scholarly journals Chronic inflammatory demyelinating polyradiculoneuropathy presenting as predominantly sciatic monomelic neuropathy

2020 ◽  
Vol 2 (1) ◽  
pp. e000045
Author(s):  
Shadi El-Wahsh ◽  
Cecilia Cappelen-Smith ◽  
Judith Spies
Keyword(s):  
Lower Limb ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Nerve Biopsy ◽  
Chronic Inflammatory Demyelinating Polyradiculoneuropathy ◽  
Lumbosacral Plexus ◽  
Proximal Muscle ◽  
Diagnostic Challenge ◽  
Nerve Roots ◽  
Muscle Groups ◽  
Atypical Presentations

BackgroundChronic inflammatory demyelinating polyneuropathy (CIDP) is a common yet underdiagnosed cause of potentially treatable chronic sensorimotor neuropathy. The classical form of the disease is characterised by symmetrical weakness in both distal and proximal muscle groups accompanied by sensory dysfunction and diminished tendon reflexes lasting more than 2 months.MethodThe diagnosis of CIDP is supplemented by electrodiagnostic studies and biopsy findings confirming demyelination, in accordance with well-established diagnostic criteria. Atypical presentations of CIDP often pose a diagnostic challenge.ResultsIn this paper, we present a case of isolated lower limb involvement due to CIDP to raise awareness of this focal lower limb variant. Of particular, significance is the use of lumbosacral plexus MRI to assist in the diagnosis.ConclusionFocal CIDP is an atypical presentation that should be considered in patients presenting with chronic monomelic neuropathy and should be investigated with electrodiagnostic studies, lumbar puncture, nerve biopsy and MRI of the nerve roots and plexuses.

Chronic Inflammatory Demyelinating Polyradiculoneuropathy

2019 ◽  
Vol 39 (05) ◽  
pp. 596-607 ◽  
Author(s):  
Jeffrey Shije ◽  
Thomas H. Brannagan
Keyword(s):  
Peripheral Nerves ◽  
Autoimmune Disorder ◽  
Nerve Biopsy ◽  
Chronic Inflammatory Demyelinating Polyradiculoneuropathy ◽  
Nerve Roots ◽  
Hematopoietic Stem ◽  
Immunomodulatory Agents ◽  
Csf Analysis ◽  
Degree Of Disability

AbstractChronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a relatively common autoimmune disorder affecting the peripheral nerves and nerve roots, often causing progressive or recurrent weakness with diminished reflexes. Electrodiagnostic (EDx) studies, cerebral spinal fluid (CSF) analysis, and nerve biopsy may help provide supportive evidence for the diagnosis. Most cases have a favorable response to one of the three first-line treatments: corticosteroids, IV immunoglobulin (IVIG) and plasmapheresis. Responses to these treatments may vary among individual patients. There is evidence that a small percentage of CIDP patients with IgG class 4 (IgG4) autoantibodies to paranodal proteins have characteristic clinical features and poorer response to IVIG. Chemotherapy and other immunomodulatory agents, as well as hematopoietic stem cell transplantation, may be considered in refractory cases. The degree of disability varies, and most patients require ongoing treatment to maintain stable disease, although long-term remission or cure may be achieved in some patients.

scholarly journals Development of Lower Extremity Strength in Ambulatory Children With Bilateral Spastic Cerebral Palsy in Comparison With Typically Developing Controls Using Absolute and Normalized to Body Weight Force Values

2021 ◽  
Vol 12 ◽  
Author(s):  
Nicolaos Darras ◽  
Eirini Nikaina ◽  
Magda Tziomaki ◽  
Georgios Gkrimas ◽  
Antigone Papavasiliou ◽  
...  
Keyword(s):  
Body Weight ◽  
Cerebral Palsy ◽  
Muscle Strength ◽  
Lower Limb ◽  
Age Groups ◽  
Knee Extensors ◽  
Spastic Cerebral Palsy ◽  
Typically Developing ◽  
Antagonist Muscles ◽  
Muscle Groups

