Intracranial Hypertension Associated With Poly-Cranio-Radicular-Neuropathies: A Case Report and Review of the Literature.

INTRODUCTION: We present the case of a gentleman who developed rapidly progressive vision loss, ophthalmo-paresis, and flaccid quadriparesis in the context of severe intracranial hypertension. We reviewed the available cases in the literature to increase awareness of this rare clinical entity.Case Report:A 36-year-old man developed rapidly progressive vision loss, ophthalmo-paresis, and flaccid quadriparesis. He had an extensive workup, only notable for severe intracranial hypertension, >55 cm of H 2 O. No inflammatory features were present, and the patient responded to CSF diversion. Few similar cases are available in the literature, but all show markedly elevated intracranial pressure associated with extensive neuroaxis dysfunction. Similarly, these patients improved with CSF diversion but did not appear to respond to immune-based therapies. CONCLUSIONS: We term this extensive neuroaxis dysfunction intracranial hypertension associated with poly-cranio-radicular-neuropathy (IHP) and distinguish it from similar immune-mediated clinical presentations. Clinicians should be aware of the different etiologies of this potentially devastating clinical presentation to inform appropriate and timely treatment.

Repeated Acute Exacerbations of Chronic Inflammatory Demyelinating Polyradiculoneuropathy Accompanied by Pain and Swelling in Distal Extremities.

An 81-year-old man experienced acute progression of weakness in the extremities accompanied by a fever, tenderness, and swelling in distal parts of the extremities. He had flaccid tetraparesis with fasciculations and general hyporeflexia. Nerve conduction studies indicated demyelinating sensorimotor neuropathy. A cerebrospinal fluid examination revealed elevated proteins without pleocytosis. Immunological treatments were effective, but his symptoms exhibited repeated relapse and remission phases. He was diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with an acute onset. The highlight of this case is pain with inflammatory reaction recognized as red flags of CIDP, with the clinical course and electrophysiological findings compatible with CIDP.

Immune-mediated diseases involving central and peripheral nervous systems.

BACKGROUND AND PURPOSE: In addition to combined central and peripheral demyelination, other immune diseases could involve both the central nervous system (CNS) and peripheral nervous system (PNS). METHODS: To identify immune-mediated diseases responsible for symptomatic combined central/peripheral nervous system involvement (ICCPs), we conducted a multicentric retrospective study and assessed clinical, electrophysiological, and radiological features of patients fulfilling our ICCP criteria. RESULTS: Thirty patients (20 males) were included and followed during a median of 79.5 months (interquartile range [IQR] = 43-145). The median age at onset was 51.5 years (IQR = 39-58). Patients were assigned to one of four groups: (i) monophasic disease with concomitant CNS/PNS involvement including anti-GQ1b syndrome (acute polyradiculoneuropathy + rhombencephalitis, n = 2), checkpoint inhibitor-related toxicities (acute polyradiculoneuropathy + encephalitis, n = 3), and anti-glial fibrillary acidic protein astrocytopathy (subacute polyradiculoneuropathy and meningoencephalomyelitis with linear gadolinium enhancements, n = 2); (ii) chronic course with concomitant CNS/PNS involvement including paraneoplastic syndromes (ganglionopathy/peripheral hyperexcitability + limbic encephalitis, n = 4); (iii) chronic course with sequential CNS/PNS involvement including POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) syndrome (polyradiculoneuropathy + strokes, n = 2), histiocytosis (polyradiculoneuropathy + lepto-/pachymeningitis, n = 1), and systemic vasculitis (multineuropathy + CNS vasculitis/pachymeningitis, n = 2); and (iv) chronic course with concomitant or sequential CNS/PNS involvement including combined central and peripheral demyelination (polyradiculoneuropathy + CNS demyelinating lesions, n = 10) and connective tissue diseases (ganglionopathy/radiculopathy/multineuropathy + limbic encephalitis/transverse myelitis/stroke, n = 4). CONCLUSIONS: We diagnosed nine ICCPs. The timing of central and peripheral manifestations and the disease course help determine the underlying immune disease. When antibody against neuroglial antigen is identified, CNS and PNS involvement is systematically concomitant, suggesting a common CNS/PNS antigen and a simultaneous disruption of blood-nerve and blood-brain barriers.

Temporal evolution of nerve conduction study abnormalities in anti-myelin-associated glycoprotein neuropathy.

