scholarly journals One-and-a-half Syndrome Associated with Multiple Sclerosis in a 14-year-old Girl

2021 ◽  
Vol 62 (8) ◽  
pp. 1155-1159
Author(s):  
Yeon Ju Lim ◽  
Soo Jung Lee
Keyword(s):  
Multiple Sclerosis ◽  
Brain Magnetic Resonance Imaging ◽  
Underlying Disease ◽  
Oligoclonal Bands ◽  
Cerebral White Matter ◽  
Fluid Attenuated Inversion Recovery ◽  
Poor Outcomes ◽  
Intravenous Steroids ◽  
The Right ◽  
Mild Headache

Purpose: To report the case of a 14-year-old female patient with one-and-a-half syndrome subsequently diagnosed with multiple sclerosis involving the pons.Case summary: A 14-year-old girl without any underlying disease presented with difficulty focusing and mild headache for 5 days. The patient showed conjugate gaze palsy to the left, limited adduction in the left eye, and abducting nystagmus in the right eye, which indicated one-and-half syndrome. T2 fluid-attenuated inversion recovery brain magnetic resonance imaging revealed multiple punctate hyperintensities in the pontine tegmentum, bilateral cerebral white matter, and cerebellum. Cerebrospinal fluid examination revealed oligoclonal bands and multiple sclerosis was diagnosed. The patient was treated with intravenous steroids and beta-interferon. Seven weeks later, limitation of eyeball movement and nystagmus had resolved completely.Conclusions: At the young age of 14 years, a patient presenting with one-and-a-half syndrome was diagnosed with multiple sclerosis. Children with multiple sclerosis may experience severe physical and cognitive impairments, and brainstem involvement predicts an especially poor prognosis. Early diagnosis and active treatment may help to prevent poor outcomes.

scholarly journals Pediatric Multiple Sclerosis in Portugal: A Multicentre Study

2016 ◽  
Vol 29 (7-8) ◽  
pp. 425 ◽  
Author(s):  
Ana Sofia Correia ◽  
Luís Augusto ◽  
Joana Meireles ◽  
Joana Pinto ◽  
Ana Paula Sousa
Keyword(s):  
Multiple Sclerosis ◽  
Cerebrospinal Fluid ◽  
Brain Magnetic Resonance Imaging ◽  
Oligoclonal Bands ◽  
Childhood Onset ◽  
Multicentric Study ◽  
Pediatric Multiple Sclerosis ◽  
Adolescent Groups ◽  
Reported Data ◽  
Cerebrospinal Fluid Pleocytosis

Introduction: Multiple sclerosis is most often diagnosed among young adults but less frequently it may present during childhood or adolescence. In Portugal, there has been only one previous single-center, pediatric multiple sclerosis study. The main objective was the evaluation of the demographic, clinical, laboratorial and neuroradiological characteristics of patients with pediatric-onset multiple sclerosis in Portugal. The secondary objectives were to compare the characteristics of childhood-onset multiple sclerosis and adolescent-onset multiple sclerosis and to characterize the treatments prescribed.Material and Methods: We performed a retrospective observational, multicentric study. We reviewed data of all patients with multiple sclerosis younger than 18 years at the onset of their first multiple sclerosis symptoms.Results: There were 46 patients (72% female) included with a mean age at diagnosis of 16.1 years. Six cases had childhood-onset and 40 cases had adolescence-onset. The median value of Expanded Disability Status Scale was two. Relapsing-remitting multiple sclerosis was most prevalent (98% of cases). In the cerebrospinal fluid study, 74% of patients had positive oligoclonal bands. Brain magnetic resonance imaging studies showed a predominant supratentorial involvement (98% of cases), whereas the cervical segment was the most frequently affected in the spinal cord. All the patients enrolled in the study underwent immunomodulatory therapy, 75% ofpatients with beta-interferon. Concerning differences between the childhood and the adolescent groups, we found a greater proportion of male patients and of individuals with cerebrospinal fluid pleocytosis among the childhood-onset group.Discussion: This study provides new data on pediatric multiple sclerosis characteristics in Portugal and our results are similar to previously reported data in other parts of the worldConclusion: This is the first multicentric study characterizing pediatric multiple sclerosis in Portugal. The mechanisms underlying the particularities of pediatric multiple sclerosis remain largely unknown and further studies are required.