This cross-sectional study aimed to examine the development of lower limb voluntary strength in 160 ambulatory patients with bilateral spastic cerebral palsy (CP) (106 diplegics/54 quadriplegics) and 86 typically developing (TD) controls, aged 7–16 years. Handheld dynamometry was used to measure isometric strength of seven muscle groups (hip adductors and abductors, hip extensors and flexors, knee extensors and flexors, and ankle dorsiflexors); absolute force (AF) values in pounds were collected, which were then normalized to body weight (NF). AF values increased with increasing age (p < 0.001 for all muscle groups), whereas NF values decreased through adolescence (p < 0.001 for all muscle groups except for hip abduction where p = 0.022), indicating that increases in weight through adolescence led to decreases in relative force. Both AF and NF values were significantly greater in TD subjects when compared with children with CP in all muscle and all age groups (p < 0.001). Diplegics and quadriplegics demonstrated consistently lower force values than TD subjects for all muscle groups, except for the hip extensors where TD children had similar values with diplegics (p = 0.726) but higher than quadriplegics (p = 0.001). Diplegic patients also exhibited higher values than quadriplegics in all muscles, except for the knee extensors where their difference was only indicative (p = 0.056). The conversion of CP subjects' force values as a percentage of the TD subjects' mean value revealed a pattern of significant muscle strength imbalance between the CP antagonist muscles, documented from the following deficit differences for the CP muscle couples: (hip extensors 13%) / (hip flexors 32%), (adductors 27%) / (abductors 52%), and (knee extensors 37%) / (knee flexors 53%). This pattern was evident in all age groups. Similarly, significant force deficiencies were identified in GMFCS III/IV patients when compared with TD children and GMFCS I/II patients. In this study, we demonstrated that children and adolescents with bilateral CP exhibited lower strength values in lower limb muscles when compared with their TD counterparts. This difference was more prevalent in quadriplegic patients and those with a more severe impairment. An important pattern of muscle strength imbalance between the antagonist muscles of the CP subjects was revealed.

scholarly journals Diagnostic challenges in chronic inflammatory demyelinating polyradiculoneuropathy

Brain ◽  
2020 ◽  
Vol 143 (11) ◽  
pp. 3214-3224
Author(s):  
Filip Eftimov ◽  
Ilse M Lucke ◽  
Luis A Querol ◽  
Yusuf A Rajabally ◽  
Camiel Verhamme
Keyword(s):  
Peripheral Nerves ◽  
Diagnostic Tests ◽  
High Sensitivity ◽  
Nerve Biopsy ◽  
Chronic Inflammatory Demyelinating Polyradiculoneuropathy ◽  
Control Group ◽  
Nerve Ultrasound ◽  
Hand Performance ◽  
The One ◽  
Csf Examination

Abstract Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) consists of a spectrum of autoimmune diseases of the peripheral nerves, causing weakness and sensory symptoms. Diagnosis often is challenging, because of the heterogeneous presentation and both mis- and underdiagnosis are common. Nerve conduction study (NCS) abnormalities suggestive of demyelination are mandatory to fulfil the diagnostic criteria. On the one hand, performance and interpretation of NCS can be difficult and none of these demyelinating findings are specific for CIDP. On the other hand, not all patients will be detected despite the relatively high sensitivity of NCS abnormalities. The electrodiagnostic criteria can be supplemented with additional diagnostic tests such as CSF examination, MRI, nerve biopsy, and somatosensory evoked potentials. However, the evidence for each of these additional diagnostic tests is limited. Studies are often small without the use of a clinically relevant control group. None of the findings are specific for CIDP, meaning that the results of the diagnostic tests should be carefully interpreted. In this update we will discuss the pitfalls in diagnosing CIDP and the value of newly introduced diagnostic tests such as nerve ultrasound and testing for autoantibodies, which are not yet part of the guidelines.

scholarly journals Chronic inflammatory axonal polyneuropathy

2020 ◽  
Vol 91 (11) ◽  
pp. 1175-1180 ◽  
Author(s):  
Shin J Oh ◽  
Liang Lu ◽  
Mohammad Alsharabati ◽  
Marla B Morgan ◽  
Peter King
Keyword(s):  
Sural Nerve ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Axonal Neuropathy ◽  
Nerve Biopsy ◽  
Spinal Fluid ◽  
Motor Neuropathy ◽  
Distinct Entity ◽  
Axonal Polyneuropathy ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Therapeutic Responses

