Prognostic value of cerebrospinal fluid analysis at the time of a first demyelinating event

Background and Objective: This study aimed to assess the value of cerebrospinal fluid (CSF) findings for predicting conversion to clinically definite multiple sclerosis (CDMS). Methods: From a database of 447 patients with a first demyelinating event, the records of 208 patients less than 51 years old who had baseline magnetic resonance imaging (MRI) and CSF examinations and a follow-up of at least 1 year were included. A multivariable Cox model was used to assess the short-term risk of CDMS according to baseline CSF findings after adjustment for prognostic factors (including brain MRI) and to provide a simple classification for predicting CDMS. Results: During a median follow-up of 3.5 years, 141 (67.8%) patients converted to CDMS. In multivariate analysis, younger age (hazard ratio [HR]: 1.44 [95% CI 1.02–2.01]), spatial dissemination on brain MRI (HR: 2.07 [95% CI 1.47–2.91]) and more than 4 WBC/mm3 in CSF (HR: 1.44 [95% CI 1.03–2.02]) were independently associated with CDMS. The Cox score obtained from these three predictors enabled patients to be divided into three groups with significant increased risks of CDMS at 1, 2 and 3 years; groups were classified as high-risk (64.7%, 77.4%, 96.1%), intermediate-risk (33.3%, 51.5%, 61.5%), and low-risk (11.1%, 18.3%, 40.3%). Conclusions: Age at onset, spatial dissemination on brain MRI and CSF white blood cell count are independently associated with short-term conversion to CDMS. The three proposed risk group classifications could be a useful tool to select patients for early therapeutic intervention.

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Predictors of short-term disease activity following a first clinical demyelinating event: analysis of the CHAMPS placebo groupy

Multiple Sclerosis Journal ◽

10.1191/1352458502ms825oa ◽

2002 ◽

Vol 8 (5) ◽

pp. 405-409 ◽

Cited By ~ 8

Author(s):

Keyword(s):

Disease Activity ◽

Brain Mri ◽

Brain Magnetic Resonance Imaging ◽

Event Analysis ◽

Short Term ◽

T2 Lesions ◽

Disease Modifying Therapy ◽

Increased Risk ◽

Definite Multiple Sclerosis ◽

Magnetic Resonance Imaging Mri

We evaluated 190 patients in the placebo group of the CHAMPS trial in order to assess factors associated with short-term clinical and brain magnetic resonance imaging (MRI) outcomes in patients with a first clinical demyelinating event involving the optic nerve, spinal cord, or brainstem/cerebellum, and subclinical demyelination on brain MRI. The two study outcomes were 1) development of clinically definite multiple sclerosis (CDMS) and 2) development of CDMS or two or more new or enlarging brain MRI T2 lesions. The presence of gadolinium (Gd)- enhancing lesions on the baseline scan was the only MRI characteristic associated with a higher risk of both the clinical and combined outcomes (p=0.003 and <0.001, respectively). The only demographic or clinical characteristic associated with an increased risk of these outcomes was younger age (p<0.001 for both outcomes). The lowest risk subgroups we could define had a 30% risk of CDMS and a 65% risk of the combined clinical/MRI outcome. Our results indicate that all patients presenting with a first demyelinating event who also have brain MRI evidence of subclinical demyelination have at least a moderate risk of short-term disease activity. This finding provides support for initiating disease-modifying therapy at the time of the first demyelinating event in patients meeting the CHAMPS enrollment criteria.

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Severity of Downgaze Palsy in the Context of Disease Duration Could Estimate Survival Duration in Patients With Progressive Supranuclear Palsy

Frontiers in Neurology ◽

10.3389/fneur.2021.736784 ◽

2021 ◽

Vol 12 ◽

Author(s):

Tao Xie ◽

Carlen A. Yuen ◽

Wenjun Kang ◽

Mahesh Padmanaban ◽

Timothy C. Hain ◽

...