Paroxysmal kinesigenic dyskinesia–like phenotype in multiple sclerosis

2017 ◽  
Vol 23 (13) ◽  
pp. 1795-1797 ◽  
Author(s):  
Roxana Pop ◽  
Stefan Kipfer
Keyword(s):  
Multiple Sclerosis ◽  
Gene Mutations ◽  
Brain Magnetic Resonance Imaging ◽  
Acute Onset ◽  
Intravenous Steroid ◽  
Paroxysmal Kinesigenic Dyskinesia ◽  
Hyperkinetic Movement Disorder ◽  
Magnetic Resonance Imaging Mri ◽  
The Right ◽  
Rapid Regression

In April 2015, a 20-year-old woman with multiple sclerosis (MS) presented with acute onset of repetitive abnormal postures and choreatic movements of the right arm, precipitated by voluntary movements (online video 1 and 2). Brain magnetic resonance imaging (MRI) showed a new active MS lesion involving the basal ganglia on the left side (Figure 1(a)). Intravenous steroid treatment resulted in rapid regression of this paroxysmal kinesigenic dyskinesia (PKD)-like hyperkinetic movement disorder. The patient became asymptomatic within 3 months. PKD is characterized by recurrent uni- or bilateral choreoathetosis and usually represents an autosomal dominant inherited disorder caused by PRRT2 gene mutations. As in the present case, a PKD-like phenotype may be associated with MS relapses in presumably genetic negative cases.

A Septuagenarian With Progressive Hemiparesis

2021 ◽  
pp. 48-50
Author(s):  
Roman Kassa ◽  
B. Mark Keegan
Keyword(s):  
Multiple Sclerosis ◽  
Late Onset ◽  
Progressive Multiple Sclerosis ◽  
Oligoclonal Bands ◽  
Hyperintense Lesion ◽  
Fluid Analysis ◽  
Hemiparetic Gait ◽  
Magnetic Resonance Imaging Mri ◽  
Disease Modifying Agents ◽  
The Right

A 78-year-old man with no pertinent medical history sought care for an 18-month history of progressive right lower extremity weakness, gait impairment, and falls. On neurologic examination, he had a hemiparetic gait. He had normal higher cognitive function and cranial nerve function. Motor examination showed decreased bulk over the right hand with no fasciculations, mild spasticity over the right leg, and right hemiparesis with an upper motor neuron pattern. Deep tendon reflexes were brisk throughout his limbs, and he had an extensor plantar reflex on the right side. He had impaired vibratory sense at the toes, with otherwise normal sensory and coordination examinations. Magnetic resonance imaging (MRI) of the brain showed ovoid periventricular and punctate subcortical and deep white matter T2 hyperintense foci. Some of these had corresponding T1 hypointensity. MRI of the cervical spine showed 1 eccentrically located T2 hyperintense lesion over the right lateral aspect of C2. Cerebrospinal fluid analysis showed no pleocytosis, an increased protein concentration of 66 mg/dL, and 4 unique oligoclonal bands. A diagnosis of primary progressive multiple sclerosis, very late onset, was made. With any diagnosis of late-onset multiple sclerosis, a decision about whether multiple sclerosis disease-modifying agents are indicated should be carefully considered. Our older patient had a progressive disease course, and neuroimaging studies did not reveal evidence of active disease. Based on this, a decision was made to monitor him clinically and radiologically. Management of spasticity with regular daily stretching exercises was discussed with him. A first clinical manifestation of multiple sclerosis can occur at a later-than-typical age. Most studies consider an onset at age 50 years or older to be late-onset multiple sclerosis, whereas first symptoms occurring at age 60 years or older are commonly referred to as very late–onset MS.

