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Neurology India 2021We report about two young males who developed significant proximal weakness of all four limbs secondary to intracranial hypertension due to intracranial venous sinus...
We report about two young males who developed significant proximal weakness of all four limbs secondary to intracranial hypertension due to intracranial venous sinus thrombosis. Intracranial venous sinus thrombosis can manifest in a variety of ways which includes isolated intracranial hypertension, focal neurological symptoms or signs and acute or subacute encephalopathy. Various false localising signs have been reported to occur in patients with raised intracranial pressure including cranial nerve palsies and extensive radiculopathy. In a patient presenting with flaccid areflexic quadriparesis and papilledema, the possibility of a potentially reversible dysfunction of the cranial nerves and spinal nerve roots due to a marked rise in intracranial and intraspinal pressure must be recognised. Lumboperitoneal shunt to reduce the intraspinal pressure on the spinal nerve roots has been advocated to reverse the symptoms of extensive radiculopathy in such patients. Both of our patients showed remarkable improvement in symptoms and signs with medical treatment of CVT.
Topics: Cranial Sinuses; Humans; Intracranial Hypertension; Male; Papilledema; Polyradiculopathy; Sinus Thrombosis, Intracranial
PubMed: 33642294
DOI: 10.4103/0028-3886.310084 -
Annals of Indian Academy of Neurology 2020A wide variety of neurological diseases result in clinical and/or radiological enlargement of nerves, roots and plexuses. With the advancement in techniques and use of...
BACKGROUND AND AIMS
A wide variety of neurological diseases result in clinical and/or radiological enlargement of nerves, roots and plexuses. With the advancement in techniques and use of magnetic resonance neurography (MRN), aided by electrophysiology, proximal segments of the lower motor neuron (LMN) can be well studied. The relative merits of investigative modalities have not been well defined and comprehensive information on this subject is sparse.
METHODS
This retrospective study included data from January 2010 to June 2018. Patients having clinical and/or radiological enlargements of lower motor neuron were included. Clinical and laboratory work up, electrophysiology, MRN and biopsy studies were documented and analyzed.
RESULTS
133 patients fulfilled the inclusion criteria. The diagnostic categories were of leprosy (32%), immune neuropathies (27.8%), nerve infiltrations (8.2%), inherited neuropathies (9%), diabetic radiculopathies (9%) and others (12.7%). MRN was essential to diagnosis in 24.8% and supportive in 31.5% patients. Electrophysiology was essential in diagnosis in 70.6%, biopsy in 45.8% and genetic studies in 6.4% patients.
CONCLUSION
The manuscript presents a large cohort of diseases causing enlargement of LMN with clinical and investigative aspects of 7 patients of the most unusual condition of chronic immune sensorimotor polyradiculopathy (CISMP) and details of 7 other patients with chronic mononeuropathies at non-entrapment sites. A table of comparative utility and an algorithm depicting the optimization of investigations has been presented.
PubMed: 33623269
DOI: 10.4103/aian.AIAN_504_19 -
Cureus Jan 2021Guillain-Barre syndrome (GBS) is a clinical syndrome with multiple variants. GBS is defined as an acute demyelinating polyneuropathy commonly preceded by infection...
Guillain-Barre syndrome (GBS) is a clinical syndrome with multiple variants. GBS is defined as an acute demyelinating polyneuropathy commonly preceded by infection (bacterial or viral), trauma, or inflammatory processes, which triggers an autoimmune response that affects the peripheral nervous system. This case report describes a patient with high-grade osteosarcoma that completed neoadjuvant chemotherapy and underwent surgical resection with no immediate complications. Fourteen days after the surgery, the patient developed an acute inflammatory demyelinating polyradiculopathy consistent with GBS. As the five-year survival without chemotherapy is only around 20%, this challenging clinical scenario raised questions regarding adjuvant chemotherapy's safe completion in this setting.
PubMed: 33542879
DOI: 10.7759/cureus.12432 -
Medical Hypotheses Mar 2021
Topics: COVID-19; Humans; Myocarditis; Polyradiculopathy; SARS-CoV-2; Takotsubo Cardiomyopathy
PubMed: 33540143
DOI: 10.1016/j.mehy.2021.110509 -
IDCases 2021Brucellosis is a bacterial disease caused by different species of Brucella. Neurobrucellosis is one of the complications of brucellosis and polyradiculopathy is an...