Keyword(s):

Progressive Supranuclear Palsy ◽

Disease Duration ◽

Cox Model ◽

Brain Mri ◽

Age At Onset ◽

Unmet Need ◽

Survival Duration ◽

Total Survival ◽

Predicting Factors ◽

Clinical Counseling

It is an unmet need to estimate survival duration for patients with progressive supranuclear palsy (PSP). The objective of this study was to identify factors associated with the survival duration in patients with PSP. We followed up 23 patients with probable PSP-RS (Richardson syndrome) or PSP-P (parkinsonism) in our PSP center until death from 2011 to 2019. We prospectively and quantitatively rated their downgaze palsy whenever first noticed in our clinic. This was utilized along with the disease duration, motor function, medication use for parkinsonism, sex, age at onset of PSP, comorbid pulmonary and cardiovascular diseases, and the total survival duration from the onset of PSP to death for prediction analysis. A well-fitted linear regression model and a multivariant Cox model were applied to identify predicting factors for total survival duration. All patients had the specific hummingbird sign on brain MRI for PSP when downgaze palsy was documented. We found that the severity of downgaze palsy and the disease duration at the assessment were consistently correlated with the total survival duration in both models. The total survival duration could be further estimated by a formed regression equation. We conclude that severity and time to develop downgaze palsy could help to estimate the total survival duration in patients with probable PSP-RS and PSP-P, the major forms of PSP, which has significant clinical applications in clinical counseling and trial enrollment.

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Short-lasting unilateral neuralgiform headache with autonomic symptoms associated with idiopathic hypertrophic pachymeningitis

Cephalalgia Reports ◽

10.1177/2515816318790546 ◽

2018 ◽

Vol 1 ◽

pp. 251581631879054

Author(s):

J Matthijs Biesbroek ◽

Dirk R Rutgers ◽

Sander van Gulik ◽

Catherina JM Frijns

Keyword(s):

Brain Mri ◽

Resonance Imaging ◽

Hypertrophic Pachymeningitis ◽

Trigeminal Autonomic Cephalalgia ◽

Autonomic Symptoms ◽

Meningeal Inflammation ◽

Magnetic Resonance Imaging Mri ◽

Multiple Episodes ◽

Rule Out

Short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA) is a rare form of trigeminal autonomic cephalalgia. SUNA is frequently associated with a trigeminal neurovascular conflict and rarely occurs secondary to other intracranial pathology. We report a patient with SUNA that was associated with ipsilateral meningeal inflammation caused by idiopathic hypertrophic pachymeningitis (HP). During the 9-year follow-up, she experienced multiple episodes of SUNA, most of which occurred during exacerbations of the pachymeningitis. This is the third case of SUNA associated with HP reported in the literature. Based on this observation, we suggest that in patients presenting with SUNA, besides dedicated magnetic resonance imaging (MRI) of the trigeminal nerve, gadolinium-enhanced brain MRI should be performed to rule out HP.

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Onset of progressive motor impairment in patients with critical central nervous system demyelinating lesions

Multiple Sclerosis Journal ◽

10.1177/1352458520940983 ◽

2020 ◽

pp. 135245852094098

Author(s):

Roman M Kassa ◽

Elia Sechi ◽

Eoin P Flanagan ◽

Timothy J Kaufmann ◽

Orhun H Kantarci ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Brain Mri ◽

