De novo aphasic status epilepticus: finally making the diagnosis by long-term EEG

OBJECTIVEThe authors conducted a study to ascertain the long-term durability of coiled aneurysms completely occluded at 36 months’ follow-up given the potential for delayed recanalization.METHODSIn this retrospective review, the authors examined 299 patients with 339 aneurysms, all shown to be completely occluded at 36 months on follow-up images obtained between 2011 and 2013. Medical records and radiological data acquired during the extended monitoring period (mean 74.3 ± 22.5 months) were retrieved, and the authors analyzed the incidence of (including mean annual risk) and risk factors for delayed recanalization.RESULTSA total of 5 coiled aneurysms (1.5%) occluded completely at 36 months showed recanalization (0.46% per aneurysm-year) during the long-term surveillance period (1081.9 aneurysm-years), 2 surfacing within 60 months and 3 developing thereafter. Four showed minor recanalization, with only one instance of major recanalization. The latter involved the posterior communicating artery as an apparent de novo lesion, arising at the neck of a firmly coiled sac, and was unrelated to coil compaction or growth. Additional embolization was undertaken. In a multivariate analysis, a second embolization for a recurrent aneurysm (HR = 22.088, p = 0.003) independently correlated with delayed recanalization.CONCLUSIONSAlmost all coiled aneurysms (98.5%) showing complete occlusion at 36 months postembolization proved to be stable during extended observation. However, recurrent aneurysms were predisposed to delayed recanalization. Given the low probability yet seriousness of delayed recanalization and the possibility of de novo aneurysm formation, careful monitoring may be still considered in this setting but at less frequent intervals beyond 36 months.

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Correction to: Clinical benefit of glasdegib plus low-dose cytarabine in patients with de novo and secondary acute myeloid leukemia: long-term analysis of a phase II randomized trial

Annals of Hematology ◽

10.1007/s00277-021-04545-5 ◽

2021 ◽

Author(s):

Michael Heuser ◽

B. Douglas Smith ◽

Walter Fiedler ◽

Mikkael A. Sekeres ◽

Pau Montesinos ◽

...

Keyword(s):

Acute Myeloid Leukemia ◽

Clinical Benefit ◽

Myeloid Leukemia ◽

Low Dose ◽

De Novo ◽

Secondary Acute Myeloid Leukemia ◽

Term Analysis ◽

Low Dose Cytarabine ◽

Acute Myeloid

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Long-term outcomes of fully covered self-expandable metal stents versus plastic stents in chronic pancreatitis

Scientific Reports ◽

10.1038/s41598-021-94726-z ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Sang Hoon Lee ◽

Yeon Suk Kim ◽

Eui Joo Kim ◽

Hee Seung Lee ◽

Jeong Youp Park ◽

...

Keyword(s):

Chronic Pancreatitis ◽

Clinical Efficacy ◽

De Novo ◽

Plastic Stents ◽

Metal Stents ◽

Plastic Stent ◽

Sems Group ◽

Spontaneous Migration

AbstractChronic pancreatitis (CP) related main pancreatic duct (MPD) stricture has been a challenge for endoscopists. Fully covered self-expandable metal stents (FC-SEMS) has been tried in CP patients, but the efficacy and safety are still controversial. Thus, we aim to compare the long-term clinical efficacy of FC-SEMS vs. plastic stent placement in persistent MPD strictures secondary to CP. Between 2007 and 2018, 80 chronic pancreatitis patients (58 males, median age 49 years), who underwent endoscopic placement of FC-SEMS (n = 26) and plastic stent (n = 54) for persistent MPD strictures after at least 3 months of initial single plastic stenting, were retrospectively analyzed during a median follow-up duration of 33.7 months. As a result, MPD stricture resolution rate was statistically higher in FC-SEMS group (87.0% vs. 42.0%, p < 0.001). Although immediate complications occurred similarly (38.5% vs. 37.0%, p = 0.902), spontaneous migration (26.9%) and de novo strictures (23.1%) were pronounced delayed complications in FC-SEMS group. Pain relief during follow-up was significantly higher in FC-SEMS group (76.9% vs. 53.7%, p = 0.046). The total procedure cost was similar in both groups ($1,455.6 vs. $1,596.9, p = 0.486). In comparison with plastic stent, FC-SEMS placement for persistent MPD strictures had favorable long-term clinical efficacy, with its typical complications like spontaneous migration and de novo strictures.