BACKGROUND: A distal-predominant demyelinating symmetric pattern is most frequent in patients with neuropathy associated with anti-myelin-associated glycoprotein (MAG) antibodies. The literature however lacks longitudinal data to describe whether this is consistent over time. METHODS: From the Ottawa Neuromuscular Center database, we identified 23 patients with both immunoglobulin M gammopathy and anti-MAG antibodies. For median, ulnar and fibular motor conduction studies, we analyzed distal latency and amplitude, negative peak duration, terminal latency index (TLI), and conduction velocity. For median, ulnar, sural, and superficial fibular sensory conduction studies, we analyzed distal latency and amplitude. Results were compared for the earliest and the latest data sets. RESULTS: The mean time interval between the two assessment points was 6.5 years. Median and ulnar motor nerve conduction studies did not show a significant change for any of the parameters tested. There was disproportionate prolongation of median distal motor latency and reduction in TLI, compared to the ulnar nerve. Deep fibular motor conduction studies showed a marked reduction in amplitudes over time. Sensory potentials were recordable in the upper limb in less than 50% at the first study and less than 25% on the most recent study. There was an even larger attrition of recordable sural and superficial fibular sensory potentials. CONCLUSIONS: Our results highlight the stability of median and ulnar motor conduction study results over a mean observation period of 6.5 years. In contrast, lower limb motor and all sensory potentials show a marked trend toward becoming unrecordable.

MOG antibody-related disease with recurrent optic neuritis and sensory polyradiculoneuropathy: A case report.

Myelin oligodendrocyte glycoprotein (MOG) antibody-related diseases are inflammatory demyelinating conditions of the central nervous system (CNS) that induce a broad spectrum of symptoms. Since MOG is expressed exclusively in the CNS, the lesions are thought to be confined to the CNS. However, few cases of MOG antibody-related disease involve the peripheral nervous system (PNS); the mechanisms underlying such PNS involvement remain unclear. We herein present the case of a patient with MOG antibody-related disease with recurrent optic neuritis and sensory polyradiculoneuropathy unaccompanied by CNS lesions. Our report presents a novel phenotype of PNS involvement in MOG antibody-related disease.

Acute idiopathic polyradiculoneuritis with secondary arterial hypertension in a 5-year-old male Siberian Husky.

Acute canine idiopathic polyradiculoneuritis (ACIP) is one of the most common generalised neuromuscular diseases affecting dogs. In this report, we describe a 5-year-old, 25-kg, male, intact, Siberian Husky dog with ACIP with secondary induced arterial hypertension {systolic blood pressure [mean (m) ± standard deviation (sd)], 214 ± 19 mmHg; mean blood pressure (m ± sd), 164 ± 6.36 mmHg; and diastolic blood pressure (m ± sd), 137 ± 0.7 mmHg} and sinus tachycardia. Heart rate variability analysis indicated decreased vagal activity (low root-mean-square values of successive RR interval differences and percentages of the RR intervals differing by more than 50 ms in the entire recording) and predominance of sympathetic activity. Arterial hypertension was treated with amlodipine but remained greater than the upper limit for 51 days until the dog recovered ambulation. This is the first case report of ACIP and secondary arterial hypertension in a dog. Routine blood pressure measurements should be included in the monitoring of patients with ACIP if arterial hypertension might interfere with patient prognosis.

Polineuropatía sensitiva desmielinizante asociada a anticuerpos anti-Yo y cáncer papilar de tiroides / Sensitive demyelinating neuropathy associated with anti-Yo antibodies and papillary thyroid cancer

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[Varicella-zoster virus-associated polyradiculoneuritis with concomitant herpes zoster eruption: a case report].

A 76-year-old Japanese female who was treated with long-term use of prednisolone at 10 mg/day for interstitial pneumonia developed acute right-dominant lower limb paralysis and then upper limb paralysis with herpes zoster eruptions on the right C7-Th1 dermatomes. On admission, right predominant quadriplegia was observed with sensory symptoms; Hughes functional grade was level 4; the hand grip power was right, 0, and left, 7 kg, the deep tendon reflexes were abolished throughout without pathologic reflexes. Twenty days after the onset of the symptoms, the cerebrospinal fluid (CSF) revealed mild increases of lymphocytes (13 cells/µl) and protein content (73 mg/dl). Varicella-zoster virus (VZV) PCR was negative in the CSF, but an enzyme immunoassay for VZV was positive in her serum and CSF, and the high titers were prolonged. Peripheral nerve conduction and F wave studies suggested right-dominant demyelinating polyradiculoneuropathy. A T1-weighted MR contrast image exhibited right-dominant high-intensity lesions on the C7-Th1 spinal roots and similar lesions on the L4-5 spinal roots. We compared with several similar cases from the literature and proposed that VZV itself involves the pathogenesis of the polyradiculoneuritis in immunocompromised hosts.