Age At Onset ◽

Motor Impairment ◽

Resonance Imaging ◽

Total Brain ◽

Demyelinating Lesions ◽

Magnetic Resonance Imaging Mri ◽

Brain And Spinal Cord

Objective: To compare progressive motor impairment onset attributable to a “critical” central nervous system (CNS) demyelinating lesion in patients with highly restricted versus unlimited magnetic resonance imaging (MRI) lesion burden. Methods: We identified 135 patients with progressive motor impairment for ⩾1 year attributable to a “critical” demyelinating lesion with: MRI burden of 1 lesion (“progressive solitary sclerosis”), 2–5 lesions (“progressive paucisclerosis”), or unrestricted (>5) lesions and “progressive unilateral hemiparesis.” Neuroradiology review of brain and spinal cord MRI documented unequivocally demyelinating lesions. Results: A total of 33 (24.4%) patients had progressive solitary sclerosis; 56 (41.5%) patients had progressive paucisclerosis; and 46 (34.1%) patients had progressive unilateral hemiparesis. Median age at onset of progressive motor impairment was younger in progressive solitary sclerosis (49 years; range 24–73) and progressive paucisclerosis (50 years; range 30–64) than in progressive unilateral hemiparesis (54 years; range 39–77; p = 0.02 and p = 0.003, respectively). Within progressive unilateral hemiparesis, motor-progression onset was similar between those with 4–10, 11–20, or >20 brain lesions (55, 54, 53 years of age, respectively; p = 0.44). Conclusion: Motor-progression age is similar, but paradoxically earlier, in cohorts with highly restricted CNS lesion burden than in those with unrestricted lesion burden with progressive unilateral hemiparetic MS. The “critical” demyelinating lesion rather than total brain MRI lesion burden is the major contributor to motor-progression onset in these cohorts.

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High-risk syndrome for neuromyelitis optica: a descriptive and comparative study

Multiple Sclerosis Journal ◽

10.1177/1352458510396923 ◽

2011 ◽

Vol 17 (6) ◽

pp. 720-724 ◽

Cited By ~ 10

Author(s):

N Collongues ◽

R Marignier ◽

H Zéphir ◽

F Blanc ◽

S Vukusic ◽

...

Keyword(s):

High Risk ◽

Optic Neuritis ◽

Neuromyelitis Optica ◽

Clinical Laboratory ◽

Age At Onset ◽

Igg Antibodies ◽

Resonance Imaging ◽

National Cohort ◽

Magnetic Resonance Imaging Mri

Background: Neuromyelitis optica (NMO) frequently begins with a monofocal episode of optic neuritis or myelitis. A concept named high-risk syndrome (HRS) for NMO has been proposed for patients with monofocal episodes and NMO-IgG antibodies. Objective: To describe HRS patients and compare them with NMO patients. Methods: We identified 30 patients with HRS: 18 with extensive myelitis (HRM) and 12 with optic neuritis (HRON), in a database pooling patients from 25 centres in France. Clinical, laboratory/magnetic resonance imaging (MRI) data and outcome were analysed and compared with a national cohort of 125 NMO patients extracted from the same database. Results: Mean follow-up was 4.8 years. Mean age at onset was 42.8 years (range: 12.4–70) with a female:male ratio of 0.9. Asymptomatic lesions were report on visual evoked potentials in 4/8 tested HRM patients and on spinal cord MRI in 2/7 HRON patients. Three patients died, two owing to a cervical lesion. HRS and NMO patients had similar clinical/paraclinical data, except for a predominance of men in the HRS group and a later mean age at onset in the HRM subgroup. Conclusion: The description of HRS patients is compatible with a monofocal form of NMO. Asymptomatic lesions could be included in a new set of NMO diagnostic criteria.

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Diagnostic performance of brain MRI in pharmacovigilance of natalizumab-treated MS patients

Multiple Sclerosis Journal ◽

10.1177/1352458515615225 ◽

2016 ◽

Vol 22 (9) ◽

pp. 1174-1183 ◽

Cited By ~ 15

Author(s):

Mike P Wattjes ◽

Martijn T Wijburg ◽

Anke Vennegoor ◽

Birgit I Witte ◽

Stefan D Roosendaal ◽

...

Keyword(s):

