A Fulminant Case of Demyelinating Encephalitis With Extensive Cortical Involvement Associated With Anti-MOG Antibodies

AbstractAcquired demyelinating syndromes (ADS) are frequently associated with myelin oligodendrocytes glycoprotein (MOG) antibodies in children. Clinical phenotypes are heterogeneous and may delay the diagnosis, especially when they relapse and are atypical, mimicking diseases such as multiple sclerosis or neuromyelitis optica spectrum disorders . Here, we describe two children: one with a progressive cognitive and behavioral deterioration with seizures after only one relapse and the other with similar clinical impairments associated with multiple relapses. Brain magnetic resonance imaging revealed a subsequent progressive leukodystrophy-like lesion with diffuse bilateral white matter injuries in both patients. Cerebrospinal fluid analysis showed pleiocytosis, increased level of proteins with no oligoclonal bands. Metabolic and inflammatory blood markers were all negative. Brain biopsy was performed in the second child and nonspecific inflammatory lesions with no argument for histiocytosis or tumor were observed. Clinical and radiological stabilization were obtained after active immunotherapy. Retrospective analysis of anti-MOG antibodies in these two children was positive at the earlier stage of the disease and turned negative after treatment and during follow-up. Leukodystrophy-like ADS with anti-MOG-antibodies may display distinct progressive phenotype and have a severe neurological prognosis. Early diagnosis and appropriate treatment may improve outcome in these children.

Download Full-text

Neuropathological Alzheimer’s Disease Lesions in Nasu-Hakola Disease with TREM2 Mutation: Atypical Distribution of Neurofibrillary Changes

Journal of Alzheimer s Disease ◽

10.3233/jad-201085 ◽

2021 ◽

Vol 79 (1) ◽

pp. 25-30

Author(s):

Emanuela Maderna ◽

Silvia Visonà ◽

Vittorio Bolcato ◽

Veronica Redaelli ◽

Paola Caroppo ◽

...

Keyword(s):

Alzheimer’S Disease ◽

Alzheimer's Disease ◽

Autosomal Recessive ◽

Cerebral Atrophy ◽

Autosomal Recessive Disorder ◽

Homozygous Mutation ◽

Neurofibrillary Changes ◽

Axonal Spheroids ◽

Temporal Structures ◽

Cortical Involvement

Nasu-Hakola disease is a rare autosomal recessive disorder associated to mutations in TREM2 and DAP12 genes, neuropathologically characterized by leukoencephalopathy with axonal spheroids. We report the neuropathologic findings of a 51-year-old female with a homozygous mutation (Q33X) of TREM2 gene. Beside severe cerebral atrophy and hallmarks of Nasu-Hakola disease, significant Alzheimer’s disease lesions were present. Neurofibrillary changes showed an atypical topographic distribution being severe at spots in the neocortex while sparing the mesial temporal structures. Our finding suggests that TREM2 genetic defects may favor Alzheimer’s disease pathology with neurofibrillary changes not following the hierarchical staging of cortical involvement identified by Braak.

Download Full-text

Acute demyelinating encephalitis after jequirity pea ingestion (Abrus precatorius)

Clinical Toxicology ◽

10.1080/15563650601006116 ◽

2007 ◽

Vol 45 (1) ◽

pp. 77-79 ◽

Cited By ~ 21

Author(s):

Vaibhav Sahni ◽

Satish K. Agarwal ◽

Narinder P. Singh ◽

Sunandan Sikdar

Keyword(s):

Abrus Precatorius ◽

Demyelinating Encephalitis

Download Full-text

Pregnancy in Patients With AQP4-Ab, MOG-Ab, or Double-Negative Neuromyelitis Optica Disorder

Neurology ◽

10.1212/wnl.0000000000011744 ◽

2021 ◽

Vol 96 (15) ◽

pp. e2006-e2015

Author(s):

Nicolas Collongues ◽

Cecilia Alves Do Rego ◽

Bertrand Bourre ◽

Damien Biotti ◽

Romain Marignier ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Previous History ◽