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Clinical benefit of glasdegib plus low-dose cytarabine in patients with de novo and secondary acute myeloid leukemia: long-term analysis of a phase II randomized trial

Annals of Hematology ◽

10.1007/s00277-021-04465-4 ◽

2021 ◽

Author(s):

Michael Heuser ◽

B. Douglas Smith ◽

Walter Fiedler ◽

Mikkael A. Sekeres ◽

Pau Montesinos ◽

...

Keyword(s):

Acute Myeloid Leukemia ◽

Phase Ii ◽

Clinical Benefit ◽

Myeloid Leukemia ◽

Low Dose ◽

De Novo ◽

Secondary Acute Myeloid Leukemia ◽

Low Dose Cytarabine ◽

Acute Myeloid

AbstractThis analysis from the phase II BRIGHT AML 1003 trial reports the long-term efficacy and safety of glasdegib + low-dose cytarabine (LDAC) in patients with acute myeloid leukemia ineligible for intensive chemotherapy. The multicenter, open-label study randomized (2:1) patients to receive glasdegib + LDAC (de novo, n = 38; secondary acute myeloid leukemia, n = 40) or LDAC alone (de novo, n = 18; secondary acute myeloid leukemia, n = 20). At the time of analysis, 90% of patients had died, with the longest follow-up since randomization 36 months. The combination of glasdegib and LDAC conferred superior overall survival (OS) versus LDAC alone; hazard ratio (HR) 0.495; (95% confidence interval [CI] 0.325–0.752); p = 0.0004; median OS was 8.3 versus 4.3 months. Improvement in OS was consistent across cytogenetic risk groups. In a post-hoc subgroup analysis, a survival trend with glasdegib + LDAC was observed in patients with de novo acute myeloid leukemia (HR 0.720; 95% CI 0.395–1.312; p = 0.14; median OS 6.6 vs 4.3 months) and secondary acute myeloid leukemia (HR 0.287; 95% CI 0.151–0.548; p < 0.0001; median OS 9.1 vs 4.1 months). The incidence of adverse events in the glasdegib + LDAC arm decreased after 90 days’ therapy: 83.7% versus 98.7% during the first 90 days. Glasdegib + LDAC versus LDAC alone continued to demonstrate superior OS in patients with acute myeloid leukemia; the clinical benefit with glasdegib + LDAC was particularly prominent in patients with secondary acute myeloid leukemia. ClinicalTrials.gov identifier: NCT01546038.

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Malpigmentation of Common Sole (Solea solea) during Metamorphosis Is Associated with Differential Synaptic-Related Gene Expression

Animals ◽

10.3390/ani11082273 ◽

2021 ◽

Vol 11 (8) ◽

pp. 2273

Author(s):

Menelaos Kavouras ◽

Emmanouil E. Malandrakis ◽

Ewout Blom ◽

Kyriaki Tsilika ◽

Theodoros Danis ◽

...

Keyword(s):

Gene Expression ◽

Nervous System ◽

De Novo ◽

Close Association ◽

Ionic Channels ◽

Long Term Potentiation ◽

General Term ◽

The Difference ◽

Common Sole

In farmed flatfish, such as common sole, color disturbances are common. Dyschromia is a general term that includes the color defects on the blind and ocular sides of the fish. The purpose was to examine the difference in gene expression between normal pigmented and juveniles who present ambicoloration. The analysis was carried out with next-generation sequencing techniques and de novo assembly of the transcriptome. Transcripts that showed significant differences (FDR < 0.05) in the expression between the two groups, were related to those of zebrafish (Danio rerio), functionally identified, and classified into categories of the gene ontology. The results revealed that ambicolorated juveniles exhibit a divergent function, mainly of the central nervous system at the synaptic level, as well as the ionic channels. The close association of chromophore cells with the growth of nerve cells and the nervous system was recorded. The pathway, glutamate binding–activation of AMPA and NMDA receptors–long-term stimulation of postsynaptic potential–LTP (long term potentiation)–plasticity of synapses, appears to be affected. In addition, the development of synapses also seems to be affected by the interaction of the LGI (leucine-rich glioma inactivated) protein family with the ADAM (a disintegrin and metalloprotease) ones.

