CISP-Plus: Expanding the Spectrum of Chronic Immune Sensory Polyradiculopathy (CISP)

Objectives:Sensory loss with normal nerve conduction studies (NCS) from focal sensory root inflammatory demyelination is characteristic of chronic immune sensory polyradiculopathy (CISP). However, non-pure cases involving motor and distal sensory nerves exist (CISP-plus). We hypothesize that CISP-plus and CISP are fundamentally part of the same syndrome through comparison of clinical, neurophysiological and pathological features.Methods:CISP-plus (primary dorsal root with lesser motor and sensory nerve involvement) and CISP cases were retrospectively analyzed (1986-2019).Results:We identified 44 CISP-plus and 28 CISP cases (n=72) with 86% (38/44) of CISP-plus and 79% (22/28) of CISP patients experiencing imbalance. On examination, large fiber sensory loss was present in 98% (43/44) of CISP-plus and 96% (27/28) of CISP. Gait ataxia was evident in 93% (41/44) of CISP-plus and 79% (22/28) of CISP. Mild distal weakness was common in CISP-plus (75%, 33/44). NCS showed mild abnormalities in all CISP-plus and were normal (by definition) in all CISP. Elevated cerebral spinal fluid (CSF) protein, slowing of somatosensory evoked potentials and MRI root enhancement occurred in most CISP-plus and CISP cases. Eleven CISP-plus nerve biopsies showed loss of large myelinated fibers and onion-bulb formation most prominent in rootlet biopsies. Immunotherapy resulted in marked improvement of gait ataxia in 84% (27/32) of CISP-plus and 93% (14/15) of CISP cases with return to normal neurological examination in half (25/46).Conclusion:The recognition of CISP-plus expands the spectrum of CIDP by combining CISP-plus (predominant sensory polyradiculopathy with mild motor and sensory nerve involvement) with pure-CISP (focal sensory polyradiculopathy) together as proximal sensory CIDP.

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Hereditary Motor Sensory Neuropathy Type I Presenting as Scapuloperoneal Atrophy (Davidenkow Syndrome) Electrophysiological and Pathological Studies

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/s0317167100036404 ◽

1986 ◽

Vol 13 (3) ◽

pp. 264-266 ◽

Cited By ~ 15

Author(s):

Gabriel M. Ronen ◽

Noel Lowry ◽

John H. Wedge ◽

Harvey B. Sarnat ◽

Alan Hill

Keyword(s):

Muscular Atrophy ◽

Sensory Neuropathy ◽

Nerve Biopsy ◽

Sural Nerve Biopsy ◽

Sensory Loss ◽

Type I ◽

Pes Cavus ◽

Hereditary Motor ◽

Bulb Formation ◽

Onion Bulb Formation

ABSTRACT:A 14 year old boy with scapuloperoneal muscular atrophy, pes cavus, areflexia and distal sensory loss (Davidenkow syndrome) is described. Nerve conduction velocities were diminished. Sural nerve biopsy demonstrated a reduction in the number of myelinated fibers and early “onion-bulb” formation. These observations support the hypothesis that the scapuloperoneal amyotrophy associated with distal sensory loss may represent a variant of type I hereditary motor sensory neuropathy.

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Abstract MP45: Deletion Of The Transient Receptor Vanilloid-1 (TRPV1) Channel In Rats Attenuates 2 Kidney 1 Clip Hypertension

Hypertension ◽

10.1161/hyp.78.suppl_1.mp45 ◽

2021 ◽

Vol 78 (Suppl_1) ◽

Author(s):

Sean D Stocker ◽

Leon J DeLalio

Keyword(s):