Progressive Spasticity With a Single Magnetic Resonance Imaging Lesion

2021 ◽  
pp. 20-21
Author(s):  
Samantha A. Banks ◽  
Eoin P. Flanagan
Keyword(s):  
Magnetic Resonance Imaging ◽  
Multiple Sclerosis ◽  
Magnetic Resonance ◽  
Oligoclonal Bands ◽  
Resonance Imaging ◽  
Fluid Analysis ◽  
Skin Cancers ◽  
Clinical Episode ◽  
History Of ◽  
The Right

A 59-year-old White man with a history of excised basal and squamous cell skin cancers was evaluated for gait difficulties. He had erectile dysfunction but no bowel or bladder dysfunction. He also reported fatigue. He began using a cane for ambulation 2 weeks before evaluation at our facility. His medications included vitamin D and sildenafil. He was a lifelong nonsmoker and had no family history of multiple sclerosis. Neurologic examination at the time of our evaluation 3 years after onset was notable for a positive Hoffman sign on the right and mild weakness of the right triceps but preserved strength elsewhere. He had a spastic gait with moderate spasticity in both lower extremities, hyperreflexic patellar and ankle jerks bilaterally, and bilateral positive Babinski sign. The remainder of the examination was essentially normal. Magnetic resonance imaging of the brain showed a single lesion at the cervicomedullary junction and medullary pyramids, more prominent on the right. There was also some accompanying atrophy that was also visible on cervical spine magnetic resonance imaging. Results of cerebrospinal fluid analysis showed a normal white blood cell count, increased protein concentration (108 mg/dL), and positive oligoclonal bands. The progressive nature of his symptoms, spinal fluid results, and lesion appearance were all consistent with a diagnosis of progressive solitary sclerosis. At the time this patient was seen, no immunomodulatory medications for progressive solitary sclerosis were approved, so no immunomodulatory medication was tried. Ongoing symptomatic management was recommended. Progressive solitary sclerosis is a rare variant of multiple sclerosis in which patients have a single central nervous system demyelinating lesion and development of motor progression attributable to that lesion. Patients can initially have a clinical episode followed by progression or can have a progressive course without an initial relapse.

An uncommon first manifestation of multiple sclerosis: Tako-Tsubo cardiomyopathy

2016 ◽  
Vol 22 (6) ◽  
pp. 842-846 ◽  
Author(s):  
Luciana Midaglia ◽  
Jesús M Juega Mariño ◽  
Jaume Sastre-Garriga ◽  
Alex Rovira ◽  
Angela Vidal-Jordana ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Brain Magnetic Resonance Imaging ◽  
Oligoclonal Bands ◽  
Magnetic Resonance Imaging Scan ◽  
Intravenous Methylprednisolone ◽  
Ventricular Failure ◽  
Demyelinating Lesions ◽  
Tako Tsubo Cardiomyopathy ◽  
Excessive Secretion ◽  
Disease Modifying Treatment

Background: Clinically isolated syndromes affecting the brainstem may present with rare manifestations such as neurogenic pulmonary oedema (NPO). Objectives: We present the case of a 23 year-old man with NPO caused by Tako-Tsubo cardiomyopathy (TTC) as a first manifestation of multiple sclerosis (MS). Methods and Results: A brain magnetic resonance imaging scan at admission showed multiple supra and infratentorial white matter inflammatory demyelinating lesions. This examination was repeated 2 and 4 weeks after symptoms onset and active lesions showing contrast uptake were identified, two of them involving the pons and the medulla oblongata, probably affecting the solitary tract nucleus. Cerebrospinal fluid oligoclonal bands were detected. The patient was treated with a 3-day course of 1g intravenous methylprednisolone presenting a significant and progressive improvement. The proposed underlying physiopathology is an excessive secretion of catecholamines resulting in myocardial stunning and ventricular failure. Two months later he developed optic neuritis and disease-modifying treatment was initiated. Conclusions: Clinicians should consider a possible neurological origin of TTC, and according to the clinical characteristics of the patient, MS may be suspected.

