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Intractable & Rare Diseases Research Nov 2023Varicella zoster virus (VZV) causes chickenpox at the primary infection and then becomes latent in the spinal dorsal root ganglia; VZV can reactivate with aging,...
Varicella zoster virus (VZV) causes chickenpox at the primary infection and then becomes latent in the spinal dorsal root ganglia; VZV can reactivate with aging, immunosuppression, stress, and other factors, occurring as herpes zoster (HZ) at 1-2 skin segments. HZ peripheral nerve complications caused by VZV reactivation include Hunt syndrome, segmental HZ paresis, post-herpetic neuralgia, and Guillain-Barré syndrome (GBS). We have encountered the rare HZ complications of upper-limb paresis, myeloradiculitis, and polyradiculoneuritis: an adult woman with upper-limb paresis consistent with the nerve root on segments above the thoracic HZ dermatome; another woman exhibiting ascending myeloradiculitis originating at the Th11-12 roots; an elderly woman with ascending VZV polyradiculoneuritis resembling GBS; an adult with VZV quadriplegia with disseminated HZ; and an elderly patient with VZV-associated polyradiculoneuritis. The three polyradiculoneuritis cases may be a new subtype of HZ peripheral neuropathy, but the pathophysiology for these HZ peripheral nerve complications unrelated to HZ dermatomes is unclear. We analyzed host factors, skin lesions, neurological and virological findings, and MRI results including 3D NerveVIEW in 15 Japanese patients treated at our facility for HZ peripheral neuropathy, including six differing from the HZ dermatome. Based on the clinical findings including MRI results of spinal ganglia and roots, we identified four possible routes for the patterns of VZV spread: () ascending spinal roots, () ascending spinal cord, () polyradiculopathy, and () intrathecal spread. The incidence of HZ is increasing with the aging of many populations, and clinicians should be aware of these HZ neuropathies.
PubMed: 38024578
DOI: 10.5582/irdr.2023.01090 -
BMC Neurology Nov 2023Syphilis is associated with a wide variety of systemic presentations, earning it the moniker "The great mimicker". Neurosyphilis is classically associated with...
BACKGROUND
Syphilis is associated with a wide variety of systemic presentations, earning it the moniker "The great mimicker". Neurosyphilis is classically associated with meningovasculitis in the acute-subacute stage and tabes dorsalis and dementia paralytica in later stages. However, one of the less well described presentations include Guillain-Barre Syndrome. This case presents a patient with an ascending polyneuropathy suspicious for Guillain-Barre Syndrome who also had other atypical findings including a truncal sensory loss, optic disc swelling, and rash ultimately found to have neurosyphilis. Electrodiagnostic testing was consistent with demyelination, supporting a diagnosis of neurosyphilis associated Guillain-Barre Syndrome.
CASE PRESENTATION
A 37-year-old female presented to the emergency department with a weakness and difficulty swallowing. She described a three-month history of symptoms, initially starting with a persistent headache followed by one month of a pruritic rash on her chest, palms, and soles. Two weeks prior to presentation, she developed progressive weakness in her arms, numbness in her arms and chest, and difficulty swallowing. Neurological exam was notable for multiple cranial neuropathies, distal predominant weakness in all extremities, length-dependent sensory loss, and hyporeflexia. Investigation revealed a positive Venereal Disease Research Laboratory in her cerebrospinal fluid without significant pleocytosis, contrast enhancement in cranial nerves V, VII, and VIII on MRI, and a demyelinating polyneuropathy on electrodiagnostic testing. She was diagnosed with Guillain-Barre syndrome, secondary to neurosyphilis. The patient acutely declined and required intubation, and ultimately made a full recovery after treatment with plasmapheresis and penicillin.
CONCLUSIONS
This case describes a clinical entity of syphilitic Guillain-Barre Syndrome and highlights the importance of including syphilis in the differential of any patient presenting with ascending polyradiculopathy, especially given the resurgence of syphilis.
Topics: Humans; Female; Adult; Guillain-Barre Syndrome; Syphilis; Neurosyphilis; Exanthema
PubMed: 38001427
DOI: 10.1186/s12883-023-03471-5 -
Journal of the American Academy of... Nov 2023This case offers a safe and effective method for treating lumbopelvic dissociation in a pediatric patient with cauda equina syndrome using a triangular osteosynthesis...