Sensitivity And Specificity ◽

Diagnostic Performance ◽

Brain Mri ◽

Lesion Detection ◽

Interrater Agreement ◽

Diagnostic Study ◽

Mri Scans ◽

Interrater Variability ◽

Magnetic Resonance Imaging Mri

Background: In natalizumab-treated multiple sclerosis (MS) patients, magnetic resonance imaging (MRI) is considered as a sensitive tool in detecting both MS disease activity and progressive multifocal leukoencephalopathy (PML). Objective: To investigate the performance of neuroradiologists using brain MRI in detecting new MS lesions and asymptomatic PML lesions and in differentiating between MS and PML lesions in natalizumab-treated MS patients. The secondary aim was to investigate interrater variability. Methods: In this retrospective diagnostic study, four blinded neuroradiologists assessed reference and follow-up brain MRI scans of 48 natalizumab-treated MS patients with new asymptomatic PML lesions ( n = 21) or new MS lesions ( n = 20) or no new lesions ( n = 7). Sensitivity and specificity for detection of new lesions in general (MS and PML lesions), MS and PML lesion differentiation, and PML detection were determined. Interrater agreement was calculated. Results: Overall sensitivity and specificity for the detection of new lesions, regardless of the nature of the lesions, were 77.4% and 89.3%, respectively; for PML-MS lesion differentiation, 74.2% and 84.7%, respectively; and for asymptomatic PML lesion detection, 59.5% and 91.7%, respectively. Interrater agreement for the tested categories was fair to moderate. Conclusion: The diagnostic performance of trained neuroradiologists using brain MRI in pharmacovigilance of natalizumab-treated MS patients is moderately good. Interrater agreement among trained readers is fair to moderate.

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A case of chronic progressive Lyme encephalitis as a manifestation of late Lyme neuroborreliosis

Infectious Disease Reports ◽

10.4081/idr.2014.5496 ◽

2014 ◽

Vol 6 (4) ◽

Cited By ~ 2

Author(s):

Vivek Verma ◽

Matthew Roman ◽

Disha Shah ◽

Marina Zaretskaya ◽

Mohamed H. Yassin

Keyword(s):

White Matter ◽

Short Term Memory ◽

Treatment Options ◽

Chronic Fatigue ◽

Short Term ◽

Mri Findings ◽

White Matter Changes ◽

Neurological Improvement ◽

Magnetic Resonance Imaging Mri

A 54-year-old female living in Europe presented with gait ataxia, dizziness, and bilateral hearing loss. Magnetic resonance imaging (MRI) revealed non-specific white matter changes. The patient’s condition gradually deteriorated over two years without diagnosis. The patient continued to decline cognitively and neurologically with worsening ataxia and upper motor neuron signs. Repeat MRI showed worsening white matter changes. Lumbar puncture, not previously done, showed positive Lyme testing. Treatment with intravenous ceftriaxone resulted in marked neurological improvement. Four years after symptom, the patient has short-term memory deficits and chronic fatigue, but is otherwise neurologically, cognitively, and functionally intact. Follow up MRI findings remain largely unchanged. Because cases of intraparenchymal or encephalopathic neuroborreliosis in America are lacking, so are treatment options. We present a rare case and discuss our experience with antibiotic treatment. This case lends evidence to define optimal treatment of this disease, imperative for hastening neurological recovery.

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Relationship between brain MRI lesion load and short-term disease evolution in non-disabling MS: a large-scale, multicentre study

Multiple Sclerosis Journal ◽

10.1177/1352458510388824 ◽

2010 ◽

Vol 17 (3) ◽

pp. 319-326 ◽

Cited By ~ 8

Author(s):

Marco Rovaris ◽

Maria A Rocca ◽

Frederik Barkhof ◽

Massimiliano Calabrese ◽

Nicola De Stefano ◽

...

Keyword(s):

Clinical Data ◽

Disease Duration ◽

Brain Mri ◽

Large Population ◽

Lesion Volume ◽

Disease Course ◽

Short Term ◽

Disease Evolution ◽

Edss Score

Background and Objectives: We evaluated clinical and conventional MRI features of a large population of patients with non-disabling MS to identify potential markers of a benign disease course. Methods: In seven MAGNIMS centres we retrospectively identified 182 patients with benign (B) MS (EDSS score ≤3.0, disease duration ≥15 years) and 187 patients with non-disabling relapsing–remitting MS (NDRRMS) (Expanded Disability Status Scale score ≤3.0, disease duration between 5 and 14 years), in whom clinical data were collected within two weeks from a brain T2-weighted scan. Brain T2 lesion volume (LV) was measured in all patients. In 146 BMS and 146 NDRRMS patients, clinical data were also available after a median follow up of 29 months (range: 7–104 months). Results: Mean LV was higher in BMS than in NDRRMS patients ( p < 0.001), but the mean ratio between LV and disease duration was higher in NDRRMS than in BMS patients (1.1 vs. 0.6 ml/year, p < 0.001). In BMS patients, brain LV was correlated with EDSS score increase at follow up ( r = 0.18, p = 0.03). Conclusions: An overall low rate of brain LV increase during a long-lasting disease course might be a feature of BMS. In BMS patients, a high brain LV might be associated with worsening of locomotor disability at short-term follow up.