Foreign accent syndrome as a first sign of multiple sclerosis

2009 ◽  
Vol 15 (9) ◽  
pp. 1123-1125 ◽  
Author(s):  
JB Chanson ◽  
S Kremer ◽  
F Blanc ◽  
C Marescaux ◽  
IJ Namer ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Right Hemisphere ◽  
Brain Mri ◽  
Photon Emission ◽  
Brain Magnetic Resonance Imaging ◽  
Foreign Accent ◽  
Oligoclonal Bands ◽  
Emission Computed Tomography ◽  
Initial Symptom ◽  
Foreign Accent Syndrome

Background Foreign accent syndrome (FAS) consists of a speech rhythm disorder different from dysarthia or aphasia. It is unusually met in multiple sclerosis (MS). Objective We report a case of FAS as an initial symptom of a MS. Methods A right-handed French woman developed an isolated German foreign accent. Brain magnetic resonance imaging (MRI), SPECT and analysis of CSF were performed. Results Brain MRI revealed a large hypersignal on T2-weighted images in the left prerolandic white matter. Single photon emission computed tomography showed a right prerolandic hypoperfusion. Unmatched oligoclonal bands in cerebrospinal fluid and occurrence of new abnormal hypersignals on the following MRI led us to diagnose MS. Conclusion FAS may be the first symptom of MS. It could result from extensive disturbances of brain function involving the right hemisphere.

Smoking is a risk factor for early conversion to clinically definite multiple sclerosis

2008 ◽  
Vol 14 (8) ◽  
pp. 1026-1030 ◽  
Author(s):  
F Di Pauli ◽  
M Reindl ◽  
R Ehling ◽  
F Schautzer ◽  
C Gneiss ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Risk Factor ◽  
Smoking Status ◽  
Brain Magnetic Resonance Imaging ◽  
Clinically Isolated Syndrome ◽  
Oligoclonal Bands ◽  
Time Interval ◽  
Increased Risk ◽  
Definite Multiple Sclerosis ◽  
Clinically Definite Multiple Sclerosis

Background Cigarette smoking increases the risk for development of multiple sclerosis and modifies the clinical course of the disease. In this study, we determined whether smoking is a risk factor for early conversion to clinically definite multiple sclerosis after a clinically isolated syndrome. Methods We included 129 patients with a clinically isolated syndrome, disseminated white-matter lesions on brain magnetic resonance imaging, and positive oligoclonal bands in the cerebrospinal fluid. The patients’ smoking status was obtained at the time of the clinically isolated syndrome. Results During a follow-up time of 36 months, 75% of smokers but only 51% of non-smokers developed clinically definite multiple sclerosis, and smokers had a significantly shorter time interval to their first relapse. The hazard ratio for progression to clinically definite multiple sclerosis was 1.8 (95% confidence interval, 1.2–2.8) for smokers compared with non-smokers ( P = 0.008). Conclusions Smoking is associated with an increased risk for early conversion to clinically definite multiple sclerosis after a clinically isolated syndrome, and our results suggest that smoking is an independent but modifiable risk factor for disease progression of multiple sclerosis. Therefore, it should be considered in the counseling of patients with a clinically isolated syndrome.

scholarly journals Bilateral internuclear ophthalmoplegia in multiple sclerosis of very late onset

2021 ◽  
Author(s):  
Guilherme Drumond Jardini Anastacio ◽  
Stella de Angelis Trivellato ◽  
Hendrick Henrique Fernandes Gramasco ◽  
Ana Claudia Pires Carvalho ◽  
Omar Gurrola Arambula ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Visual Acuity ◽  
Demyelinating Disease ◽  
Hip Prosthesis ◽  
Age Of Onset ◽  
Late Onset ◽  
Oligoclonal Bands ◽  
Internuclear Ophthalmoplegia ◽  
Lower Limb Pain ◽  
The Right

Context: Multiple sclerosis (MS) is the most common demyelinating disease of the central nervous system. The average age of onset is in the range of 28 to 31 years, but it can occur from the first year of life or after the seventh decade. If diagnosed over 60 years of age, it is called very late onset, with low prevalence. Bilateral internuclear ophthalmoplegia is a rare syndrome characterized by bilateral restriction of the adduction of the eyes in the conjugated horizontal gaze, which is generally secondary to demyelinating and vascular diseases. Case report: A 64-year-old man, brown, with a history of alcoholism, gout, hypothyroidism, arterial hypertension and generalized osteoarthritis with a right hip prosthesis with difficulty walking, presented a five-day subacute bilateral visual turbidity and binocular horizontal diplopia. On physical examination, he presented bilateral internuclear ophthalmoplegia, with a reduction in bilateral visual acuity worse on the right, in addition to lower limb pain pseudoparesis, with bilateral Babinski sign. Investigation with visual evoked potential demonstrated bilateral demyelinating optic neuropathy on the right, cerebrospinal fluid with more than two oligoclonal bands present, neuroaxis magnetic resonance imaging showing a change in the signal of the right optic nerve and multiple demyelinating lesions, fulfilling the criteria for dissemination in space and time. Therapy with Methylprednisolone 1g / day for 5 days was performed, with improvement of the condition, maintaining only visual sequelae with decreased visual acuity, and disease-modifying therapy was also started with stabilization of the condition. Conclusions: Despite being more rare, very late-onset MS should be considered as a diagnosis, even with singular presentations as in the case reported.

