Categories
Developmental Epilepsy

How promising is sirolimus for epilepsy in tuberous sclerosis?

Sirolimus improves seizure control in pediatric patients with tuberous sclerosis: a prospective cohort study.

He W, Chen J, Wang YY, et al.

Seizure 2020; 79:20-26.

Abstract

Background

This study aimed to analyze the therapeutic effect of sirolimus on seizures in pediatric patients with tuberous sclerosis.

Methods

We first compared the efficacy of controlling seizures in all patients after they had taken sirolimus for one year, and then we performed a subgroup analysis based on whether the administered antiepileptic drugs were changed to determine whether the efficacy was associated with changes of antiepileptic drugs.

Results

A total of 91 eligible children were enrolled. The response rate was 78.0 % (71/91), and 47.2 % (43/91) of all patients were became seizure-free. The improvement in seizure control before and after treatment with sirolimus was significant (p < 0.001). In the AEDs unaltered group, 34 were responders (34/45, 75.6 %, 95 % CI 17.4-88.3), of which 24 were seizure-free (24/34, 70.6 %). In the AEDs-altered group, 37 were responders (37/46, 80.4 %, 95 % CI 56.7-88.1), of which 19 were seizure-free (19/37, 51.4 %). There was no significant difference between the two groups for reductions in rate of seizure frequency (p = 0.308). In the patients with refractory epilepsy, treatment with sirolimus was also effective (p = 0.01). Logistic regression analysis showed that age was an important factor affecting outcome of epilepsy (p = 0.003, 95 % CI 2.05-38.31). No Grade 3 or 4 adverse events were noted during the follow-up.

Conclusions

Sirolimus has a significant effect on seizures associated with tuberous sclerosis complex (TSC), with no or only moderate adverse events after long-term administration. Sirolimus could be used as the first-line medication for pediatric patients with TSC-associated epilepsy.

This paper is cited in the Neurochecklist:

Tuberous sclerosis complex (TSC): treatment

Abstract link

By Own work. – From <a href=”https://en.wikipedia.org/wiki/Protein_Data_Bank&#8221; class=”extiw” title=”w:Protein Data Bank”>PDB</a> entry <a rel=”nofollow” class=”external text” href=”http://www.rcsb.org/pdb/cgi/explore.cgi?pdbId=1FAP”>1FAP</a&gt;., Public Domain, Link
Categories
Neurochecklists updates

The remarkable scope of neurology…in 83 revealing numbers

Exploring Neurochecklists is a revealing experience…

It always highlights the expansive range of neurological disorders.

To illustrate this extensive breadth and scope of neurology…

Here are 83 striking numbers..all linked to their checklists.

83. Numbers in a city: New Haven, CT. See Ming-Lee on Flickr https://www.flickr.com/photos/seeminglee/142117353

11 neurological complications of aortic dissection

11 causes of normal MRI scan myelopathy

11 causes of supranuclear gaze palsy

13 distinctive stroke subtypes

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13 MRI signs of NPH

13 causes of pulsatile tinnitus

15 risk factors of NPH

16 causes of reverse Horner’s syndrome

16 types and variants of multiple sclerosis

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16 drug mimics of IIH

16 risk factors of cerebral vein thrombosis

16 modifiable risk factors of multiple sclerosis

16 MRI features of IIH

17 causes of bilateral Horner’s syndrome

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17 radiological mimics of meningioma

17 causes of myopathy with respiratory failure

17 neurological manifestations of SLE

18 neurological manifestations of mitochondrial disorders

18 CT mimics of subarachnoid haemorrhage

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19 causes of foot drop

19 causes of Horner’s syndrome

20 causes of subarachnoid haemorrhage

20 major causes of stroke in pregnancy

20 risk factors of osmotic demyelination disorder

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21 neurological manifestations of systemic sclerosis

21 causes of self-mutilation

22 neurological manifestations of antiphospholipid syndrome

22 neurological complications of renal dialysis

22 causes of Othello syndrome

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23 presentations of uraemic encephalopathy

23 causes of rapidly progressive weakness

23 causes of muscle hypertrophy 

23 risk factors of delirium

24 neuromuscular causes of dropped head syndrome

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24 systemic manifestations of mitochondrial disorders 

