Extensive Neurological Involvement Research Articles

PARESTHESIA, PAIN, AND ANOSMIA: AN UNCOMMON PRESENTATION OF LUNG CANCER

SESSION TITLE: Case Report Semifinalists 11 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: While paraneoplastic neurological disorders (PND) remain underreported in small cell lung cancer (SCLC), they have been most commonly associated with this tumor as compared with other forms of lung malignancy CASE PRESENTATION: An 84 year-old female with history of prior tobacco abuse presented following a fall, causing right hip fracture. She reported a 1 year history of debilitating symptoms of numbness and pain beginning in her feet, which gradually progressed to involve her distal lower extremities, and similar symptoms subsequently developing in her hands and progressing to involve both upper extremities and left shoulder accompanied by weakness in both upper and lower extremities, with subsequent loss of balance contributing to her fall. Additionally she reported total loss of smell, altered taste, early satiety, and an unintentional weight loss of 30 lbs. She had been evaluated in the outpatient setting and underwent an unremarkable upper endoscopy, colonoscopy, chest X-ray, and CT-scan of the abdomen/pelvis. An EMG demonstrated diffuse sensory polyneuropathy involving both upper and lower extremities. Prior to her presentation to the ED, serologic testing for a suspected paraneoplastic process was performed. On presentation, neurologic examination was notable for fluent speech, normal orientation and cognition, anosmia, normal cranial nerve II-XII testing, and diffuse bilateral upper and lower extremity weakness. Laboratory evaluation demonstrated normocytic anemia (HCT 31), otherwise unremarkable. During hospitalization, the paraneoplastic workup ordered by her neurologist returned as positive for ANNA-1 and CRMP-5-IgG titers. A CXR was repeated and demonstrated a well-circumscribed density in the superior left hilar region, new compared to prior imaging. CT chest demonstrated a 3.2 x 4.8 x 4.8 cm mass in the left hilar region, adjacent to the left mainstem bronchus with narrowing the left pulmonary artery, and a filling density within the left inferior pulmonary vein extending to the left atrium concerning for thrombosis or direct tumor extension. A PET/CT demonstrated a hypermetabolic left hilar mass and hypermetabolic pretracheal and bilateral hilar adenopathy. Biopsy demonstrated small cell lung cancer. DISCUSSION: We add to the literature a case of a diffuse sensory, motor and cranial neuropathy attributable to paraneoplastic neurologic syndrome secondary to SCLC. Lambert Eaton myasthenic syndrome and limbic encephalitis are the most commonly described PND associated with SCLC. Although peripheral sensory neuropathy is well described with SCLC, especially in the presence of positive onconeural antibodies (mainly ANNA-1 and CRMP-5), such extensive neurological involvement has rarely been described. CONCLUSIONS: This case highlights the need to consider early evaluation for underlying malignancy in patients with otherwise unexplained diffuse neurologic symptoms Reference #1: Gozzard, P., Woodhall, M., Chapman, C., Nibber, A., Waters, P., Vincent, A., Lang, B., Maddison, P. (2015). Paraneoplastic neurologic disorders in small cell lung carcinoma: A prospective study. Neurology, 85(3), 235-9 Reference #2: Raspotnig M., Vedeler C., Storstein A. (2015) Paraneoplastic neurological syndromes in lung cancer patients with or without onconeural antibodies. Journal of the Neurological Sciences, 348 (1-2) , pp. 41-45. Reference #3: Kanaji, N., Watanabe, N., Kita, N., Bandoh, S., Tadokoro, A., Ishii, T., Dobashi, H., Matsunaga, T. (2014). Paraneoplastic syndromes associated with lung cancer. World journal of clinical oncology, 5(3), 197-223 DISCLOSURES: No relevant relationships by Matthew Ferrantino, source=Web Response No relevant relationships by Sandrine Hanna, source=Web Response No relevant relationships by Tala Salem, source=Web Response

