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1.
BMC Neurol ; 23(1): 329, 2023 Sep 15.
Article in English | MEDLINE | ID: mdl-37715128

ABSTRACT

BACKGROUND: This is a unique case that describes the presentation, investigations, and disease trajectory of a fatal, clonal CD8-positive T-cell lymphoproliferative disorder in an otherwise healthy and immunocompetent patient with Epstein-Barr virus seronegative status. Central nervous system involving T-cell lymphoproliferative disorders are rare and typically encountered in the setting of immunocompromise. These disorders are often associated with aggressive cytomorphological features and characteristic magnetic resonance imaging patterns, which were not seen in this case. CASE PRESENTATION: Here we describe a case of a 65 year-old male presenting with neuropsychiatric symptoms, truncal ataxia, and falls who's bone marrow, cerebrospinal fluid, and brain biopsy were consistent with a clonal CD8-positive T-cell lymphoproliferative disorder that did not meet existing World Health Organization criteria for classification as T-cell lymphoma. The patient was treated with intrathecal methotrexate resulting in transient improvement of his symptoms followed by disease progression and death related to aspiration. CONCLUSIONS: This case highlights the importance of urgent and comprehensive work-up in patients with clinical features suggestive of lymphoma with central nervous system involvement, despite atypical imaging features and lack of cytomorphological features satisfying current World Health Organization classification criteria.


Subject(s)
Central Nervous System Neoplasms , Epstein-Barr Virus Infections , Lymphoproliferative Disorders , Neoplasms, Second Primary , Male , Humans , Aged , Herpesvirus 4, Human , CD8-Positive T-Lymphocytes
4.
Neurologist ; 22(4): 127-130, 2017 Jul.
Article in English | MEDLINE | ID: mdl-28644253

ABSTRACT

OBJECTIVE: To describe the spectrum of autonomic dysfunction in a uniformly evaluated cohort of patients with Sjögren syndrome. METHODS: A series of 13 patients underwent a comprehensive evaluation for suspected autonomic impairment, including a neurological examination, autonomic testing, and laboratory studies. A diagnosis of Sjögren syndrome was established as the cause of autonomic dysfunction in all. Clinical features, findings on autonomic testing, and laboratory results are described. RESULTS: All patients in this series reported postural lightheadedness and syncope or near-syncope. Autonomic testing confirmed the presence of orthostatic hypotension on tilt-table testing in 5 patients and an excessive postural tachycardia and/or hypertensive response in 8 patients. Only 2 of the patients with orthostatic hypotension had a significant sensory neuropathy. Symptoms suggestive of gastrointestinal and genitourinary impairment were seen in nearly all patients, with abnormal motility testing (most frequently esophageal dysmotility) in 5 of 6 patients who underwent formal testing. Patients in this series treated with immune-modulating therapy experienced significant improvement. CONCLUSIONS: A diagnosis of Sjögren syndrome should be aggressively pursued in patients with signs and symptoms suggestive of autonomic nervous system impairment. Although the spectrum of adrenergic failure is variable, ranging from orthostatic hypotension to an excessive postural tachycardia, most patients do have symptoms of more generalized autonomic failure. Patients who were treated with immune-modulating therapy did improve.


Subject(s)
Autonomic Nervous System Diseases/diagnosis , Autonomic Nervous System Diseases/etiology , Autonomic Nervous System Diseases/physiopathology , Sjogren's Syndrome/complications , Sjogren's Syndrome/diagnosis , Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult
5.
Neurol Clin Pract ; 7(6): 464-473, 2017 Dec.
Article in English | MEDLINE | ID: mdl-29431168

ABSTRACT

BACKGROUND: Sports concussion has an annual incidence of approximately 3.8 million. Over half go unreported and a substantial number may be asymptomatic. A rapid, cost-effective, and reliable tool that facilitates diagnosis of concussion is needed. The King-Devick (K-D) test is a vision-based tool of rapid number naming for assessment of concussion. In this study, we evaluated the utility of the K-D test in real time for identification of symptomatic concussion in youth athletes and to determine if similar impairment (subclinical concussion) exists in youth athletes without an obvious head injury or symptoms. METHODS: Youth hockey players underwent K-D testing preseason, postseason, and immediately after suspected concussion. Additional testing was performed in a subgroup of nonconcussed athletes immediately before and after a game to determine effects of fatigue on K-D scores. RESULTS: Among 141 players tested, 20 had clinically diagnosed concussion. All 20 had immediate postconcussion K-D times >5 seconds from baseline (average 7.3 seconds) and all but 2 had worse postseason scores (46.4 seconds vs 52.4 seconds, p < 0.05, Wilcoxon signed rank test). Nonconcussed athletes saw minimal improvement postseason (43.9 seconds vs 42.1 seconds, p < 0.05) and 51 nonconcussed players assessed before and after a game revealed no significant time change as a result of fatigue. CONCLUSIONS: Rapid number naming using the K-D test accurately identifies real-time, symptomatic concussion in youth athletes. Scores in concussed players may remain abnormal over time. Athletes should undergo preseason and postseason K-D testing, with additional evaluation real time to inform the assessment of suspected concussion. CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that the K-D test accurately identifies real-time concussions in youth athletes.