24 neurological syndromes of HIV

25 causes of Cotard’s syndrome 

25 causes of sensory neuronopathy

25 causes of small fiber neuropathy

Property numbers. Leo Reynolds on Flickr. https://www.flickr.com/photos/lwr/120236940

25 co-morbidities of ADHD

26 causes of rapidly progressive dementia

26 causes of demyelinating neuropathy

26 causes of painful ophthalmoplegia

26 causes of systemic vasculitic peripheral neuropathy

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28 causes of young onset dementia

28 non-genetic causes of optic atrophy

28 predictors of CIS conversion to multiple sclerosis

29 medical mimics of IIH

29 congenital myasthenic syndromes

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29 neurological causes of chorea

29 genetic causes of optic atrophy

30 causes of dizziness

30 causes of tinnitus

31 causes of Capgras syndrome

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31 causes of ptosis

31 risk factors of PRES

32 causes of trigeminal neuropathy

33 causes of central retinal artery occlusion

33 causes of thunderclap headache

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33 causes of muscle fasciculations

26 causes of bilateral thalamic lesions

31 triggers of CRPS

32 typical causes of facial pain

33 enhancing meningeal lesions

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33 neurological paraneoplastic syndromes

33 malformations of cortical development

33 radiological features of meningioma

34 cerebello-pontine angle lesions

34 causes of spastic paraparesis

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35 drug-induced neuropathies

36 mimics of myasthenic crisis

38 differential diagnoses of multiple sclerosis

38 neurological syndromes of alcohol

38 risk factors of reversible dementia

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39 risk factors of cervical artery dissection

39 manifestations of spina bifida

40 radiological mimics of brain tumours

40 drug triggers of myasthenia gravis

43 causes of subacute encephalopathy

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43 causes of non-compressive myelopathy

45 systemic causes of chorea

47 causes of diaphragmatic paralysis

50 causes of hereditary neuropathy

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Go on then…

discover neurology

37 numbers. lee Reynolds on Flickr. https://www.flickr.com/photos/lwr/222241077/
Categories
Epilepsy

How does the shape of the hippocampus affect memory in temporal lobe epilepsy?

Hippocampal shape is associated with memory deficits in temporal lobe epilepsy.

Postma TS, Cury C, Baxendale S, et al.

Ann Neurol 2020; 88:170-182.

Abstract

Objective

Cognitive problems, especially disturbances in episodic memory, and hippocampal sclerosis are common in temporal lobe epilepsy (TLE), but little is known about the relationship of hippocampal morphology with memory. We aimed to relate hippocampal surface-shape patterns to verbal and visual learning.

Methods

We analyzed hippocampal surface shapes on high-resolution magnetic resonance images and the Adult Memory and Information Processing Battery in 145 unilateral refractory TLE patients undergoing epilepsy surgery, a validation set of 55 unilateral refractory TLE patients, and 39 age- and sex-matched healthy volunteers.

Results

Both left TLE (LTLE) and right TLE (RTLE) patients had lower verbal (LTLE 44 ± 11; RTLE 45 ± 10) and visual learning (LTLE 34 ± 8, RTLE 30 ± 8) scores than healthy controls (verbal 58 ± 8, visual 39 ± 6; p < 0.001). Verbal learning was more impaired the greater the atrophy of the left superolateral hippocampal head. In contrast, visual memory was worse with greater bilateral inferomedial hippocampal atrophy. Postsurgical verbal memory decline was more common in LTLE than in RTLE (reliable change index in LTLE 27% vs RTLE 7%, p = 0.006), whereas there were no differences in postsurgical visual memory decline between those groups. Preoperative atrophy of the left hippocampal tail predicted postsurgical verbal memory decline.

Interpretation

Memory deficits in temporal lobe epilepsy are associated with specific morphological alterations of the hippocampus, which could help stratify TLE patients into those at high versus low risk of presurgical or postsurgical memory deficits. This knowledge could improve planning and prognosis of selective epilepsy surgery and neuropsychological counseling in TLE.