The neurological manifestations in 52 patients with primary Sjögren's syndrome

Objective: To summarize the neurological manifestations in patients with primary Sjögren's syndrome (pSS). Methods: A total of 68 patients were diagnosed as pSS in neurology department of Peking Union Medical College Hospital from March 2014 to February 2018, among whom sixteen cases were excluded due to modified final diagnoses of primary neurological diseases. Therefore 52 pSS patients with neurological involvement were enrolled and retrospectively analyzed. They were divided into two groups as extensive group in which both central and peripheral nervous system were involved, non-extensive group in which either central or peripheral nervous system was involved. Results: Neurological manifestations were presented as primary symptoms in 98.1%(51/52) patients, while 35 had neurological involvement as their only extraglandular manifestations. Thirteen cases were in extensive group. The other 39 in non-extensive group including 22 cases with only peripheral nervous system involved and 17 cases with only single central nervous system involved. Compared to non-extensive group, the proportion of woman patients [13/13 vs.71.8% (28/39), P=0.047], serum IgG level [17.73(11.11,22.41)g/L vs. 11.49(9.58,13.40)g/L, P=0.017] and positive rates of oligoclonal band (OB) in cerebral spinal fluid (CSF) [7/13 vs. 22.6%(7/31), P=0.042)] were significantly higher in extensive involvement group. Conclusions: Neurological manifestations in pSS patients could be extensive, both central and peripheral nervous system might be associated. Female patients, high serum IgG level and positive OB in CSF are risk factors of extensive neurological involvement, suggesting that the immune system may be generally over-stimulated.

Anaesthesia for Spinal Tumor in the Pregnant Patient: A Case Report

The spinal cord tumor being rare in pregnancy can cause serious problems continuing pregnancy Extensive neurological involvement which is rapidly progressive due to compression should be considered for immediate decompression We present a case of a patient in the th weeks of pregnancy who had paraparesis due to thoracic intradural extramedullary meningoma Surgical intervention was deemed necessary and was performed in the prone position The early operative treatment and appropriate anaesthetic procedure resulted in good clinical outcome with complete neurological recovery

Incontinencia Pigmenti: rara genodermatosis ligada con el cromosoma X. Reporte de un caso clínico

Incontinentia pigmenti (Bloch-Sulzberger Syndrome) is a rare genodermatosis. The disease is X-linked and the most common molecular defect includes mutations in the NEMO gene on chromosome Xq28 in approximately 70% of patients. It is characterized by a multisystem compromise in which the skin manifestations occur in all patients. Also, the dermatological findings occur early and in chronological order. The prevalence of extra skin compromise is approximately 80% of patients. The ophthalmological and neurological manifestations are the major cause of disability in patients and may have a great impact on the quality of life. Some of damages are irreversible. We present the clinical case of a child on her first day of life. It began with skin manifestations typical of incontinentia pigmenti and extensive neurological involvement. In addition, the family history includes a sister with the disease. A multidisciplinary approach is necessary to care for patients and to detect and prevent long-term complications.

The armadillo as an animal model and reservoir host for Mycobacterium leprae

Apart from humans, armadillos are the only known natural hosts of Mycobacterium leprae. They are well developed as hosts for in vivo propagation of M leprae and are advancing as models for studying the pathogenesis of leprosy and translational research. Armadillos are immunologically intact. They exhibit the full Ridley-Jopling spectrum of histopathologic responses to M leprae and uniquely manifest extensive neurological involvement that closely recapitulates human leprosy. In addition, free-ranging armadillos in some regions are known to harbor a naturally occurring infection with M leprae, and zoonotic transmission between armadillos and humans has been implicated in a large number of new case presentations. We review the role of the armadillo as a model for leprosy and reservoir for human infection.

