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.

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.

scholarly journals Atypical Parkinsonism Revealing a Late Onset, Rigid and Akinetic Form of Huntington's Disease

2011 ◽  
Vol 2011 ◽  
pp. 1-3
Author(s):  
A. Ciammola ◽  
J. Sassone ◽  
B. Poletti ◽  
N. Mencacci ◽  
R. Benti ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neurodegenerative Disorder ◽  
Brain Mri ◽  
Photon Emission ◽  
Cag Repeats ◽  
Cortical Atrophy ◽  
Emission Computed Tomography ◽  
Initial Symptom ◽  
Caudate Nuclei

Huntington's disease (HD) is a rare hereditary neurodegenerative disorder characterized in over 90 percent of cases by chorea as the presenting motor symptom. We report a 54-year-old male who presented with Parkinsonism as the initial symptom of the disease. Genetic analysis revealed expansion of 40 CAG repeats, and brain MRI showed both severe caudate nuclei and cortical atrophy. Single-photon emission computed tomography (SPECT) imaging of the dopamine transporter showed nigrostriatal pathway degeneration. Here, we also describe his 2 years of clinical followup after ensuing dopaminergic stimulation.

Predicting multiple sclerosis at optic neuritis onset

2003 ◽  
Vol 9 (2) ◽  
pp. 135-141 ◽  
Author(s):  
Y P Jin ◽  
J de Pedro-Cuesta ◽  
Y H Huang ◽  
M Söderström
Keyword(s):  
Multiple Sclerosis ◽  
Optic Neuritis ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Oligoclonal Bands ◽  
Individual Risk ◽  
Relative Hazard ◽  
Hazard Ratios ◽  
Individual Probability

Using multivariate analyses, individual risk of clinically definite multiple sclerosis (C DMS) after monosymptomatic optic neuritis (MO N) was quantified in a prospective study with clinical MO N onset during 1990 -95 in Stockholm, Sweden. During a mean follow-up time of 3.8 years, the presence of MS-like brain magnetic resonance imaging (MRI) lesions and oligoclonal immunoglobulin (Ig) G bands in cerebrospinal fluid (CSF) were strong prognostic markers of C DMS, with relative hazard ratios of 4.68 {95% confidence interval (CI) 2.21 -9.91} and 5.39 (95% C I 1.56 -18.61), respectively. Age and season of clinical onset were also significant predictors, with relative hazard ratios of 1.76 (95% C I 1.02 -3.04) and 2.21 (95% C I 1.13 -3.98), respectively. Based on the above two strong predicto rs, individual probability of C DMS development after MO N was calculated in a three-quarter sample drawn from a cohort, with completion of follow-up at three years. The highest probability, 0.66 (95% C I 0.48 -0.80), was obtained for individuals presenting with three or more brain MRI lesions and oligoclonal bands in the C SF, and the lowest, 0.09 (95% C I 0.02 -0.32), for those not presenting with these traits. Medium values, 0.29 (95% C I 0.13 -0.53) and 0.32 (95% C I 0.07 -0.73), were obtained for individuals discordant for the presence of brain MRI lesions and oligoclonal bands in the C SF. These predictions were validated in an external one-quarter sample.

scholarly journals Shifting of global aphasia to Wernicke’s aphasia in a patient with intact motor function: a case report

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Ya-Chi Chuang ◽  
Chuan-Ching Liu ◽  
I-Ching Yu ◽  
Yu-Lin Tsai ◽  
Shin-Tsu Chang
Keyword(s):  
Computed Tomography ◽  
Right Hemisphere ◽  
Single Photon ◽  
Ct Perfusion ◽  
Single Case ◽  
Photon Emission ◽  
Emission Computed Tomography ◽  
Speech Fluency ◽  
Language Functions ◽  
The Right

Abstract Background Global aphasia without hemiparesis (GAWH) is a rare stroke syndrome characterized by the dissociation of motor and language functions. Here, we present a case of GAWH with the patient later regaining speech fluency. Case presentation A 73-year-old man was admitted to our emergency department immediately after an episode of syncope. On arrival, we noted his global aphasia but without any focal neurologic signs. Computed tomography (CT) perfusion scans showed a large hypodense region over his left perisylvian area. Under the impression of acute ischaemic stroke, he received recombinant tissue plasminogen activator (rtPA) injection and was treated as an inpatient. The patient was later discharged with GAWH status and received regular speech rehabilitation. After 14 months of rehabilitation, the patient gradually recovered his language expression ability. The degree of aphasia was evaluated with the Concise Chinese Aphasia Test (CCAT), and we obtained brain single photon emission computed tomography (SPECT) scans to assess cerebral blood flow. Conclusion A patient with severe impairments of Broca’s and Wernicke’s areas was able to talk fluently despite being unintelligible. SPECT revealed relative high level of radioactivity uptake in the right frontal lobe, suggesting the deficits in speech fluency could have been compensated by the right hemisphere. Although this is a single case demonstration, the results may strengthen the role of the right hemisphere in GAWH patients and suggests additional study that examines the possible benefits of stimulating activity at right homologous regions for recovering language function after global aphasia.

scholarly journals Frontal Hypoperfusion and the Effectiveness of Perampanel in Long-Lived Patient with Lafora Disease

2021 ◽  
pp. 211-217
Author(s):  
Koji Obara ◽  
Erika Abe ◽  
Itaru Toyoshima
Keyword(s):  
Single Photon ◽  
Early Stage ◽  
Photon Emission ◽  
Cerebellar Atrophy ◽  
Brain Magnetic Resonance Imaging ◽  
Emission Computed Tomography ◽  
Homozygous Mutation ◽  
Lafora Disease ◽  
Mental Deterioration ◽  
Exon 1