This paper is cited in the Neurochecklist:

Temporal lobe epilepsy with hippocampal sclerosis (TLE-HS)

Abstract link

By <a href=”//commons.wikimedia.org/w/index.php?title=User:Mrazvan22&amp;action=edit&amp;redlink=1″ class=”new” title=”User:Mrazvan22 (page does not exist)”>RAZVAN V. MARINESCU</a> – <span class=”int-own-work” lang=”en”>Own work</span>, CC BY 4.0, Link
Categories
Neuroinflammation

Which anti-MOG antibody treatment has the lowest relapse risk?

Steroid-sparing maintenance immunotherapy for MOG-IgG associated disorder.

Chen JJ, Flanagan EP, Bhatti MT, et al.

Neurology 2020; 95:e111-e120.

Abstract

Objective

Myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG) associated disorder (MOGAD) often manifests with recurrent CNS demyelinating attacks. The optimal treatment for reducing relapses is unknown. To help determine the efficacy of long-term immunotherapy in preventing relapse in patients with MOGAD, we conducted a multicenter retrospective study to determine the rate of relapses on various treatments.

Methods

We determined the frequency of relapses in patients receiving various forms of long-term immunotherapy for MOGAD. Inclusion criteria were history of ≥1 CNS demyelinating attacks, MOG-IgG seropositivity, and immunotherapy for ≥6 months. Patients were reviewed for CNS demyelinating attacks before and during long-term immunotherapy.

Results

Seventy patients were included. The median age at initial CNS demyelinating attack was 29 years (range 3-61 years; 33% <18 years), and 59% were female. The median annualized relapse rate (ARR) before treatment was 1.6. On maintenance immunotherapy, the proportion of patients with relapse was as follows: mycophenolate mofetil 74% (14 of 19; ARR 0.67), rituximab 61% (22 of 36; ARR 0.59), azathioprine 59% (13 of 22; ARR 0.2), and IV immunoglobulin (IVIG) 20% (2 of 10; ARR 0). The overall median ARR on these 4 treatments was 0.3. All 9 patients treated with multiple sclerosis (MS) disease-modifying agents had a breakthrough relapse on treatment (ARR 1.5).

Conclusion:

This large retrospective multicenter study of patients with MOGAD suggests that maintenance immunotherapy reduces recurrent CNS demyelinating attacks, with the lowest annualized relapse rate being associated with maintenance IVIG therapy. Traditional MS disease-modifying agents appear to be ineffective. Prospective randomized controlled studies are required to validate these conclusions.

This paper is cited in the Neurochecklist:

Anti MOG antibody disorders: treatment

Abstract link

By Artwork by Holly Fischer – <a rel=”nofollow” class=”external free” href=”http://open.umich.edu/education/med/resources/second-look-series/materials”>http://open.umich.edu/education/med/resources/second-look-series/materials</a&gt; – CNS Slide 9, CC BY 3.0, Link
Categories
Neuroophthalmology Vascular

Is thrombolysis beneficial for central retinal artery occlusion?

Intravenous fibrinolysis for central retinal artery occlusion: a cohort study and updated patient-level meta-analysis.

Mac Grory B, Nackenoff A, Poli S, et al.

Stroke 2020; 51:2018-2025.

Abstract

Background 

Central retinal artery occlusion results in sudden, painless, usually permanent loss of vision in the affected eye. There is no proven, effective treatment to salvage visual acuity and a clear, unmet need for an effective therapy. In this work, we evaluated the efficacy of intravenous tissue-type plasminogen activator (IV alteplase) in a prospective cohort study and an updated systematic review and meta-analysis.

Methods

We enrolled consecutive patients with acute central retinal artery occlusion within 48 hours of symptoms onset and with a visual acuity of <20/200 from January 2009 until May 2019. The primary outcomes were safety and functional visual acuity recovery. We compared rates of visual recovery between those treated with alteplase within 4.5 hours of symptom onset to those who did not receive alteplase (including an analysis restricted to untreated patients presenting within the window for treatment). We incorporated these results into an updated systematic review and patient-level meta-analysis.