DEVELOPMENT OF GANGLIONOPATHY AND TABETIC VISCERAL CRISES ON THE BACKGROUND OF POLYRADICULONEUROPATHY ASSOCIATED WITH MONOCLONAL GAMMOPATHY (CASE REPORT).

The article presents an analysis of the clinical occurrence of development of chronic polyradiculoneuropathy associated with monoclonal IgG/k (kappa) gammopathy of the undetermined significance. The peculiarity of this occurrence is the uniqueness of the development of the symptoms which are characteristic of tabes dorsalis in this pathology with episodic severe visceral crises and also with ganglionopathy. The example describes the clinical polymorphism of the course of visceral crises, the problems of their diagnosis and as a consequence of inadequate treatment with the development of severe social maladaptation. The importance of timely diagnosis and treatment of such conditions is discussed.

The clinical features of combined central and peripheral demyelination in Chinese patients.

BACKGROUND: Combined central and peripheral demyelination (CCPD) is rare and has never been reported as a spectrum disease in Han Chinese population. OBJECTIVES: To study the clinical features of CCPD in Han Chinese patients. METHODS: Twenty-two CCPD patients were selected from 788 demyelination cases. We reviewed and compared the clinical manifestation, laboratory data, electrophysiological examination, MRI and the prognosis. RESULTS: CCPD patients presented with sensory disturbance (86.4%), plegia (77.3%), cranial nerve involvement (77.3%), abnormal deep tendon reflexes (72.7%). CSF data showed increased CSF protein in 81% patients. Oligoclonal IgG bands (OB) were negative. Cortical or juxtacortical, periventricular, infratentorial lesions, thoracic and cervical spinal cord were mostly affected. Visual evoked potentials indicated optic nerves demyelinating in 50% cases. 21 CCPD patients were treated with intravenous immunoglobulin or steroids or both of them, and the efficacy was 33.3%, 54.5%, 71.4%, respectively. One case that showed no response to steroids plus intravenous immunoglobulin treatment was improved significantly after using cyclophosphamide. CONCLUSIONS: CCPD is a spectrum disease that can't be regarded as a simple combination of MS and CIDP. A suspected CCPD should receive brain and spinal MRI as well as electrophysiological examination to obtain a precise diagnosis.

Investigation of the Role of Campylobacter Infection in Suspected Acute Polyradiculoneuritis in Dogs.

BACKGROUND: Acute polyradiculoneuritis (APN) is an immune-mediated peripheral nerve disorder in dogs that shares many similarities with Guillain-Barré syndrome (GBS) in humans, in which the bacterial pathogen Campylobacter spp. now is considered to be a major triggering agent. Little information is available concerning the relationship between APN and Campylobacter spp. in dogs. HYPOTHESIS/OBJECTIVES: To estimate the association between Campylobacter spp. infection and APN. Associations with additional potential risk factors also were investigated, particularly consumption of raw chicken. ANIMALS: Twenty-seven client-owned dogs suffering from suspected APN and 47 healthy dogs, client-owned or owned by staff members. METHODS: Case-control study with incidence density-based sampling. Fecal samples were collected from each enrolled animal to perform direct culture, DNA extraction, and polymerase chain reaction (PCR) for detection of Campylobacter spp. In some cases, species identification was performed by sequence analysis of the amplicon. Data were obtained from the medical records and owner questionnaires in both groups. RESULTS: In cases in which the fecal sample was collected within 7 days from onset of clinical signs, APN cases were 9.4 times more likely to be positive for Campylobacter spp compared to control dogs (P < 0.001). In addition, a significant association was detected between dogs affected by APN and the consumption of raw chicken (96% of APN cases; 26% of control dogs). The most common Campylobacter spp. identified was Campylobacter upsaliensis. CONCLUSIONS AND CLINICAL IMPORTANCE: Raw chicken consumption is a risk factor in dogs for the development of APN, which potentially is mediated by infection with Campylobacter spp.

Subacute demyelinating polyradiculoneuropathy complicating Epstein-Barr virus infection in GATA2 haploinsufficiency.