We report a long-lived patient with Lafora disease (LD). A 34-year-old woman experienced onset of seizures at the age of 11 years. She was bedridden in her early twenties due to frequent generalized tonic-clonic seizures, myoclonus, and progressive mental deterioration. Her seizures occurred all the time despite administration of multiple anticonvulsants at high doses. At the age of 31, she started perampanel, which resulted in reduction of anticonvulsants after her visible myoclonus and convulsions disappeared. Brain magnetic resonance imaging showed marked cerebral and cerebellar atrophy, and single-photon emission computed tomography using N-isopropyl-p-[123I] iodoamphetamine (IMP-SPECT) revealed significant hypoperfusion of the frontal lobe and cerebellum. We identified a W219R homozygous mutation in exon 1 of the NHLRC1 gene. Because perampanel may not only control seizures but also prevent mental deterioration in LD, we propose that perampanel should be administered from the early stage of LD.

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.

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.

Relevance of hypointense brain MRI lesions for long-term worsening of clinical disability in relapsing multiple sclerosis

2013 ◽  
Vol 20 (2) ◽  
pp. 214-219 ◽  
Author(s):  
Antonio Giorgio ◽  
Maria Laura Stromillo ◽  
Maria Letizia Bartolozzi ◽  
Francesca Rossi ◽  
Marco Battaglini ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Brain Mri ◽  
Univariate Analysis ◽  
Brain Lesion ◽  
Brain Magnetic Resonance Imaging ◽  
Stepwise Multiple Regression ◽  
Lesion Volume ◽  
Magnetic Resonance Imaging Mri ◽  
Relapsing Remitting Multiple Sclerosis

Background: The accrual of brain focal pathology is considered a good substrate of disability in relapsing–remitting multiple sclerosis (RRMS). However, knowledge on long-term lesion evolution and its relationship with disability progression is poor. Objective: The objective of this paper is to evaluate in RRMS the long-term clinical relevance of brain lesion evolution. Methods: In 58 RRMS patients we acquired, using the same scanner and protocol, brain magnetic resonance imaging (MRI) at baseline and 10±0.5 years later. MRI data were correlated with disability changes as measured by the Expanded Disability Status Scale (EDSS). Results: The annualized 10-year lesion volume (LV) growth was +0.25±0.5 cm3 (+6.7±8.7%) for T2-weighted (T2-W) lesions and +0.20±0.31 cm3 (+11.5±12.3%) for T1-weighted (T1-W) lesions. The univariate analysis showed moderate correlations between baseline MRI measures and EDSS at 10 years ( p < 0.001). Also, 10-year EDSS worsening correlated with LV growth and the number of new/enlarging lesions measured over the same period ( p < 0.005). In the stepwise multiple regression analysis, EDSS worsening over 10 years was best correlated with the combination of baseline T1-W lesion count and increasing T1-W LV ( R = 0.61, p < 0.001). Conclusion: In RRMS patients, long-term brain lesion accrual is associated with worsening in clinical disability. This is particularly true for hypointense, destructive lesions.

Factors predicting incomplete recovery from relapses in multiple sclerosis: a prospective study

2008 ◽  
Vol 14 (4) ◽  
pp. 485-493 ◽  
Author(s):  
MA Leone ◽  
S. Bonissoni ◽  
L. Collimedaglia ◽  
F. Tesser ◽  
S. Calzoni ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Brain Mri ◽  
Total Duration ◽  
Oligoclonal Bands ◽  
Relapsing Remitting ◽  
Environmental Background ◽  
Maximum Improvement ◽  
A Prospective Study ◽  
Relapsing Remitting Multiple Sclerosis ◽  
Speed Of Onset

Objective To prospectively evaluate predictors of incomplete recovery after the first attacks in a cohort of patients with clinically isolated syndrome or relapsing—remitting multiple sclerosis. Methods Seventy-two consecutive patients recruited from January 2001 to December 2003, evaluated every six months or at any relapse up to 31 July 2005. Relapse intervals were calculated from the date of onset, nadir, onset of improvement and maximum improvement. Predictive factors analysed were relapse-related (age at relapse onset, season and severity of the relapse, type of symptoms, speed of onset, plateau and total duration, number of affected Functional systems, preceding infections) and individual-related (gender, age at first attack, season of birth and first attack, characteristics of first brain MRI and cerebrospinal fluid oligoclonal bands, Link Index, IgG). Results We counted 209 attacks: 44 (21%) left mild sequelae, and 27 (13%) severe. The highest probability of sequelae was associated with sphincteric symptoms (9/20; 45%), followed by sensitive (38/113; 34%), motor (20/84; 24%), visual (13/61; 21%), cerebellar (4/24; 17%), brainstem (5/44; 11%) and others (0/6) ( P 0.005). Four variables were still relevant to predict sequelae after multivariate analysis: mild, moderate or severe relapses versus very mild (Odds ratio = 17.2, 95% confidence limits = 2.2—136.4), intermediate or long relapses versus short (3.2, 1.5—6.9), age ≥ 30 at relapse onset (2.9, 1.5—5.7) and bi-polysymptomatic versus monosymptomatic (2.2, 1.1—4.3). Conclusions Factors predicting incomplete recovery are more closely linked to the characteristics of the single relapse (extension and duration of tissue damage) than to the patient's genetic and environmental background. Multiple Sclerosis 2008; 14: 485—493. http://msj.sagepub.com

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.

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