Results

We enrolled 112 patients, of whom 25 (22.3% of the cohort) were treated with IV alteplase. One patient had an asymptomatic intracerebral hemorrhage after IV alteplase treatment. Forty-four percent of alteplase-treated patients had recovery of visual acuity when treated within 4.5 hours versus 13.1% of those not treated with alteplase (P=0.003) and 11.6% of those presenting within 4 hours who did not receive alteplase (P=0.03). Our updated patient-level meta-analysis of 238 patients included 67 patients treated with alteplase within 4.5 hours since time last known well with a recovery rate of 37.3%. This favorably compares with a 17.7% recovery rate in those without treatment. In linear regression, earlier treatment correlated with a higher rate of visual recovery (P=0.01).

Conclusions

This study showed that the administration of intravenous alteplase within 4.5 hours of symptom onset is associated with a higher likelihood of a favorable visual outcome for acute central retinal artery occlusion. Our results strongly support proceeding to a randomized, placebo-controlled clinical trial.

Also see:

  • Dumitrascu OM, Newman NJ, Biousse V. Thrombolysis for Central Retinal Artery Occlusion in 2020: Time Is Vision! J Neuroophthalmol 2020; 40:333-345.

Both papers are cited in the Neurochecklist:

Central retinal artery occlusion (CRAO)

Abstract link 2

By <a href=”//commons.wikimedia.org/w/index.php?title=User:12mccl&amp;action=edit&amp;redlink=1″ class=”new” title=”User:12mccl (page does not exist)”>sidthedoc</a> – <span class=”int-own-work” lang=”en”>Own work</span>, CC BY-SA 4.0, Link
Categories
Neurochecklists updates

25 completely new practical neurology checklists

This is a short message just to say that we continue to add to our database.

For illustration, below are 25 checklists, completely new to Neurochecklists.

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Amnestic syndromes

Anti GAD autoimmune encephalitis

Antiplatelet resistance: causes

Antiplatelet resistance: management

Bow Hunter’s syndrome

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Central retinal artery occlusion (CRAO)

Central retinal vein occlusion (CRVO)

Edaravone

Eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome)

Giant serpentine cerebral aneurysms

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Granulomatosis with polyangiitis (Wegener’s disease)

Intracranial pseudo-aneurysms

Kawasaki disease

LAMB1 leukoencephalopathy

Low pressure hydrocephalus

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Movement disorders emergencies

Neurobartonellosis

Neurolymphomatosis

Ofatumumab

Orthostatic headache

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Parkinson’s disease hallucinations

Peduncular hallucinosis

Posterior ischaemic optic neuropathy (PION)

Russell Silver syndrome

Takayasu’s arteritis

***

Categories
Vertigo

Does vitamin D deficiency increase the risk of BPPV?

Association between serum vitamin D levels and benign paroxysmal positional vertigo: a systematic review and meta-analysis of observational studies.

Yang B, Lu Y, Xing D, et al.

Eur Arch Otorhinolaryngol 2020; 277:169-177.

Abstract

Objective

Benign paroxysmal positional vertigo (BPPV) was the most common neuro-otological disorder manifests as recurrent positional vertigo, but its risk factors are elusive. Recent studies suggest that decreased Vitamin D level may be a risk factor, but the literature is inconsistent.

Methods

The databases PubMed, Web of Science, Chinese National Knowledge Infrastructure, Wanfang, SinoMed, and Embase were systematically searched for studies on the association between BPPV and serum Vitamin D levels published up to June 2019. Data from eligible studies were meta-analyzed using Stata 12.0.

Results

A total of 18 studies were included in the analysis. Serum Vitamin D levels were significantly lower in individuals with BPPV than in controls (WMD – 2.46, 95% CI – 3.79 to – 1.12, p < 0.001). Subgroup analysis by geographical area showed that vitamin D level was significantly lower in BPPV than in controls in China (WMD – 3.27, 95% CI – 4.12 to – 2.43, p < 0.001), but not outside China (WMD – 0.90, 95% CI – 4.36 to 2.56, p = 0.611). Vitamin D levels were significantly lower in recurrent than non-recurrent BPPV across all countries in the sample (WMD 2.59, 95% CI 0.35-4.82, p = 0.023). Vitamin D deficiency emerged as an independent risk factor of BPPV (OR 1.998, 95% CI 1.400-2.851, p < 0.001).