This case offers a safe and effective method for treating lumbopelvic dissociation in a pediatric patient with cauda equina syndrome using a triangular osteosynthesis construct. After a high-speed accident, an 11-year-old girl was admitted to the hospital with bowel and bladder incontinence and bilateral lower extremity weakness. The orthopaedic trauma and spine teams elected for surgical treatment with a triangular osteosynthesis construct, a procedure usually reserved for adults. The surgery was uncomplicated, and the patient experienced complete resolution of her preoperative symptoms. She is doing well over 1-year postoperatively, with full neurologic recovery, maintained reduction, bony healing, and subsequent implant removal. To our knowledge, this is the youngest reported case of lumbopelvic dissociation treated in this manner and represents a viable treatment option.
Topics: Humans; Adult; Female; Child; Cauda Equina Syndrome; Polyradiculopathy; Decompression, Surgical; Spine; Fracture Fixation, Internal
PubMed: 37967093
DOI: 10.5435/JAAOSGlobal-D-23-00090 -
Scientific Reports Nov 2023The clinical course of Lyme neuroborreliosis (LNB) is highly variable. Delayed diagnosis and treatment still remain actual challenges. Moreover, there is a lack of...
The clinical course of Lyme neuroborreliosis (LNB) is highly variable. Delayed diagnosis and treatment still remain actual challenges. Moreover, there is a lack of studies analyzing the factors associated with different LNB syndromes. We aimed to analyze clinical and epidemiological features of LNB in hospitalized adults in eastern Lithuania. A retrospective study was performed for patients presenting in the years 2010-2021. A total of 103 patients were included in the study, 100 with early, and three with late LNB. Patients with early LNB most often presented polyradiculitis [75/100, (75%)], which was also the most common initial neurological syndrome. Peripheral facial palsy was diagnosed in 53/100 (53%) patients, in 16/53 (30.2%) cases both facial nerves were affected. Encephalitis or myelitis was diagnosed in 14% of patients with LNB. A total of 76/103 (73.8%) patients were discharged with residual symptoms or signs. One patient presenting encephalomyelitis died because of bacterial complications. The absence of observed erythema migrans (EM) was the predictor of peripheral facial palsy, while female sex and EM untreated with antibiotics were predictors of isolated polyradiculitis. A fever of ≥ 38 ° °C and pleocytosis of ≥ 300 × 10/l were associated with the development of encephalitis or myelitis in patients with early LNB.
Topics: Humans; Adult; Female; Facial Paralysis; Lyme Neuroborreliosis; Retrospective Studies; Polyradiculopathy; Bell Palsy; Encephalitis; Myelitis; Erythema Chronicum Migrans
PubMed: 37964035
DOI: 10.1038/s41598-023-47312-4 -
European Spine Journal : Official... Mar 2024Symptoms of cauda equina syndrome (CES) secondary to degenerative lumbar spine diseases are sometimes mild and tend to be ignored by patients, resulting in delayed...
BACKGROUND
Symptoms of cauda equina syndrome (CES) secondary to degenerative lumbar spine diseases are sometimes mild and tend to be ignored by patients, resulting in delayed treatment. In addition, the long-term efficacy of surgery is unclear.
OBJECTIVE
To determine the predictive factors of CES and post-operative recovery in patients with symptoms lasting > 3 months.
METHODS
From January 2011 to December 2020, data of 45 patients with CES secondary to lumbar disk herniation/lumbar spinal stenosis were collected from a single center. The patients had bladder, bowel or sexual dysfunction and decreased perineal sensation that lasted for > 3 months. A 2-year post-operative follow-up was conducted to evaluate recovery outcomes, which were measured by validated self-assessment questionnaires conducted by telephone and online.
RESULTS
Overall, 45 CES patients (57.8% female; mean age, 56 years) were included. The duration of pre-operative CES symptoms was 79.6 weeks (range, 13-730 weeks). The incidence of saddle anesthesia before decompression was 71.1% (n = 32), bladder dysfunction 84.4% (n = 38), bowel dysfunction 62.2% (n = 28) and sexual dysfunction 64.4% (n = 29). The overall recovery rate of CES after a 2-year follow-up was 64.4%. The rates of the residual symptoms at the last follow-up were as follows: saddle anesthesia 22.2%, bladder dysfunction 33.3%, bowel dysfunction 24.4% and sexual dysfunction 48.9%. Pre-operative saddle anesthesia, overactive bladder and sexual dysfunction were risk factors for poor prognosis after decompression.
CONCLUSION
CES patients with symptoms lasting > 3 months may recover after surgery. Sexual dysfunction has a high residual rate and should not be ignored during diagnosis and treatment.