6.
Neurologist ; 19(5): 145-8, 2015 May.
Article in English | MEDLINE | ID: mdl-25970838

ABSTRACT

BACKGROUND: Immune therapies such as intravenous immunoglobulin (IVIG) and plasma exchange (PLEX) are first line in the treatment of worsening myasthenia gravis. Although PLEX is favored in myasthenic crisis, IVIG is increasingly used in exacerbations due to cost and ease of administration. OBJECTIVES: To review and critically assess current evidence on the effects of IVIG and PLEX on functional outcomes in patients with worsening myasthenia gravis. METHODS: A structured critical appraisal was conducted on the objective topic. This included a creation of a structured question based on a clinical scenario, comprehensive literature search, selection of evidence for review, and critical appraisal of selected evidence. Evidence was summarized and commentary provided. Participants included consultant and resident neurologists, a medical librarian, clinical epidemiologists, and content experts in the field of neuromuscular neurology. RESULTS: A single-blinded, randomized-controlled trial that compared IVIG and PLEX in 84 patients with worsening myasthenia gravis was selected for review. Primary outcome measure was functional status at 14 days after treatment, as assessed by the Quantitative Myasthenia Gravis Score. Change in Quantitative Myasthenia Gravis Score at day 14 for all subjects was 4.0, without statistically significant differences between IVIG and PLEX groups. CONCLUSIONS: IVIG and PLEX are equally effective in worsening myasthenia gravis. Treatment decisions may depend on several variables, including presence of respiratory distress, medical comorbidities, access to medication, and cost. PLEX will likely remain the treatment of choice in true myasthenic crisis.


Subject(s)
Immunoglobulins, Intravenous/therapeutic use , Immunologic Factors/therapeutic use , Myasthenia Gravis/therapy , Plasma Exchange/methods , Treatment Outcome , Adult , Aged , Analysis of Variance , Antibodies/blood , Electromyography , Female , Humans , MEDLINE/statistics & numerical data , Middle Aged , Myasthenia Gravis/physiopathology , Randomized Controlled Trials as Topic , Receptors, Cholinergic/immunology , Single-Blind Method , Time Factors , Young Adult
7.
Neurologist ; 18(6): 426-9, 2012 Nov.
Article in English | MEDLINE | ID: mdl-23114683

ABSTRACT

BACKGROUND: There is a well-known relationship between neurodegenerative disease, disrupted sleep, and cognition. Pathologic and imaging studies have shown that regions in the brain shown to regulate sleep and circadian rhythm are abnormal in Alzheimer disease. Most of these studies have been cross-sectional, and often look at patients already with dementia. This leaves uncertainty with regard to the temporal relationship of circadian disruption and cognitive decline. OBJECTIVE: To determine whether disrupted daytime activity and altered sleep patterns predict development of mild cognitive impairment (MCI) or dementia. METHODS: The objective was addressed through the development of a structured, critically-appraised topic. We incorporated a clinical scenario, background information, a structured question, literature search strategy, critical appraisal, results, evidence summary, commentary, and bottom line conclusions. Participants included consultant and resident neurologists, a medical librarian, and behavioral neurology and sleep medicine content experts. RESULTS: A prospective cohort study of 1282 cognitively normal women demonstrated that when peak circadian activity, as measured by wrist actigraphy, occurred later than average, there was an increased risk of MCI or dementia [odds ratio (OR), 1.83; 95% confidence interval (CI), 1.29-2.61]. Increased odds for dementia or MCI also existed for those with decreased circadian rhythm amplitude (OR, 1.57; 95% CI, 1.09-2.25) and robustness (OR, 1.57; 95% CI, 1.29-2.61). CONCLUSIONS: Disrupted circadian rhythm measures, including lower amplitude, a less robust rhythm, and delayed timing of peak activity on wrist actigraphy, were predictive of future development of MCI or dementia in cognitively normal women.


Subject(s)
Activities of Daily Living , Circadian Rhythm/physiology , Cognitive Dysfunction/diagnosis , Dementia/diagnosis , Sleep Disorders, Circadian Rhythm/complications , Aged , Cognitive Dysfunction/complications , Cognitive Dysfunction/physiopathology , Cohort Studies , Dementia/complications , Dementia/physiopathology , Disease Progression , Female , Humans , Predictive Value of Tests , Prognosis , Risk Factors , Sleep Disorders, Circadian Rhythm/physiopathology
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