Conclusion

The available evidence suggests that BPPV is associated with decreased levels of serum Vitamin D, and vitamin D deficiency was an independent risk factor for BPPV.

This paper is cited in the Neurochecklist:

Horizontal canal BPPV

Abstract link

By <a href=”//commons.wikimedia.org/wiki/File:Canaux_osseux.png” title=”File:Canaux osseux.png”>Canaux_osseux.png</a>: José Bragaderivative work: <a href=”//commons.wikimedia.org/wiki/User:Ortisa” title=”User:Ortisa”>Ortisa</a> (<a href=”//commons.wikimedia.org/wiki/User_talk:Ortisa” title=”User talk:Ortisa”><span class=”signature-talk”>talk</span></a>) – <a href=”//commons.wikimedia.org/wiki/File:Canaux_osseux.png” title=”File:Canaux osseux.png”>Canaux_osseux.png</a>, CC BY-SA 3.0, Link
Categories
Neurochecklists updates

30 recently revised and updated practical neurology checklists

As usual, we continue to revise and update our database.

And our sources remain the most recent studies, case reports, and reviews.

And we keep an eye on the most reliable journals and authors.

For illustration…

Below are just 30 checklists we recently updated…

Using some of the most important papers of 2020:

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Alzheimer’s disease: clinical features

ANCA associated vasculitis

Autism: clinical features

CANVAS

Cluster headache: chronic prophylaxis

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Dermatomyositis

GLUT1 deficiency disease

HaNDL

IIH: clinical features

Lacosamide

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Lyme neuroborreliosis: clinical features

MND C9orf72

MND: non-genetic risk factors

Myoclonus dystonia

Neurocysticercosis

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NF2: clinical features

NMDAR encephalitis: clinical features

NMO: cranial MRI features

Paraneoplastic antibodies

Parkinson’s disease: premotor features

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Peripheral neuropathy: blood tests

POL3 related leukodystrophy

Polymyositis

Post-ictal epilepsy: features

Rett syndrome

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Stiff person syndrome: treatment

Stroke: differential diagnosis

Topiramate

Valproate

Xeroderma pigmentosum

***

Categories
Motor neurone disease

Is there any association between smoking and MND?

Relationship between smoking and ALS: Mendelian randomisation interrogation of causality

Opie-Martin S, Wootton RE, Budu-Aggrey A, et al.

JNNP 2020 (Online ahead of print)

Abstract

Objective

Smoking has been widely studied as a susceptibility factor for amyotrophic lateral sclerosis (ALS), but results are conflicting and at risk of confounding bias. We used the results of recently published large genome-wide association studies and Mendelian randomisation methods to reduce confounding to assess the relationship between smoking and ALS.

Methods

Two genome-wide association studies investigating lifetime smoking (n=463 003) and ever smoking (n=1 232 091) were identified and used to define instrumental variables for smoking. A genome-wide association study of ALS (20 806 cases; 59 804 controls) was used as the outcome for inverse variance weighted Mendelian randomisation, and four other Mendelian randomisation methods, to test whether smoking is causal for ALS. Analyses were bidirectional to assess reverse causality.

Results

There was no strong evidence for a causal or reverse causal relationship between smoking and ALS. The results of Mendelian randomisation using the inverse variance weighted method were: lifetime smoking OR 0.94 (95% CI 0.74 to 1.19), p value 0.59; ever smoking OR 1.10 (95% CI 1 to 1.23), p value 0.05.

Conclusions

Using multiple methods, large sample sizes and sensitivity analyses, we find no evidence with Mendelian randomisation techniques that smoking causes ALS. Other smoking phenotypes, such as current smoking, may be suitable for future Mendelian randomisation studies.

This paper is cited in the Neurochecklist:

Motor neurone disease (MND): non-genetic risk factors

Abstract link

By Philip Van Damme, Wim Robberecht, and Ludo Van Den Bosch – http://dmm.biologists.org/content/10/5/537Philip Van Damme P, Robberecht W, Van Den Bosch L (May 2017). “Modelling amyotrophic lateral sclerosis: progress and possibilities.” Disease Models and Mechanisms. 10 (5): 537-549. doi:10.1242/dmm.029058 PMC: 5451175 PMID: 28468939., CC BY 3.0, Link