The Armadillo as a Model for Peripheral Neuropathy in Leprosy

Leprosy (also known as Hansen's Disease) is a chronic infectious disease caused by Mycobacterium leprae that primarily targets the peripheral nervous system; skin, muscle, and other tissues are also affected. Other than humans, nine-banded armadillos (Dasypus novemcinctus) are the only natural hosts of M. leprae, and they are the only laboratory animals that develop extensive neurological involvement with this bacterium. Infection in the armadillo closely recapitulates many of the structural, physiological, and functional aspects of leprosy seen in humans. Armadillos can be useful models of leprosy for basic scientific investigations into the pathogenesis of leprosy neuropathy and its associated myopathies, as well as for translational research studies in piloting new diagnostic methods or therapeutic interventions. Practical and ethical constraints often limit investigation into human neuropathies, but armadillos are an abundant source of leprotic neurologic fibers. Studies with these animals may provide new insights into the mechanisms involved in leprosy that also might benefit the understanding of other demyelinating neuropathies. Although there is only a limited supply of armadillo-specific reagents, the armadillo whole genomic sequence has been completed, and gene expression studies can be employed. Clinical procedures, such as electrophysiological nerve conduction testing, provide a functional assessment of armadillo nerves. A variety of standard histopathological and immunopathological procedures including Epidermal Nerve Fiber Density (ENFD) analysis, Schwann Cell Density, and analysis for other conserved cellular markers can be used effectively with armadillos and will be briefly reviewed in this text.

The armadillo: a model for neuropathy of leprosy and potentially other neurodegenerative diseases

Leprosy (also known as Hansen’s disease) is an infectious peripheral neurological disorder caused by Mycobacterium leprae that even today leaves millions of individuals worldwide with life-long disabilities. The specific mechanisms by which this bacterium induces nerve injury remain largely unknown, mainly owing to ethical and practical limitations in obtaining affected human nerve samples. In addition to humans, nine-banded armadillos (Dasypus novemcinctus) are the only other natural host of M. leprae, and they develop a systemically disseminated disease with extensive neurological involvement. M. leprae is an obligate intracellular parasite that cannot be cultivated in vitro. Because of the heavy burdens of bacilli they harbor, nine-banded armadillos have become the organism of choice for propagating large quantities of M. leprae, and they are now advancing as models of leprosy pathogenesis and nerve damage. Although armadillos are exotic laboratory animals, the recently completed whole genome sequence for this animal is enabling researchers to undertake more sophisticated molecular studies and to develop armadillo-specific reagents. These advances will facilitate the use of armadillos in piloting new therapies and diagnostic regimens, and will provide new insights into the oldest known infectious neurodegenerative disorder.

Liver Failure in Mitochondrial DNA Depletion Syndrome: The Importance of Serial Neuroimaging in Liver Transplantation Evaluation

Liver Failure in Mitochondrial DNA Depletion Syndrome: The Importance of Serial Neuroimaging in Liver Transplantation Evaluation

Third nerve palsies.

The diagnosis and management of third nerve dysfunction varies according to the age of the patient, characteristics of the third nerve palsy, and presence of associated symptoms and signs. Indeed, third nerve palsies may be partial or complete, congenital or acquired, isolated or accompanied by signs of more extensive neurological involvement. They can result from lesions located anywhere from the oculomotor nucleus to the termination of the third nerve in the extra-ocular muscles within the orbit. Recent advances in noninvasive neuroimaging facilitate early diagnosis; however, management of a patient presenting with an isolated third nerve palsy remains a challenge.

Magnetic resonance imaging in a case of mumps postinfectious encephalitis with asymptomatic optic neuritis

A 5-year-old male patient with asymptomatic optic neuritis and mumps postinfectious encephalitis or acute disseminated encephalomyelitis is reported. Magnetic resonance imaging (MRI) with a short inversion time inversion recovery sequence was valuable in detecting clinically silent lesions of the unilateral right optic nerve in addition to visual evoked potentials. Evidence of concurrent optic neuritis was useful for detecting more extensive neurological involvement, leading to the diagnosis of mumps postinfectious encephalitis. A systematic MRI study should be performed in children with mumps encephalitis, regardless of appreciable clinical deficits.

Brain stem tumors of childhood and adolescence.

Forty children and adolescents showed clinical and radiographic evidence of brain stem tumors. The typical case is characterized by the insidious onset of cranial nerve involvement, pyramidal tract signs, ataxia, and behavioral changes without increased intracranial pressure. Of 28 patients who received radiation therapy, 21 (75%) showed clinical improvement. The average length of survival in the treated group was 47.2 months compared to 15.5 months in the untreated group. In general, patients who responded best to radiation therapy and survived longest were those with less extensive neurological involvement but longer duration of symptoms prior to diagnosis. In 26 pathologically verified cases, it appeared that the degree of tumor anaplasia was inversely related to survival time.