Gastrointestinal involvement in neuromuscular disorders.

Although not often discussed, many of the neuromuscular disorders (NMDs) affect the gastrointestinal tract (GIT). Depending on the type of NMD, the prevalence of GIT involvement ranges from <5% (e.g. hereditary neuropathies, myofibrillar myopathies) to 100% (e.g. MNGIE, OPMD). Particularly in NMDs with multisystem affection, involvement of the GIT can dominate the clinical presentation or at least make up a significant part of the clinical picture. The most prominent representatives of NMDs with multisystem involvement are the mitochondrial disorders (MIDs) and the myotonic dystrophies. The best known syndromic MIDs with GIT involvement are MNGIE, MELAS, Leigh, and Pearson syndromes. Among neuropathies, GIT involvement is most commonly found in ALS and GBS. GIT involvement may also be a feature of myasthenia. The clinical manifestations of GIT involvement are diverse and can affect the entire GIT, from the teeth to the rectum, including the liver and pancreas. The most well-known clinical manifestations of GIT involvement are dysphagia, nausea, vomiting, reflux, hollow organ dysmotility, hepatopathy, diabetes, diarrhea, constipation, and fecal incontinence. Even if treatment can usually only be symptomatic, the therapeutic options are diverse, are often effective, and can significantly and beneficially influence the course of the underlying NMD.

Homolateral Embolic Stroke After Emergency Ligation of the Common Carotid Artery to Stop Heavy Bleeding Caused by Carotid Blowout Syndrome.

Emergency common carotid artery (CCA) ligation for bleeding control during carotid blowout syndrome with consecutive embolic stroke has been reported rarely. The patient is a 70-year-old man, who was suffering from acute profuse bleeding from the left superior thyroid artery into the piriformis sinus, which was not controlled other than by ligation of the left CCA. The cause of the bleeding remained unclear but was suspected to be related to his history of being positive for squamous cell carcinoma of the left hypopharynx with ari and postcricoid infiltration, diagnosed at age 58. Squamous cell carcinoma was treated with local resection, chemotherapy (docetaxel, carboplatin, and cetuximab), and radiotherapy. Neurological examination 4 days after CCA ligation revealed multiple subacute embolic strokes in the territory of the left middle cerebral artery, macroangiopathy of the extra and intracranial cerebral arteries, and hypoplasia of the left vertebral artery. This case demonstrates that carotid blowout syndrome may require CCA ligation when external carotid artery ligation is not possible, and that ligation can be complicated by an asymptomatic embolic stroke.

Before Diagnosing Paroxysmal Sympathetic Hyperactivity in PICU Patients Alternative Conditions must be Considered.

How to cite this article: Finsterer J. Before Diagnosing Paroxysmal Sympathetic Hyperactivity in PICU Patients, Alternative Conditions must be Considered. Indian J Crit Care Med 2024;28(5):516-517.

Transient Locked-in Syndrome after Aneurysmal Subarachnoid Bleeding due to Spasm Hypoxemia?

How to cite this article: Finsterer J. Transient Locked-in Syndrome after Aneurysmal Subarachnoid Bleeding due to Spasm Hypoxemia? Indian J Crit Care Med 2024;28(1):86.

Impact of Quality Standards on Stroke Management and Outcome Requires Appropriately Designed Studies.

How to cite this article: Mehri S, Finsterer J. Impact of Quality Standards on Stroke Management and Outcome Requires Appropriately Designed Studies. Indian J Crit Care Med 2024;28(4):406-407.

Calibration of Prediction Models of In-hospital Mortality in SARS-CoV-2 Patients Depends also on Data Quality.

How to cite this article: Finsterer J, Scorza FA. Calibration of Prediction Models of In-hospital Mortality in SARS-CoV-2 Patients Depends also on Data Quality. Indian J Crit Care Med 2024;28(2):181-182.

Parkinson's Disease and the Heart: Studying Cardiac Metabolism in the 6-Hydroxydopamine Model.

Parkinson's-disease (PD) is an incurable, age-related neurodegenerative disease, and its global prevalence of disability and death has increased exponentially. Although motor symptoms are the characteristic manifestations of PD, the clinical spectrum also contains a wide variety of non-motor symptoms, which are the main cause of disability and determinants of the decrease in a patient's quality of life. Noteworthy in this regard is the stress on the cardiac system that is often observed in the course of PD; however, its effects have not yet been adequately researched. Here, an untargeted metabolomics approach was used to assess changes in cardiac metabolism in the 6-hydroxydopamine model of PD. Beta-sitosterol, campesterol, cholesterol, monoacylglycerol, α-tocopherol, stearic acid, beta-glycerophosphoric acid, o-phosphoethanolamine, myo-inositol-1-phosphate, alanine, valine and allothreonine are the metabolites that significantly discriminate parkinsonian rats from sham counterparts. Upon analysis of the metabolic pathways with the aim of uncovering the main biological pathways involved in concentration patterns of cardiac metabolites, the biosynthesis of both phosphatidylethanolamine and phosphatidylcholine, the glucose-alanine cycle, glutathione metabolism and plasmalogen synthesis most adequately differentiated sham and parkinsonian rats. Our results reveal that both lipid and energy metabolism are particularly involved in changes in cardiac metabolism in PD. These results provide insight into cardiac metabolic signatures in PD and indicate potential targets for further investigation.