INTRODUCTION: Autosomal dominant haploinsufficiency of GATA2 causes monocytopenia and natural killer cell lymphopenia, resulting in predisposition to mycobacterial, fungal, and viral infections. METHODS: Herein we report on the clinical, serologic, electrophysiologic, and pathologic evaluations of a 29-year-old woman with GATA2 haploinsufficiency and active Epstein-Barr virus (EBV) infection complicated by subacute painful neuropathy. RESULTS: Nerve conduction and electromyography studies showed predominantly demyelinating sensorimotor polyradiculoneuropathy. Lumbar spine MRI showed thickening and enhancement of the cauda equina nerve roots. Serum and cerebrospinal fluid anti-IgG and IgM EBV capsid and nucleic acid antibodies were positive. Sural nerve biopsy showed microvasculitis and an increased frequency of fibers with segmental demyelination. Intravenous immunoglobulin and steroids improved the patient's neuropathy. CONCLUSION: GATA2 mutation-related immunodeficiency may predispose to EBV-associated subacute demyelinating polyradiculoneuropathy by both viral susceptibility and immune dysregulation. In patients who present in this manner, immunodeficiency syndromes should be considered when lymphomatous infiltration is excluded. Immunotherapy can be helpful. Muscle Nerve 57: 150-156, 2018.

Immunotherapy-responsive allodynia due to distal acquired demyelinating symmetric (DADS) neuropathy.

INTRODUCTION: Distal acquired demyelinating symmetric (DADS) neuropathy is a distal variant of chronic inflammatory demyelinating polyradiculoneuropathy. It is characterized by chronic distal symmetric sensory or sensorimotor deficits. Sensory ataxia is a common clinical presentation. Nerve conduction studies typically show markedly prolonged distal motor latencies. METHODS: We report 2 patients with chronic progressive generalized pain and fatigue, with normal neurological examinations except for allodynia. RESULTS: Nerve conduction studies were typical of DADS neuropathy. Monoclonal protein studies were negative. Cerebrospinal fluid protein levels were elevated. Sural nerve biopsies revealed segmental demyelination and remyelination. One biopsy had marked endoneurial and epineurial lymphocytic infiltration. Immunomodulatory therapy alleviated the pain and fatigue and markedly improved distal motor latencies in both patients. CONCLUSIONS: DADS neuropathy can present with pain and a normal neurological examination apart from allodynia. Nerve conduction studies are necessary for diagnosis. These patients respond to immunotherapy better than typical DADS neuropathy patients with sensory ataxia. Muscle Nerve 54: 973-977, 2016.

Case Report of Lewis and Sumner Syndrome with Bilateral Vagus Nerves Paralysis for 16 Years.

This report describes a patient with dysphonia for 16 years in combination with asymmetric and progressive decrease in sense and power of both upper and lower extremities for the past 3 years. Electrophysiological study revealed asymmetric conduction block and abnormal sensory action potential in 4 limbs. The vagus nerves palsy and abnormal electrodiagnosis of the limbs led us to diagnose the disease as Lewis and Sumner syndrome, also called multifocal acquired demyelinating sensory and motor neuropathy diagnosis, which improved by corticosteroid consumption to some extent. This case is uncommon by its long time presentation and progression. To the best of the authors' knowledge, this is the first report of simultaneous bilateral vagus nerve palsy in combination with upper and lower limbs' demyelinating neuropathy. In conclusion, persistent dysphonia can be a part of the presentation of demyelinating neuropathy.

Analgesia epidural continua para trabajo de parto en una paciente con polineuropatía desmielinizante periférica / Continuous epidural analgesia for labour and delivery in a patient with demyelinating peripheral polyneuropathy

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Harlequin syndrome in a patient with putative autoimmune autonomic ganglionopathy.

The author presents a patient with Harlequin and Horner syndromes as part of an autoimmune autonomic ganglionopathy and suggests implication for work-up and management. In general, Harlequin and Horner syndromes are reported to be caused by either a structural lesion of the sympathetic pathway or, when no structural lesion is found, are presumed to be idiopathic. In this paper, a 76 year old man developed a Harlequin and Horner syndromes in the setting of subacute autonomic failure and other systemic features. The patient's symptoms improved with a short course of intravenous methylprednisolone. An autoimmune etiology should be considered in patients with Harlequin syndrome and immunomodulatory treatment could be attempted, especially when there is evidence of a more generalized autoimmune autonomic ganglionopathy.