International Study ◽

Myelin Oligodendrocyte Glycoprotein ◽

Double Negative ◽

Neuromyelitis Optica Spectrum Disorder ◽

Pregnancy And Postpartum ◽

History Of ◽

Mog Antibodies ◽

The Mean

ObjectiveTo analyze the effects of pregnancy on neuromyelitis optica spectrum disorder (NMOSD) according to patients' serostatus and immunosuppressive therapy (IST).MethodsWe performed a retrospective multicenter international study on patients with NMOSD. Patients were tested for aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) antibodies (Ab). Informative pregnancies were reported when NMOSD onset occurred before or during pregnancy or up to 12 months postpartum. The mean annualized relapse rate (ARR) was calculated for the 12 months before conception, for each trimester of pregnancy, and postpartum. Events such as miscarriage, abortion, and preeclampsia were reported. IST was considered if taken in the 3 months before or during pregnancy.ResultsWe included 89 pregnancies (46 with AQP4-Ab, 30 with MOG-Ab, and 13 without either Ab) in 58 patients with NMOSD. Compared to the prepregnancy period, the ARR was lower during pregnancy in each serostatus group and higher during the postpartum period in patients with AQP4-Ab (p < 0.01). Forty-eight percent (n = 31) of pregnancies occurred during IST and these patients presented fewer relapses during pregnancy and the 12 months postpartum than untreated patients (26% vs 53%, p = 0.04). Miscarriages occurred in 10 (11%) pregnancies, and were mainly in patients with AQP4-Ab (with or without IST) and a previous history of miscarriage. Preeclampsia was reported in 2 (2%) patients who were AQP4-Ab-positive.ConclusionWe found a rebound in the ARR during the first postpartum trimester that was higher than the prepregnancy period only in AQP4-Ab-positive patients. Taking IST just before or during pregnancy reduces the risk of relapses in these conditions.

Download Full-text

Temporal Dynamics of MOG Antibodies in Children with Acquired Demyelinating Syndrome

10.1055/s-0041-1739665 ◽

2021 ◽

Author(s):

Eva-Maria Wendel ◽

Helen Sophie Thonke ◽

Annikki Bertolini ◽

Matthias Baumann ◽

Astrid Blaschek ◽

...

Keyword(s):

Temporal Dynamics ◽

Mog Antibodies

Download Full-text

Cortical Involvement in the Generation of Essential Tremor

Journal of Neurophysiology ◽

10.1152/jn.00477.2006 ◽

2007 ◽

Vol 97 (5) ◽

pp. 3219-3228 ◽

Cited By ~ 96

Author(s):

Jan Raethjen ◽

R. B. Govindan ◽

Florian Kopper ◽

M. Muthuraman ◽

Günther Deuschl

Keyword(s):

Essential Tremor ◽

Hot Spot ◽

Power Spectra ◽

Emg Activity ◽

Cortical Circuits ◽

Coherent Signals ◽

Cortical Motor ◽

Tremor Frequency ◽

Cortical Involvement

Conflicting results on the existence of tremor-related cortical activity in essential tremor (ET) have raised questions on the role of the cortex in tremor generation. Here we attempt to address these issues. We recorded 64 channel surface EEGs and EMGs from forearm muscles in 15 patients with definite ET. EEG and EMG power spectra, relative power of the rhythmic EMG activity, relative EEG power at the tremor frequency, and EEG–EMG and EEG–EEG coherence were calculated and their dynamics over time explored. Corticomuscular delay was studied using a new method for narrow-band coherent signals. Corticomuscular coherence in the contralateral central region at the tremor frequency was present in all patients in recordings with a relative tremor EMG power exceeding a certain level. However, the coherence was lost intermittently even with tremors far above this level. Physiological 15- to 30-Hz coherence was found consistently in 11 patients with significantly weaker EMG activity in this frequency range. A more frontal (mesial) hot spot was also intermittently coupled with the tremor and the central hot spot in five patients. Corticomuscular delays were compatible with transmission in fast corticospinal pathways and feedback of the tremor signal. Thus the tremor rhythm is intermittently relayed only in different cortical motor areas. We hypothesize that tremor oscillations build up in different subcortical and subcortico-cortical circuits only temporarily entraining each other.

Download Full-text