Comments on "Tako-Tsubo syndrome in patients with COVID-19: a single centre retrospective case series".

Dear Editor, We read with interest the article by Alonzo et al. about a retrospective study of four patients with Takotsubo syndrome (TTS), which was attributed to SARS-CoV-2 infection (SC2I)...

Renal involvement in mitochondrial disorders.

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Opsoclonus Myoclonus Ataxia Syndrome Due to SARS-CoV-2.

Opsoclonus myoclonus syndrome (OMS)/opsoclonus myoclonus ataxia syndrome (OMAS), also known as Kinsbourne's syndrome or 'dancing eyes-dancing feet' syndrome, is a rare central nervous system manifestation of COVID-19 but an increasing number of articles have reported patients in whom COVID-19 was complicated by OMS/OMAS. This narrative review aims at summarising and discussing current knowledge about the clinical presentation, diagnosis, treatment and outcome of SARS-CoV-2 associated OMS/OMAS. Altogether, 29 articles reporting 45 patients with SARS-CoV-2 associated OMS/OMAS were retrieved. Their ages ranged from 2 to 88 years. Three patients were children and the remainder adults. Gender was male in 32 patients and female in 13 patients. Opsoclonus was described in 29 patients, which was associated with myoclonus in 28 cases. Myoclonus was described in 43 patients, which was associated with opsoclonus and ataxia in 18 patients. Cerebral magnetic resonance imaging and cerebrospinal fluid investigations were not informative in the majority of the cases. OMS/OMAS was treated with steroids in 28 patients and with intravenous immunoglobulin (IVIG) in 15 patients. Clonazepam was given to 18 patients, levetiracetam to 13 patients, and sodium valproate to eight patients. Complete recovery was achieved in 12 cases and incomplete recovery in 22 cases. Diagnosing SARS-CoV-2 associated OMS/OMAS requires extensive neurological work up and exclusion of various differentials. SARS-CoV-2 associated OMS/OMAS may not always present with the full spectrum of manifestations but as an abortive syndrome. OMS/OMAS should not be missed as it usually responds favourably to steroids or IVIG.

Characteristics of stroke-like lesions on cerebral imaging.

Objective – Stroke-like lesions (SLLs) are pathognomonic for mitochondrial ence­pha­lopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome but occur in other mitochondrial and non-mitochondrial disorders as well. This mini-review aims at summarising and discussing recent findings to open up future perspectives how to manage this fleeting phenomenon.
Results – Typically, SLLs are dynamic lesions, which increase in size and intensity to regress after a nadir. SLLs are incongruent with a vascular territory, originate frequently from the cortex to spread subcortically, can be monofocal or multifocal, run through an acute (attack) and chronic (remission) stage, and may either completely disappear or end up as laminar cortical necrosis, white matter lesion, subcortical atrophy, cyst, or the toenail sign. On cerebral CT, SLLs are hypodense. SLLs can be best visualized on multimodal MRI showing up as hyperintensity on T2, FLAIR, DWI, and PWI, and as hypointensity on OEF-MRI. On MR-spectroscopy, SLLs typically present with a decreased N-acetyl-aspartate peak and an increased lactate peak. DTI in acute SLLs reveals reduced connectivity, increased global efficiency, and reduced focal efficiency. Tc-HMPAO SPECT of SLLs indicates hyperperfusion and L-iomazenil SPECT reduced tracer uptake. FDG-PET typically shows hypometabolism within a SLL.
Conclusion – SLLs present with typical findings on various imaging modalities but the combination of cerebral CT, multimodal MRI, MRS, and PET clearly delineate a SLL from other acute or chronic cerebral lesions. 

Hiccups before a Pulmonary Embolism Speak against This as a Cause.

How to cite this article: Finsterer J. Hiccups before a Pulmonary Embolism Speak against This as a Cause. Indian J Crit Care Med 2023;27(3):228.

Status Epilepticus is Not Uncommon as the First Presentation of PRES the Diagnosis of Which Requires the Exclusion of All Differential Diagnoses.

How to cite this article: Finsterer J. Status Epilepticus is Not Uncommon as the First Presentation of PRES the Diagnosis of Which Requires the Exclusion of All Differential Diagnoses. Indian J Crit Care Med 2023;27(12):939-940.

Factors Requiring Improvement for Timely and Effective Treatment of Acute Stroke.

How to cite this article: Finsterer J. Factors Requiring Improvement for Timely and Effective Treatment of Acute Stroke. Indian J Crit Care Med 2023;27(12):947-948.

The Spectrum of Neuro-COVID is Broader than Frequently Anticipated.