INTRODUCTION
Brucellosis is a bacterial disease caused by different species of Brucella. Neurobrucellosis is one of the complications of brucellosis and polyradiculopathy is an uncommon manifestation.
CASE REPORT
In this article we report a 29-year-old male patient diagnosed with neurobrucellosis who presented with subacute lower limbs weakness and inability to walk in the last 50 days. The patient declared usage of non-pasteurized dairy products in his past medical history. Diagnosis was confirmed by the LP of CSF and serological tests. Although PCR for Brucella was negative, Wright, Coombs Wright and 2ME tests were reported positive in both CSF and serum. MRI and EMG were also performed that highlighted polyradiculopathy. After six months treatment, complete clinical recovery along with elimination of nerve root enhancement in MRI by injection in the lumbosacral region was seen.
CONCLUSION
Neurobrucellosis is a serious manifestation of Brucellosis that can have many side effects. Therefore, clinicians must pay attention to the neurological manifestations of this disease, but also reduce the effects of this disease by accelerating the start of treatment.
PubMed: 33384926
DOI: 10.1016/j.idcr.2020.e01028 -
Pathogens (Basel, Switzerland) Nov 2020Coronavirus 2019 (COVID-19) has been reported to trigger Guillain-Barré syndrome (GBS). While uncommon, recurrent GBS (rGBS) episodes, triggered by antecedent viral...
Coronavirus 2019 (COVID-19) has been reported to trigger Guillain-Barré syndrome (GBS). While uncommon, recurrent GBS (rGBS) episodes, triggered by antecedent viral infections, have been reported in a small proportion of GBS patients, here we describe a patient with a recurrent case of GBS, occurring secondary to COVID-19 infection. Before this patient's episode, he had two prior GBS flares, each precipitated by a viral infection followed by complete recovery besides intermittent paresthesias. We also consider the nosology of this illness in the spectrum of rGBS and Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), with their differing natural histories, prognosis, and therapeutic approaches. For patients who have a history of inflammatory demyelinating polyradiculopathies who develop COVID-19, we recommend close observation for neurologic symptoms over the next days and weeks.
PubMed: 33228253
DOI: 10.3390/pathogens9110965 -
Annals of Indian Academy of Neurology 2020Normal-sized ventricles and absence of papilledema do not rule out shunt failure and raised intracranial pressure (ICP). Raised ICP can present with false localizing...
Normal-sized ventricles and absence of papilledema do not rule out shunt failure and raised intracranial pressure (ICP). Raised ICP can present with false localizing signs which may be cranial nerve palsies or extensive polyradiculopathy. Our patient with a history of ventriculoperitoneal (VP) shunt presented with rapidly progressive vision loss without papilledema, as well as multiple cranial nerve palsies and radiculopathy. Imaging did not reveal hydrocephalus, however, cerebrospinal fluid (CSF) manometry revealed high CSF opening pressure. After lumbar thecoperitoneal shunting, vision did not improve, but the rest of cranial nerve palsies and radiculopathy improved. In a patient in whom VP shunt is , headache and vomiting should prompt evaluation for raised ICP though there is no ventriculomegaly of papilledema. Vision can be saved if raised ICP is suspected, CSF opening pressure measured at presentation and prompt surgery is performed.
PubMed: 33223673
DOI: 10.4103/aian.AIAN_431_18 -
The American Journal of Tropical... Jan 2021Spinal sparganosis of the cauda equina has been rarely reported. A 54-year-old man presented at the hospital after having experienced lower back pain for 10 months,... (Review)
Review
Spinal sparganosis of the cauda equina has been rarely reported. A 54-year-old man presented at the hospital after having experienced lower back pain for 10 months, progressive weakness and numbness of the left leg for 4 months, and urinary incontinence for 3 weeks. Magnetic resonance imaging of the lumbosacral spine revealed a heterogeneous enhancing mass at the T12-S1 level. Spinal sparganosis was diagnosed by histological examination and molecular identification of the parasite in the tissue section. The patient was treated with a high dose of praziquantel because the parasitic mass was only partially removed and symptoms worsened following surgery.
Topics: Anthelmintics; Anti-Ulcer Agents; Cauda Equina; Cimetidine; Humans; Male; Middle Aged; Polyradiculopathy; Praziquantel; Sparganosis
PubMed: 33124542
DOI: 10.4269/ajtmh.20-0712 -
Neuropediatrics Feb 2021Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare and disabling immunomediated radiculoneuropathy. Its worldwide epidemiology is heterogeneous...