Topics: Humans; Female; Middle Aged; Male; Cauda Equina Syndrome; Self-Assessment; Retrospective Studies; Intervertebral Disc Displacement; Decompression; Polyradiculopathy; Cauda Equina
PubMed: 37947889
DOI: 10.1007/s00586-023-07984-y -
World Neurosurgery Oct 2023
Topics: Humans; Cauda Equina Syndrome; Cauda Equina; Bibliometrics; Dermatitis; Polyradiculopathy
PubMed: 37803677
DOI: 10.1016/j.wneu.2023.06.045 -
Muscle & Nerve Nov 2023In our experience, patients with late-onset facioscapulohumeral muscular dystrophy type 1 (FSHD1) are frequently misdiagnosed, some for many years. The aim of this...
INTRODUCTION/AIMS
In our experience, patients with late-onset facioscapulohumeral muscular dystrophy type 1 (FSHD1) are frequently misdiagnosed, some for many years. The aim of this report is to document this clinical experience including the presenting symptoms and misdiagnoses and to discuss the challenges in diagnosing patients with late-onset FSHD1.
METHODS
We performed a retrospective medical record review and recorded clinical data on patients with a genetically confirmed diagnosis of FSHD1, who began to have symptoms at 50 years of age or older, and either had no family history of FSHD1 or had a history of an undiagnosed weakness in a family member.
RESULTS
Thirteen patients, 7 men and 6 women, met the study inclusion criteria. Age of onset ranged from 52 to 74 (mean, 59.8) years, age of diagnosis ranged from 54 to 80 (mean, 66.5) years, and duration of symptoms from onset to diagnosis was 1 to 15 (mean, 6.7) years. Prior diagnoses included lumbosacral polyradiculopathy in five (38%); statin-related myopathy in two (15%); and one each of polymyositis, inclusion-body myositis, distal myopathy, limb-girdle muscular dystrophy, unspecific myopathy, and unspecified scapular winging. For eight patients (62%), family history was suspected in deceased members or if by confirmed DNA test postdiagnosis.
DISCUSSION
The diagnosis of late-onset FSHD1 is often delayed by many years with patients frequently receiving misdiagnoses. FSHD1 may not be considered in the differential diagnosis of late-onset weakness due to its rarity and because its clinical features are subtler, nonspecific, and mimic other neuromuscular disorders.
Topics: Male; Humans; Female; Middle Aged; Aged; Muscular Dystrophy, Facioscapulohumeral; Retrospective Studies; Muscular Dystrophies, Limb-Girdle; Diagnostic Errors
PubMed: 37638785
DOI: 10.1002/mus.27962 -
Oxford Medical Case Reports Jul 2023Eosinophilic granulomatous polyangiitis (EGPA) like other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis has multisystemic involvement. It commonly...
Eosinophilic granulomatous polyangiitis (EGPA) like other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis has multisystemic involvement. It commonly manifests with prodromal pulmonary involvement as asthma, chronic sinusitis followed by systemic vasculitic complications associated with blood and tissue eosinophilia. Central nervous system manifestations at presentation are uncommon compared with peripheral nervous system involvement. Vasculitic neuropathy in EGPA commonly presents as mononeuritis multiplex but rarely as polyradiculopathy. Late onset EGPA often presents with systemic involvement, and early diagnosis is a key to prevent further complications. The neuropathy in late onset EGPA is often refractory to immunosuppression and corticosteroids treatment. We report a case of EGPA with late onset asthma presenting with acute infarct and demyelinating polyradiculoneuropathy that progressed with bulbar paralysis and profound dysautonomia. This illustrates simultaneous involvement of central and peripheral nervous system with EGPA. Autonomic dysfunction can occur in patients of EGPA with multisystem involvement, which may predict severe complications.
PubMed: 37484549
DOI: 10.1093/omcr/omad067 -
Giornale Italiano Di Nefrologia :... Jun 2023There is a strong correlation between SARS-CoV-2 and the onset of autoimmune neurological disease with atypical clinical presentation, characterized by limited response...
There is a strong correlation between SARS-CoV-2 and the onset of autoimmune neurological disease with atypical clinical presentation, characterized by limited response to medical therapy, likely caused by the underlying mechanism of the virus itself. In situations like these, after the failure of pharmacological therapy, therapeutic apheresis, including immunoadsorption, can be pursued. Treatments with IMMUSORBA TR-350 columns have proven to be particularly effective in managing refractory forms of post-Covid-19 nephropathies, leading to complete recovery of disability and elimination of neurological signs and symptoms. We discuss the case of a patient with chronic inflammatory polyradiculopathy post-Covid-19, resistant to medical therapy, effectively treated with immunoadsorption.
Topics: Humans; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; COVID-19; SARS-CoV-2; Blood Component Removal; Kidney Diseases
PubMed: 37427907
DOI: No ID Found