use. All three patients developed small fiber neuropathy following teriflunomide exposure. Laboratory workup was negative for metabolic, infectious, vitamin deficiency-related, and autoimmune etiologies, except for one patient who had chronic metabolic syndromes (impaired glucose, hyperlipidemia) before medication intake. However, the patient developed neuropathy following teriflunomide treatment. Electrophysiological findings were negative for large fiber neuropathy in all three patients with positive skin biopsy, with reduced epidermal nerve fiber density (ENFD) in two of the three patients. Teriflunomide was discontinued in all cases, after which symptoms stabilized. Current literature on leflunomide supports a direct neurotoxic effect or buildup of toxic intermediates from uridine synthesis inhibition. Cessation of teriflunomide use in the described cases resulted in symptom stabilization. Early recognition and treatment may lead to good clinical outcomes in these patients.

Post-infectious Painful Sensory Neuronopathy Following Giardia Infection Responsive to Intravenous Immunoglobulin Treatment.

Sensory neuronopathy is a rare pure sensory disorder with characteristic clinical features of early-onset ataxia and a multifocal distribution of non-length-dependent sensory deficits. Diabetes is the most common cause of length-dependent peripheral neuropathy. However, in acute to subacute presentations, conditions such as autoimmune diseases, paraneoplastic syndrome, exposure to toxins, and viral infection could be common etiologies. This report presents a patient with sensory neuronopathy following a Giardia infection. Gait disturbance, neuropathic pain, ataxia, and pseudoathetosis improved by varying degrees following the monthly maintenance of intravenous immunoglobulin (IVIG). An immune-mediated or direct pathogenic attack can explain the underlying pathogenesis behind this patient's peripheral nerve dysfunction.

Overlapping Autoimmune Neurological Syndrome: A Case Report of Triple-Positive Antibody.

The presentation of several autoimmune neurological disorders in a single patient is rare and often debilitating. However, early diagnosis and efficacious treatment can lead to a significant recovery. Here, we present an interesting case of a triple antibody-positive autoimmune neurological syndrome patient who manifested the clinical features of neuromyelitis optica (NMO) spectrum disorder (NMOSD), N-methyl-D-aspartate (NMDA) receptor (NMDAR) encephalitis, and myasthenia gravis (MG). Hence, the patient manifested both central and peripheral nervous system immune-mediated neurological syndromes. A middle-aged female with a history of seropositive aquaporin-4 (AQP4) NMOSD on mycophenolate 1 g twice daily presented with severe fatigue and right eye ptosis (three months since NMOSD diagnosis) and tested positive for acetylcholine receptor (AchR) binding antibody, consistent with MG. Six months after the patient's NMOSD diagnosis, she began to experience subacute progressive cognitive decline, behavioral changes, imbalance, anxiety/panic attacks, and paranoid delusions. NMDAR encephalitis was suspected, and she tested positive for cerebrospinal fluid NMDAR antibodies. After treatment with steroids failed, she was given two doses of rituximab 1 g, two weeks apart, and reported improvement in her symptoms shortly after the second dose.

Relevance of Medullary Vein Sign in Neurosarcoidosis.

BACKGROUND: Central nervous system involvement is uncommon in patients with sarcoidosis. It remains a diagnostic challenge for clinicians, as there is a broad differential diagnosis that matches the presenting neurological signs. Often, the imaging findings also overlap with other disease entities. One understudied finding in patients with neurosarcoidosis is the presence of medullary vein engorgement on SWI imaging, termed the "medullary vein sign", which has been postulated to be a specific sign for neurosarcoidosis. This study aims to provide an understanding of the diagnostic potential of the medullary vein sign. METHODS: Thirty-two patients who presented with neurologic signs concerning for possible neurosarcoidosis were analyzed retrospectively for the presence of the medullary vein sign. RESULTS: Out of these cases, 7 cases of definitive neurosarcoidosis cases were found based on other imaging signs, biopsy and CSF analysis; the remaining were classified into groups as possible (16), probable (5) and (4) cases of other infectious meningoencephalitis including 2 cases of autoimmune encephalitis. Seven patients among all of these cases were found to have the medullary vein sign on imaging, with five cases with confirmed and two cases from possible neurosarcoidosis. The sensitivity of the medullary vein sign in this study was 71.4%, and the specificity was 92.3%. DISCUSSION: The benefits of improving diagnostic criteria for neurosarcoidosis include more rapid diagnosis leading to more prompt treatment, less exposure to potentially harmful antibiotics or antifungals, and less long-term neurological effects. Our results support that the medullary vein sign will potentially fill in the diagnostic gaps that have challenged the timely diagnosis of neurosarcoidosis. CONCLUSIONS: Our findings support that the medullary vein sign has a high specificity and should be included in the diagnostic criteria for neurosarcoidosis.