ObjectivesChronic inflammatory axonal polyneuropathy (CIAP) is defined on the basis of the clinical, electrophysiological and nerve biopsy findings and therapeutic responses of ‘immunotherapy responding chronic axonal polyneuropathy (IR-CAP)’.MethodsThe diagnosis of IR-CAP was made when all of three of the following mandatory criterion were met: (1) acquired, chronic progressive or relapsing symmetrical or asymmetrical polyneuropathy with duration of progression >2 months; (2) electrophysiological evidence of axonal neuropathy in at least two nerves without any evidence of ‘strict criteria of demyelination’; and (3) definite responsiveness to immunotherapy.ResultsThirty-three patients with IR-CAP showed similar clinical features of chronic inflammatory demyelinating polyneuropathy (CIDP) except ‘motor neuropathy subtype’. High spinal fluid protein was found in 27/32 (78%) cases. ‘Inflammatory axonal neuropathy’ was proven in 14 (45%) of 31 sural nerve biopsies.DiscussionsIR-CAP could well be ‘axonal CIDP’ in view of clinical similarity, but not proven as yet. Thus, IR-CAP is best described as CIAP, a distinct entity that deserves its recognition in view of responsiveness to immunotherapy.ConclusionDiagnosis of CIAP can be made by additional documentation of ‘inflammation’ by high spinal fluid protein or nerve biopsy in addition to the first two diagnostic criteria of IR-CAP.

scholarly journals Vasculitis and the peripheral nervous system

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_3) ◽  
pp. iii55-iii59 ◽  
Author(s):  
Lionel Ginsberg
Keyword(s):  
Lower Limb ◽  
Vessel Wall ◽  
Early Recognition ◽  
Systemic Vasculitis ◽  
Nerve Biopsy ◽  
Chronic Neuropathic Pain ◽  
Vasculitic Neuropathy ◽  
Vasa Nervorum ◽  
Sensory Recovery ◽  
Systemic Process

Abstract Peripheral neuropathy is a common feature of systemic vasculitis and can also occur when vessel wall inflammation is confined to the vasa nervorum, as a tissue-specific condition—non-systemic vasculitic neuropathy (NSVN). Typically, the clinical picture in both systemic and non-systemic cases is of a lower limb predominant, distal, asymmetric or multifocal neuropathy, which is painful and subacute in onset. For NSVN, nerve biopsy is required to make the diagnosis, and nerve biopsy also has a role when vasculitic neuropathy is suspected and a systemic process has not yet declared itself. Early recognition of the disorder is important, because it is treatable, and without treatment potentially disabling, or even lethal if part of an undiagnosed systemic process. Treatment is generally with combination therapy (glucocorticoid plus other immunosuppressant), after which motor and sensory recovery are likely to occur, albeit slowly, but the patient may be left with chronic neuropathic pain.

scholarly journals Benign monomelic amyotrophy with proximal upper limb involvement: case report

2007 ◽  
Vol 65 (2b) ◽  
pp. 524-527 ◽  
Author(s):  
Marco Antonio Orsini Neves ◽  
Marcos R.G. de Freitas ◽  
Mariana Pimentel de Mello ◽  
Carlos Henrique Dumard ◽  
Gabriel R. de Freitas ◽  
...  
Keyword(s):  
Case Report ◽  
Upper Limb ◽  
Lower Limb ◽  
Clinical Manifestations ◽  
Rare Condition ◽  
Insidious Onset ◽  
Rare Location ◽  
Muscle Groups ◽  
Clinical Stabilization ◽  
The Right

Monomelic amyotrophy (MA) is a rare condition in which neurogenic amyotrophy is restricted to an upper or lower limb. Usually sporadic, it usually has an insidious onset with a mean evolution of 2 to 4 years following first clinical manifestations, which is, in turned, followed by stabilization. We report a case of 20-years-old man who presented slowly progressive amyotrophy associated with proximal paresis of the right upper limb, which was followed by clinical stabilization 4 years later. Eletroneuromyography revealed denervation along with myofasciculations in various muscle groups of the right upper limb. We call atention to this rare location of MA, as well as describe some theories concerning its pathophysiology .

