Unusual Symptoms and Syndromes in Multiple Sclerosis Rae-Grant, Alexander D
Continuum (Minneapolis, Minn.),
2013-August, 2013-Aug, 2013-08-00, 20130801, Letnik:
19, Številka:
4, Multiple Sclerosis
Journal Article
PURPOSE OF REVIEW:In multiple sclerosis (MS), symptoms vary widely from patient to patient. Certain events in MS are well recognized (eg, optic neuritis, brainstem and spinal cord relapses) and do ...not lead to much clinical confusion. However, other events that occur in MS may be less expected and may be underrecognized by some clinicians and may lead to an extensive and potentially unnecessary investigation for what is a known problem in MS.
RECENT FINDINGS:This article reviews Lhermitte sign, Pulfrich phenomenon, Uhthoff phenomenon, and the useless hand of Oppenheim, along with the underrecognized phenomena of transient neurologic events (including tonic spasms). Disorders of temperature regulation in MS (likely based on hypothalamic involvement) which can present with bizarre behavioral change and evade diagnosis, are also discussed. The article concludes with a review of epilepsy and sleep disorders in MS, both of which appear to occur at an increased frequency in the MS population and may have implications for therapy.
SUMMARY:This article is meant to help clinicians recognize and treat this fascinating set of underrecognized phenomena in MS and perhaps save patients trips to the emergency department, extraneous testing, and ineffective intervention.
OBJECTIVETo update the 2001 American Academy of Neurology (AAN) guideline on mild cognitive impairment (MCI).
METHODSThe guideline panel systematically reviewed MCI prevalence, prognosis, and ...treatment articles according to AAN evidence classification criteria, and based recommendations on evidence and modified Delphi consensus.
RESULTSMCI prevalence was 6.7% for ages 60–64, 8.4% for 65–69, 10.1% for 70–74, 14.8% for 75–79, and 25.2% for 80–84. Cumulative dementia incidence was 14.9% in individuals with MCI older than age 65 years followed for 2 years. No high-quality evidence exists to support pharmacologic treatments for MCI. In patients with MCI, exercise training (6 months) is likely to improve cognitive measures and cognitive training may improve cognitive measures.
MAJOR RECOMMENDATIONSClinicians should assess for MCI with validated tools in appropriate scenarios (Level B). Clinicians should evaluate patients with MCI for modifiable risk factors, assess for functional impairment, and assess for and treat behavioral/neuropsychiatric symptoms (Level B). Clinicians should monitor cognitive status of patients with MCI over time (Level B). Cognitively impairing medications should be discontinued where possible and behavioral symptoms treated (Level B). Clinicians may choose not to offer cholinesterase inhibitors (Level B); if offering, they must first discuss lack of evidence (Level A). Clinicians should recommend regular exercise (Level B). Clinicians may recommend cognitive training (Level C). Clinicians should discuss diagnosis, prognosis, long-term planning, and the lack of effective medicine options (Level B), and may discuss biomarker research with patients with MCI and families (Level C).
A syndrome of focal neurologic deficits with characteristic imaging features, acute encephalopathy, and seizures after cardiac and thoracic interventions has been previously briefly reported in the ...literature. In a retrospective observational study, we aim to identify the prevalence and characteristics of this syndrome, in addition to discussing the possible underlying pathophysiology.
In a retrospective study, we reviewed records of consecutive adult patients (≥18 years old) who underwent cardiac and thoracic procedures at a single institution between September 2014 to September 2019 and found to have evidence of focal cerebral edema following their procedure. We included and reported clinical course of patients who developed post-operative neurologic dysfunction and underwent magnetic resonance imaging (MRI) showing (1) asymmetric cerebral edema with (2) cortical diffusion restriction and (3) T2 cortical or subcortical hyperintensity and (4) no proximal vascular occlusion.
Three out of 107 patients (2.8%) met our inclusion criteria. These represented one male and two females with age at presentation of 63, 81 and 69, respectively. All patients developed severe neurologic impairment on the same day following their procedure (sternotomy with valve or bypass surgery in 2 patients; esophageal dilatation procedure in 1 patient). All patients underwent MRI of the brain and vessel imaging qualifying our inclusion criteria. Two patients improved neurologically prior to discharge, and one patient expired after family elected to withdraw care.
We present a series of cases with a rare syndrome after cardiac and thoracic interventions. Although the exact mechanism of this syndrome remains unclear, we believe it to be related to relative cerebral hyperperfusion and cerebral dysautoregulation following anesthesia and thoracic manipulation. Future studies should focus on understanding the true prevalence and pathophysiology of this syndrome.
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