Clinical and diagnostic spectrum of optic neuritis: A single-center retrospective study of disorders associated with multiple sclerosis, anti-aquaporin-4 and anti-myelin oligodendrocyte glycoprotein antibodies.

OBJECTIVE: Optic neuritis (ON) is an immune-mediated optic neuropathy associated with multiple immune-mediated neurological conditions. Our aim was to characterize the clinical and diagnostic features of first or initial episodes of ON associated with multiple sclerosis (MS)-associated (typical) and antibody-related (atypical) ON. METHODS: Retrospective, single institution, medical record review. We analyzed demographic, clinical, laboratory, and radiographic findings of 139 patients who presented with first episodes of MS-associated ON (MS-ON), aquaporin 4 antibody-associated ON (AQP4-ON), and myelin oligodendrocyte glycoprotein antibody-associated ON (MOG-ON) between January 2015 and October 2019 without preceding diagnosis. Simple hypothesis testing assessed differences between groups were performed. RESULTS: Of 139 patients (109 [79 %] women; 29 [21 %] men; mean age 47 [SD, 14] years), 106 had MS-ON, 25 had AQP4-ON, and 8 had MOG-ON. Patients with MOG-ON had the highest recurrence rate (88 %) relative to MS-ON (28 %) and AQP4-ON (56 %) patients (P < .001). Patients with AQP4-ON had the highest mean visual functional system scores (4.3 [SD, 1.8]) relative to MS-ON (2.0 [SD, 1.9]) and MOG-ON patients (2.8 [SD, 2.0]) (P < .001). CONCLUSION: Patients presenting with initial episodes of ON exhibit a range radiographic and laboratory feature depending on the underlying associated disease. Understanding the variable characteristics of typical (MS-associated) and atypical (antibody-associated) ON may help physicians accurately diagnose and effectively treat ON.

Lateral Antebrachial Cutaneous Neuropathy: A Review of 15 Cases.

BACKGROUND:  Lateral antebrachial cutaneous nerve is a terminal sensory branch of the musculocutaneous nerve. Lateral antebrachial cutaneous neuropathy (LABCN) is rare and often underdiagnosed. Less than 100 cases have been described in the orthopedic literature. METHODS:  It's a single-center retrospective study. A retrospective chart review of patients with LABCN who were seen over 16 years was performed. Demographics and detailed clinical information were recorded. In addition, electrodiagnostic data were reviewed, and clinical outcome was recorded. RESULTS:  Fifteen patients were included in this study. Postsurgical etiology was the most common (n = 7) cause of LABCN. Other cases included antecubital fossa phlebotomy and intravenous placement (n = 4), trauma (n = 1), overuse or repetitive forearm use (n = 2), and dog bite (n = 1). No etiology was found in one case, but the patient had diabetes. CONCLUSION: Our study proposes that patient positioning during orthopedic surgeries leading to stretch or compression of the lateral antebrachial cutaneous nerve is the most likely cause of LABCN. Antecubital fossa needle placement is the second most common cause of LABCN. However, it's a rare mononeuropathy and can be underdiagnosed. Therefore, detailed history, examination, and nerve conduction studies of the bilateral lateral antebrachial cutaneous nerve could help establish the diagnosis after other etiologies have been carefully excluded.

Be Smart to Identify the Stroke-Like Migraine Attacks After Radiation Therapy (SMART) Syndrome.

Stroke-like migraine attacks after radiation therapy (SMART) are uncommon, often occurring years or decades after brain radiation therapy. This syndrome is a diagnosis of exclusion, and only about 40 cases describing SMART have been published, each one describing a constellation of symptoms and findings. Because symptoms can arise years after initial radiation therapy, the ability of physicians to recognize SMART and rule out other possible causes of symptoms is critical for the long-term care of oncology patients who have undergone cranial radiation. Here we present the case of a 55-year-old man who experienced SMART nine years after radiation therapy and who was successfully treated with steroids.

Chronic vertebrobasilar insufficiency in subclavian steal syndrome.

Subclavian steal syndrome is a vascular disorder that consists of significant blood supply restriction with resultant insufficiency of the vertebrobasilar artery and the subclavian artery causing symptomatic insufficiency to the brain and upper extremity. It is important to recognize this condition in patients with subacute to chronic posterior circulation vascular insufficiency as early diagnosis and treatment can have good clinical outcomes (J Clin Neurosci. 2010;17:1339). CT angiogram of the head and neck should be considered in patients with chronic vertebrobasilar insufficiency to evaluate subclavian steal syndrome.

COVID-19 in multiple sclerosis patients and risk factors for severe infection.