scholarly journals An unusual cause of paraesthesia in a neo-adjuvant gastro-oesophageal cancer

2019 ◽  
Vol 12 (8) ◽  
pp. e225450 ◽  
Author(s):  
Sarah Derby ◽  
Janet Graham ◽  
David McIntosh
Keyword(s):  
Oesophageal Cancer ◽  
Lower Limb ◽  
Vascular Complications ◽  
Diagnostic Process ◽  
Diagnostic Challenge ◽  
Ongoing Debate ◽  
Unique Challenge ◽  
Increased Risk ◽  
Neo Adjuvant Chemotherapy ◽  
Unusual Finding

There is ongoing debate about the best neo-adjuvant strategy for localised resectable oesophageal cancer, however chemotherapy is often employed. Both oesophageal cancer and cisplatin carry an increased risk of thrombosis. Here, we look at an unusual finding in a previously fit woman who presented following neo-adjuvant chemotherapy for resectable oesophageal cancer with increasing difficulty in walking and lower limb paraesthesia. This case looks particularly at the diagnostic challenge and concerns raised in a patient undergoing radical treatment as well as the challenge of complications secondary to treatment with chemotherapy. Willingness to reassess and revisit is a vital part of the diagnostic process. Vascular complications of a disease can be notorious to diagnose and, as in this case can mimic arguably more logical diagnoses. Cancer care provides the unique challenge of investigating unusual presentations related both to disease and treatment.

Isokinetic evaluation of hip strength muscle groups in unilateral lower limb amputees

2001 ◽  
Vol 9 (4) ◽  
pp. 163-169 ◽  
Author(s):  
J.L. Croisier ◽  
B. Maertens de Noordhout ◽  
D. Maquet ◽  
G. Camus ◽  
S. Hac ◽  
...  
Keyword(s):  
Lower Limb ◽  
Hip Strength ◽  
Muscle Groups ◽  
Isokinetic Evaluation ◽  
Lower Limb Amputees

Relationship of intraoperative electrophysiological criteria to outcome after selective functional posterior rhizotomy

1995 ◽  
Vol 83 (1) ◽  
pp. 18-26 ◽  
Author(s):  
Paul Steinbok ◽  
Bengt Gustavsson ◽  
John R. W. Kestle ◽  
Ann Reiner ◽  
D. Doug Cochrane
Keyword(s):  
Lower Limb ◽  
Phase 2 ◽  
Nerve Roots ◽  
Phase 3 ◽  
Limb Function ◽  
Content Type ◽  
Limb Spasticity ◽  
Lower Limb Spasticity ◽  
Lower Limb Function ◽  
Fine Print

✓ At British Columbia's Children's Hospital, the criteria used in selective functional posterior rhizotomy (SFPR) evolved in three distinct phases. In Phase 1 the electrophysiological criteria for abnormality included a low threshold to a single stimulation, a sustained response to 50-Hz stimulation, and spread outside the segmental level being stimulated. In Phase 2 the electrophysiological criteria were unchanged, but fewer L3–4 nerve roots were cut. In Phase 3, fewer L3–4 nerve roots were cut, as in Phase 2, but based on the results of posterior nerve root stimulation in nonspastic controls, the only electrophysiological criterion used was contralateral and suprasegmental spread. The present study examined the relationship between the criteria used in each phase and patient outcome. The records of 77 consecutive children who underwent SFPR and had a minium follow-up period of 1 year were reviewed, comprising 25, 19, and 33 patients in Phases 1, 2, and 3, respectively. Outcome parameters included quantitative assessments of lower-limb spasticity and range of motion, and qualitative assessments of lower-limb function. In Phase 3, 52% of the nerve roots were cut, compared to 66% in Phases 1 and 2. In all three phases there was a significant decrease in lower-limb spasticity and an increase in range of movement, with the smallest decrease in spasticity in Phase 3. Over 90% of children in each phase improved with respect to lower-limb function, and excluding independent walkers and quadriplegics confined to a wheelchair, improvement in the level of ambulation occurred in 87.5%, 71.4%, and 73.7% of patients, in Phases 1, 2, and 3, respectively.

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