Hereditary Melkersson-Rosenthal syndrome and multiple sclerosis

2005 ◽  
Vol 11 (3) ◽  
pp. 364-366 ◽  
Author(s):  
J A Cabrera-Gómez ◽  
N Echazabal-Santana ◽  
Y Real-González ◽  
K Romero García ◽  
Manuel Junior Sobrinho ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Multiple Sclerosis ◽  
Facial Paralysis ◽  
Brain Magnetic Resonance Imaging ◽  
Oligoclonal Bands ◽  
Resonance Imaging ◽  
The Family ◽  
History Of ◽  
Magnetic Resonance Imaging Mri ◽  
Csf Oligoclonal Bands

The revision of MEDLINE from 1966 to 2003 did not report any association between multiple sclerosis (MS) and Melkersson—Rosenthal syndrome (MRS). This is a case report of a 51-year-old woman, with history of four recurrent Bell’s palsies. In 1999 she developed a right facial paralysis due to a supranuclear pyramidal lesion with right monoparesis. The family history showed five relatives with recurrent Bell’s paralysis and plicata tongue. Physical examination: right Bell’s paralysis, left supranuclear facial paralysis, furrowed tongue, right hemiparesis with pallor of the optic disks. Brain magnetic resonance imaging (MRI) demonstrated the typical lesions of MS and CSF oligoclonal bands. This is the first observation of a patient with hereditary MRS and MS. The link between both diseases is discussed.

scholarly journals Risk of Multiple Sclerosis after Idiopathic Optic Neuritis in a Pakistani Population

2010 ◽  
Vol 37 (2) ◽  
pp. 258-263 ◽  
Author(s):  
Syed Faraz Kazim ◽  
Muhammad Islam ◽  
Mustafa Khan ◽  
Bilal Hameed ◽  
Saad Shafqat
Keyword(s):  
Multiple Sclerosis ◽  
Optic Neuritis ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Oligoclonal Bands ◽  
Cumulative Probability ◽  
South Asian Population ◽  
Increased Risk ◽  
Risk Of Progression ◽  
Csf Oligoclonal Bands

Background and objective:Optic neuritis (ON) is associated with a 38% ten-year risk of developing multiple sclerosis (MS) in Western populations, but the corresponding risk in non-Western populations is unclear. We conducted this study to estimate the risk of progression to MS after an episode of ON in a South Asian population.Methods:Two hundred and fifty-three patients with idiopathic ON were identified by reviewing records of visual evoked potentials and chart notes from a single academic center spanning the years 1990-2007. A structured telephone interview was then conducted to identify patients who had subsequently received a diagnosis of MS. The diagnosis was corroborated from chart notes, where possible. Cumulative probability of conversion to MS was calculated using Kaplan-Meier survival analysis.Results:The five-year risk of developing MS was 14.6% and the ten-year risk was 24%. Patients (N=218) who had one or more typical demyelinating lesions on baseline brain magnetic resonance imaging (MRI) had a 68% 10-year risk; those with no lesions or non-typical lesions had a 14% risk (p<0.001). Female gender, recurrent ON, and occurrence of ON in winter months were also associated with increased risk (p≤ 0.001). Severity of ON and likelihood of detecting cerebrospinal fluid (CSF) oligoclonal bands were higher in patients who developed MS.Conclusion:Idiopathic ON in Pakistan carries a lower risk of progression to MS compared with Western data. As in Western populations, however, presence of abnormal baseline brain MRI and CSF oligoclonal bands correlate with increased MS risk.

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