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Long‐Term Follow‐Up of a Patient with a De Novo p. Arg769Cys Mutation in the ATP1A3 Gene

Movement Disorders Clinical Practice ◽

10.1002/mdc3.13332 ◽

2021 ◽

Author(s):

Anjali Chouksey ◽

Asish Vijayaraghavan ◽

Sony Mohan ◽

Srija Inturi ◽

A.T. Prabhakar ◽

...

Keyword(s):

De Novo ◽

Long Term Follow Up ◽

Atp1a3 Gene

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Status epilepticus from GABABR antibody positive encephalitis due to de novo mixed small cell and adenocarcinoma of the prostate

BMJ Case Reports ◽

10.1136/bcr-2020-238172 ◽

2020 ◽

Vol 13 (11) ◽

pp. e238172 ◽

Cited By ~ 1

Author(s):

Christopher Kwan ◽

Aaron Sia ◽

Cullen O'Gorman

Keyword(s):

Status Epilepticus ◽

De Novo ◽

Locally Advanced ◽

Small Cell ◽

Gamma Aminobutyric Acid ◽

Transrectal Biopsy ◽

The Status ◽

Adenocarcinoma Of The Prostate ◽

Receptor Antibodies

We present a case study of a 67-year-old man who presented with a new onset of recurrent tonic-clonic seizures. He had tested positive to gamma-aminobutyric acid B receptor antibodies in his blood and cerebrospinal fluid, and subsequent CT imaging and transrectal biopsy confirmed the presence of a locally advanced mixed small cell and Gleason 9 adenocarcinoma of the prostate. His seizures remained resistant to treatment with multiple antiepileptic drugs, including sodium valproate, clobazam, topiramate, carbamazepine, phenytoin and lacosamide. He progressed to status epilepticus, which required intravenous immunoglobulin and steroids, followed by plasma exchange 1 week later. The status epilepticus was refractory and required multiple admissions to the intensive care unit.

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A De Novo Dominant Negative Mutation in DNM1L Causes Sudden Onset Status Epilepticus with Subsequent Epileptic Encephalopathy

Neuropediatrics ◽

10.1055/s-0039-1685217 ◽

2019 ◽

Vol 50 (03) ◽

pp. 197-201

Author(s):

S. Schmid ◽

M. Wagner ◽

C. Goetz ◽

C. Makowski ◽

P. Freisinger ◽

...

Keyword(s):

Status Epilepticus ◽

Missense Mutation ◽

De Novo ◽

Mitochondrial Fission ◽

Refractory Status Epilepticus ◽

Sudden Onset ◽

Epileptic Encephalopathy ◽

Neurologic Outcome ◽

Dominant Negative Mutation ◽

Refractory Status

AbstractMitochondrial dynamics such as fission and fusion play a vital role in normal brain development and neuronal activity. DNM1L encodes a dynamin-related protein 1 (Drp1), which is a GTPase essential for proper mitochondrial fission. The clinical phenotype of DNM1L mutations depends on the degree of mitochondrial fission deficiency, ranging from severe encephalopathy and death shortly after birth to initially normal development and then sudden onset of refractory status epilepticus with very poor neurologic outcome. We describe a case of a previously healthy 3-year-old boy with a mild delay in speech development until the acute onset of a refractory status epilepticus with subsequent epileptic encephalopathy and very poor neurologic outcome. The de novo missense mutation in DNM1L (c.1207C > T, p.R403C), which we identified in this case, seems to determine a unique clinical course, strikingly similar to four previously described patients in literature with the identical de novo heterozygous missense mutation in DNM1L.

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