The spectrum of neuro-COVID is broader than anticipated. Neurological disease in COVID-19 may be due to a direct attack of the virus, due to the immune response against the virus, secondary due to affection of the heart or arteries, or due to side effects from the treatment applied against COVID-19. How to cite this article: Finsterer J. The Spectrum of Neuro-COVID is Broader than Frequently Anticipated. Indian J Crit Care Med 2023;27(5):366-367.

Before VAN can be Recommended as a Means of Assessing ELVO, Its Reliability must be Proven by Appropriate Studies.

How to cite this article: Finsterer J, Mehri S. Before VAN can be Recommended as a Means of Assessing ELVO, Its Reliability must be Proven by Appropriate Studies. Indian J Crit Care Med 2023;27(12):943-944.

Predicting the Outcome of ICU Patients with COVID-19 Requires the Inclusion of All Influencing Factors.

How to cite this article: de Almeida AG, Scorza FA, Finsterer J. Predicting the Outcome of ICU Patients with COVID-19 Requires the Inclusion of all Influencing Factors. Indian J Crit Care Med 2023;27(11):845-846.

Neurological side effects of SARS-CoV-2 vaccinations.

SARS-CoV-2 and adverse reactions to SARS-CoV-2 vaccinations show a tropism for neuronal structures and tissues. This narrative review was conducted to collect and discuss published data about neurological side effects of SARS-CoV-2 vaccines in order to discover type, frequency, treatment, and outcome of these side effects. The most frequent neurological side effects of SARS-CoV-2 vaccines are headache, Guillain-Barre syndrome (GBS), venous sinus thrombosis (VST), and transverse myelitis. Other neurological side effects occur in a much lower frequency. Neurological side effects occur with any of the approved vaccines but VST particularly occurs after vaccination with vector-based vaccines. Treatment of these side effects is not at variance from similar conditions due to other causes. The worst outcome of these side effects is associated with VST, why it should not be missed and treated appropriately in due time. In conclusion, safety concerns against SARS-CoV-2 vaccines are backed by an increasing number of studies reporting neurological side effects. The most frequent of them are headache, GBS, VST, and transverse myelitis. Healthcare professionals, particularly neurologists involved in the management of patients having undergone SARS-CoV-2 vaccinations, should be aware of these side effects and should stay vigilant to recognize them early and treat them adequately.

Small fiber neuropathy.

Small fiber neuropathy (SFN) is a peripheral nervous system disease due to affection of A-delta or C-fibers in a proximal, distal, or diffuse distribution. Selective SFN (without large fiber affection) manifests with pain, sensory disturbances, or autonomic dysfunction. Though uniform diagnostic criteria are unavailable, most of them request typical clinical features and reduced intra-epidermal nerve fiber density on proximal or distal skin biopsy. Little consensus has been reached about the treatment of SFN, why this narrative review aims at summarizing and discussing treatment options for SFN. Treatment of SFN can be classified as symptomatic, pathophysiologic, or causal. Prerequisites for treating SFN are an established diagnosis, knowledge about the symptoms and signs, and the etiology. Pain usually responds to oral/intravenous pain killers, antidepressants, anti-seizure drugs, or topical, transdermal specifications. Some of the autonomic disturbances respond favorably to symptomatic treatment. SFN related to Fabry disease or hATTR are accessible to pathogenesis-related therapy. Immune-mediated SFN responds to immunosuppression or immune-modulation. Several of the secondary SFNs respond to causal treatment of the underlying disorder. In conclusion, treatment of SFN relies on a multimodal concept and includes causative, pathophysiologic, and symptomatic measures. It strongly depends on the clinical presentation, diagnosis, and etiology, why it is crucial before initiation of treatment to fix the diagnosis and etiology. Due to the heterogeneous clinical presentation and multi-causality, treatment of SFN should be individualized with the goal of controlling the underlying cause, alleviating pain, and optimizing functionality.

Triggers of Guillain-Barré Syndrome: Campylobacter jejuni Predominates.

Guillain-Barré syndrome (GBS) is a rare immune-mediated acute polyradiculo-neuropathy that typically develops after a previous gastrointestinal or respiratory infection. This narrative overview aims to summarise and discuss current knowledge and previous evidence regarding triggers and pathophysiology of GBS. A systematic search of the literature was carried out using suitable search terms. The most common subtypes of GBS are acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). The most common triggers of GBS, in three quarters of cases, are previous infections. The most common infectious agents that cause GBS include Campylobacter jejuni (C. jejuni), Mycoplasma pneumoniae, and cytomegalovirus. C. jejuni is responsible for about a third of GBS cases. GBS due to C. jejuni is usually more severe than that due to other causes. Clinical presentation of GBS is highly dependent on the structure of pathogenic lipo-oligosaccharides (LOS) that trigger the innate immune system via Toll-like-receptor (TLR)-4 signalling. AIDP is due to demyelination, whereas in AMAN, structures of the axolemma are affected in the nodal or inter-nodal space. In conclusion, GBS is a neuro-immunological disorder caused by autoantibodies against components of the myelin sheath or axolemma. Molecular mimicry between surface structures of pathogens and components of myelin or the axon is one scenario that may explain the pathophysiology of GBS.