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Increased cerebrospinal fluid albumin and immunoglobulin A fractions forecast cortical atrophy and longitudinal functional deterioration in relapsing-remitting multiple sclerosis

Multiple Sclerosis Journal ◽

10.1177/1352458517748474 ◽

2017 ◽

Vol 25 (3) ◽

pp. 338-343 ◽

Cited By ~ 3

Author(s):

Julia Kroth ◽

Dumitru Ciolac ◽

Vinzenz Fleischer ◽

Nabin Koirala ◽

Julia Krämer ◽

...

Keyword(s):

Multiple Sclerosis ◽

Cerebrospinal Fluid ◽

Functional Disability ◽

Immunoglobulin A ◽

Cortical Atrophy ◽

Disease Evolution ◽

Relapsing Remitting ◽

Magnetic Resonance Imaging Mri ◽

Relapsing Remitting Multiple Sclerosis

Background: Currently, no unequivocal predictors of disease evolution exist in patients with multiple sclerosis (MS). Cortical atrophy measurements are, however, closely associated with cumulative disability. Objective: Here, we aim to forecast longitudinal magnetic resonance imaging (MRI)-driven cortical atrophy and clinical disability from cerebrospinal fluid (CSF) markers. Methods: We analyzed CSF fractions of albumin and immunoglobulins (Ig) A, G, and M and their CSF to serum quotients. Results: Widespread atrophy was highly associated with increased baseline CSF concentrations and quotients of albumin and IgA. Patients with increased CSFIgA and CSFIgM showed higher functional disability at follow-up. Conclusion: CSF markers of blood–brain barrier integrity and specific immune response forecast emerging gray matter pathology and disease progression in MS.

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Diagnosing Ring-Enhancing Lesions in the Brain of a Patient With AIDS Without Brain Biopsy: A Case of Central Nervous System Histoplasmoma

The Neurohospitalist ◽

10.1177/1941874417725969 ◽

2017 ◽

Vol 8 (2) ◽

pp. 86-91 ◽

Cited By ~ 3

Author(s):

Rachel Beekman ◽

Jessica M. Hu ◽

Steven I. Aronin ◽

Maricar F. Malinis

Keyword(s):

Central Nervous System ◽

Cerebrospinal Fluid ◽

Nervous System ◽

Histoplasma Capsulatum ◽

Brain Mri ◽

Central Nervous System Involvement ◽

Brain Biopsy ◽

Empiric Therapy ◽

Fluid Analysis ◽

The Brain

We report a case of a Puerto Rican male with advanced AIDS who presented with multiple falls and pancytopenia. Magnetic resonance imaging (MRI) of the brain, as initial workup, revealed 2 ring-enhancing brain lesions. Initial cerebrospinal fluid analysis revealed minimal cells, mildly elevated protein, and no organism seen on gram stain. Due to prohibitive thrombocytopenia, brain biopsy was deferred. He had neither clinical nor radiographic improvement despite empiric therapy for both toxoplasmosis and bacterial abscesses. Indicated by pancytopenia, bone marrow (BM) aspiration was performed. Culture of BM aspirate grew Histoplasma capsulatum. Urine histoplasma antigen was markedly elevated. He was treated with liposomal amphotericin B (LamB) for progressive disseminated histoplasmosis with probable central nervous system involvement. Cerebrospinal fluid histoplasma antigen obtained after 2 months of LamB was detected. After prolonged course of LamB, he took itraconazole. Brain MRI at 7-month follow-up revealed significant improvement from baseline study.

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