BACKGROUND
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare and disabling immunomediated radiculoneuropathy. Its worldwide epidemiology is heterogeneous and, in adults, CIDP prevalence varies from 0.6 to 9 cases per 100,000 population. Juvenile CIDP (jCIDP) is even rarer, with age-specific prevalence rates varying from 0.23 to 1.26 owing to different diagnostic criteria (American Academy of Neurology [AAN] and European Federation of Neurological Societies/Peripheral Nerve Society [EFNS/PNS]), different age grouping or, genuine differences.
OBJECTIVES
We assessed jCIDP incidence and prevalence in Sardinia, an area at very-high risk for autoimmune diseases, using comparable methods.
DESIGN
The study area was the northern Sardinia, insular Italy, with 491,571 inhabitants and a pediatric population (0-18 years) of 79,086 individuals.
RESULTS
On prevalence day (December 31, 2019) the total crude, age-specific prevalence rate were 6.32 per 100,000 according with AAN criteria, 7.58 per 100,000 population with European Neuromuscular Center (ENMC) criteria, and 8.85 per 100,000 population with both 2006 and 2010 EFNS/PNS criteria. Crude mean incidence rate were 0.42 per 100,000 per year with AAN criteria, 0.50 per 100,000 per year with ENMC criteria, and 0.59 per 100,000 per year using 2006 and 2010 EFNS/PNS criteria. Of the eight patients, six had typical CIDP, one had multifocal-acquired demyelinating sensory and motor neuropathy (MADSAM), and one chronic immune sensory polyradiculopathy (CISP). Patient's disability was generally mild. Clinical course was progressive, monophasic, or relapsing.
CONCLUSION
jCIDP prevalence and incidence rates in Sardinia were criteria-dependent, the lowest obtained when using AAN criteria, the highest using the EFNS/PNS. Nonetheless, even with the exclusion of the "possible" category, by using comparable methodology, prevalence rates in Sardinia are considerably higher than the range reported in all previous jCIDP studies.
Topics: Adolescent; Child; Child, Preschool; Female; Humans; Incidence; Infant; Italy; Mediterranean Islands; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Practice Guidelines as Topic; Prevalence
PubMed: 33111308
DOI: 10.1055/s-0040-1715626 -
European Journal of Neurology Feb 2021The aim was to identify the clinical and diagnostic investigations that may help to support a diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy...
BACKGROUND AND PURPOSE
The aim was to identify the clinical and diagnostic investigations that may help to support a diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in patients not fulfilling the European Federation of Neurological Societies and Peripheral Nerve Society (EFNS/PNS) electrodiagnostic criteria.
METHODS
The data from patients with a clinical diagnosis of CIDP included in a national database were retrospectively reviewed.
RESULTS
In all, 535 patients with a diagnosis of CIDP were included. This diagnosis fulfilled the EFNS/PNS criteria in 468 patients (87.2%) (definite in 430, probable in 33, possible in three, while two had chronic immune sensory polyradiculopathy). Sixty-seven patients had a medical history and clinical signs compatible with CIDP but electrodiagnostic studies did not fulfill the EFNS/PNS criteria for CIDP. These patients had similar clinical features and frequency of abnormal supportive criteria for the diagnosis of CIDP compared to patients fulfilling EFNS/PNS criteria. Two or more abnormal supportive criteria were present in 40 (61.2%) patients rising to 54 (80.6%) if a history of a relapsing course as a possible supportive criterion was also included. Increased cerebrospinal fluid proteins and response to immune therapy most frequently helped in supporting the diagnosis of CIDP. Response to therapy was similarly frequent in patients fulfilling or not EFNS/PNS criteria (87.3% vs. 85.9%).
CONCLUSIONS
Patients with a clinical diagnosis of CIDP had similar clinical findings, frequency of abnormal supportive criteria and response to therapy compared to patients fulfilling EFNS/PNS criteria. The presence of abnormal supportive criteria may help in supporting the diagnosis of CIDP in patients with a medical history and clinical signs compatible with this diagnosis but non-diagnostic nerve conduction studies.
Topics: Databases, Factual; Humans; Neural Conduction; Peripheral Nerves; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Retrospective Studies
PubMed: 32959475
DOI: 10.1111/ene.14545