Multiple sclerosis (MS) patients have been considered a higher-risk population for COVID-19 due to the high prevalence of disability and disease-modifying therapy use; however, there is little data identifying clinical characteristics of MS associated with worse COVID-19 outcomes. Therefore, we conducted a multicenter prospective cohort study looking at the outcomes of 40 MS patients with confirmed COVID-19. Severity of COVID-19 infection was based on hospital course, where a mild course was defined as the patient not requiring hospital admission, moderate severity was defined as the patient requiring hospital admission to the general floor, and most severe was defined as requiring intensive care unit admission and/or death. 19/40(47.5%) had mild courses, 15/40(37.5%) had moderate courses, and 6/40(15%) had severe courses. Patients with moderate and severe courses were significantly older than those with a mild course (57[50-63] years old and 66[58.8-69.5] years old vs 48[40-51.5] years old, P = 0.0121, P = 0.0373). There was differing prevalence of progressive MS phenotype in those with more severe courses (severe:2/6[33.3%]primary-progressing and 0/6[0%]secondary-progressing, moderate:1/14[7.14%] and 5/14[35.7%] vs mild:0/19[0%] and 1/19[5.26%], P = 0.0075, 1 unknown). Significant disability was found in 1/19(5.26%) mild course-patients, but was in 9/15(60%, P = 0.00435) of moderate course-patients and 2/6(33.3%, P = 0.200) of severe course-patients. Disease-modifying therapy prevalence did not differ among courses (mild:17/19[89.5%], moderate:12/15[80%] and severe:3/6[50%], P = 0.123). MS patients with more severe COVID-19 courses tended to be older, were more likely to suffer from progressive phenotype, and had a higher degree of disability. However, disease-modifying therapy use was not different among courses.

Palatal myoclonus secondary to neurosarcoidosis.

Palatal myoclonus can be primary or secondary. In primary palatal myoclonus, no obvious structural brain lesions can be found within the triangle of Guillain and Mollaret. Common causes of secondary myoclonus include stroke, demyelination, infections, trauma, and neurodegeneration.

Foix-Alajouanine Syndrome Mimicking Longitudinally Extensive Transverse Myelitis.

Foix-Alajouanine syndrome is an arteriovenous malformation causing subacute congestive myelopathy that can lead to progressive paraplegia. It typically affects the lower thoracic and lumbosacral levels. Arteriovenous fistula (AVF) leads to increased venous pressure, decreasing the arteriovenous pressure gradient and leading to a decrease in spinal cord perfusion, oedema and necrosis. Early recognition and surgical intervention can result in a good prognosis. LEARNING POINTS: Spinodural arteriovenous fistula (SDAF) can present with longitudinally extensive transverse myelitis (LETM), progressive gait instability, and lower extremity weakness.Failure to promptly recognize this condition and misdiagnosis can result in a poor outcome.Microsurgical obliteration of the SDAF provides better results than embolization.

Value of terminal latency index and sensory electrophysiology in idiopathic and diabetic chronic inflammatory demyelinating polyradiculoneuropathy.

OBJECTIVES: To evaluate sensory electrophysiology, terminal latency index (TLI), and treatment response in idiopathic and diabetic chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). METHODS: We performed a retrospective review of 147 patients with CIDP who underwent electrodiagnostic evaluation (January 2000-December 2015). Eighty-nine patients fulfilled electrophysiological criteria described by the Ad hoc Subcommittee of the American Academy of Neurology and Albers et al. Fifty-eight patients were divided into idiopathic (N = 40) and diabetic (N = 18) groups. These groups were compared for age, sex, cerebrospinal fluid protein, response to treatment, sensory response abnormalities, and TLI measurements using chi-square tests for binary and categorical variables and using t-tests and mixed-effects models for continuous variables. RESULTS: The difference in abnormal rates of sensory responses was significant for the sural nerve, with the idiopathic group having a lower rate than the diabetic group (80% vs. 100%, p < 0.001). No group differences in the TLI measurements were significant. CONCLUSIONS: Sural sensory responses may have some value in differentiating idiopathic CIDP from diabetic CIDP. Larger prospective studies are needed to confirm our findings. SIGNIFICANCE: Our study suggests that abnormal sural sensory potentials may have some significance in differentiating idiopathic CIDP from diabetic CIDP.

Suprascapular neuropathy: A review of 87 cases.

INTRODUCTION: Suprascapular neuropathy (SSN) is rare, with an estimated prevalence of 4.3% in patients with shoulder pain. METHODS: This retrospective chart review included patients with SSN seen during a 16-year period. Demographics and clinical information were recorded. Descriptive statistics, including percentages, means, and standard deviations, were computed for the variables of interest for all patients. RESULTS: Of 87 patients included in this study, trauma (n = 27) was the most common cause of SSN, followed by neuralgic amyotrophy (n = 21). Fifty-seven patients had isolated SSN. Others had SSN associated with axillary neuropathy (23 patients), brachial plexopathy (3 patients), and long thoracic, radial, or spinal accessory neuropathy (1 patient each). DISCUSSION: SSN is commonly associated with axillary neuropathy. Trauma remains the most common cause of SSN. Electrodiagnostic findings aid in the initial diagnosis and may indicate the need for close clinical follow-up based on the severity of the axonal injury.