Heart Rate ◽

Renovascular Hypertension ◽

Sensory Nerve ◽

Experimental Models ◽

Sensory Nerves ◽

Male Rats ◽

Wild Type ◽

Renal Nerve ◽

Trpv1 Channels ◽

Arterial Blood

Renal denervation lowers arterial blood pressure (ABP) in both clinical populations and multiple experimental models of hypertension. This therapeutic effect is partly attributed to the removal of overactive renal sensory nerves that increase sympathetic efferent activity and ABP. Renal sensory nerves highly express TRPV1 channels, and administration of the TRPV1 agonist capsaicin increases renal sensory nerve activity. However, the extent by which TRPV1 channels directly contribute to renal nerve dependent models of hypertension has not been tested. To test this hypothesis, we generated a novel TRPV1 -/- rat using CRISPR/Cas9 and deletion of exon 3. Male and female TRPV1 -/- and wild-type littermates (8-12 weeks) were instrumented with telemetry. At 2 weeks later, renovascular hypertension via renal stenosis was produced by placement of a PTFE cuff (0.16 x 0.22 inches, 1mm long) around the right renal artery. Male TRPV1 -/- and wild-type rats had no differences in baseline mean ABP (99±2 vs 98±3 mmHg, respectively; n=7-9) or heart rate (390±7 vs 400±8 bpm, respectively). Renal stenosis significantly increased mean ABP in both groups; however, mean ABP was significantly lower at Day 28 in male TRPV1 -/- versus wild-type rats (125±8 vs 155±2 mmHg, respectively: P<0.01). Ganglionic blockade with chlorisondamine (2.5mg/kg, sc) at Day 28 produced a smaller fall in mean ABP of male TRPV1 -/- versus wild-type rats (-53±4 vs -86±3 mmHg, respectively; P<0.001). On the other hand, female TRPV1 -/- and wild-type rats had no differences in baseline mean ABP (102±2 vs 104±1 mmHg, respectively; n=6-9) or heart rate (419±8 vs 410±7 bpm, respectively). Renal stenosis significantly increased mean ABP in both groups; however, there were no differences at Day 28 between female TRPV1 -/- versus wild-type rats (117±8 vs 122±6 mmHg, respectively). Moreover, the increase in mean ABP was smaller in females versus males. The ganglionic blocker chlorisondamine produced similar depressor responses in female TRPV1 -/- versus wild-type rats (-64±7 vs -65±7 mmHg, respectively). These findings illustrate a sex difference in renovascular hypertension in rats, but importantly indicate that TRPV1 channels contribute to the established phase of renovascular hypertension in male rats.

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Very Early Neurophysiological Study in Guillain-Barre Syndrome

European Neurology ◽

10.1159/000494261 ◽

2018 ◽

Vol 80 (1-2) ◽

pp. 100-105 ◽

Cited By ~ 1

Author(s):

Jiaoting Jin ◽

Fangfang Hu ◽

Xing Qin ◽

Xuan Liu ◽

Min Li ◽

...

Keyword(s):

Nerve Conduction ◽

Nerve Conduction Velocity ◽

Tibial Nerve ◽

Early Stage ◽

Motor Nerve ◽

Sensory Nerve ◽

Sensory Nerves ◽

Motor Nerve Conduction ◽

Abnormal Rate ◽

Motor Nerves

Purpose: The diagnosis of Guillain-Barre syndrome (GBS) in the very early stage may be challenging. Our aim was to report the neurophysiological abnormalities in GBS within 4 days of clinical onset. We expected that GBS will be diagnosed by the assistance of neurophysiological study in the very early stage. Methods: We prospectively recruited patients with a diagnosis of GBS discharged from First Affiliated Hospital of Xi’an Jiaotong University and Xi Jing Hospital. Patients were classified into 3 groups according to the onset of symptoms to electromyography examination interval (OEI). The neurophysiological findings were carried out using standard procedures. All patients were examined by the same experienced neurophysiologist. Results: There were not significant group differences in abnormal rate, distal motor latency (DML), motor nerve conduction velocity (MNCV), F response (FR), compound muscle action potential (CMAP), conduction block (CB), sensory nerve action potential (SNAP), and sensory nerve conduction velocity among OEI ≤4 days, 4< OEI ≤10 days, and OEI > 10 days groups. Motor nerves were more affected than sensory nerves in neurophysiological presentation in very early stage patients. The difference of motor nerves and sensory nerves was statistically significant in lower limbs, but was not in upper limbs. In motor nerve conduction studies, the abnormal rate of DML, MNCV, FR, CB was more common seen in ulnar and peroneal nerve than median and tibial nerve, the abnormal rate of CMAP was the same in ulnar, median, peroneal and tibial nerve. In sensory nerve conduction studies, the abnormal rate of ulnar nerve and median nerve was higher than the superficial peroneal nerve and sural nerve. The OEI was not correlated with the SNAP decrease rate of median (r = 0.10, p = 0.23) and ulnar (r = 0.26, p = 0.06) but was statistically correlated with sural SNAP decrease rate (r = 0.29, p = 0.04). The sural-sparing pattern phenomenon was the most commonly discovered phenomenon in very early stage patients (OEI ≤4 days), followed by patients with 4< OEI ≤10 days, ultimately found in patients with OEI > 10 days. Conclusions: We suggest performing neurophysiological examination as soon as possible for suspected GBS patients, particularly focusing on multi-spots inspection of ulnar and peroneal nerves, and paying close attention to sural-sparing patterns.

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Phenomenon of Schwann cell apoptosis in a case of congenital hypomyelinating neuropathy with basal lamina onion bulb formation

Brain and Development ◽

10.1016/s0387-7604(02)00081-5 ◽

2002 ◽

Vol 24 (7) ◽

pp. 727-731 ◽

Cited By ~ 4

Author(s):

Anna Fidziańska ◽

Hanna Drac ◽

Janina Rafałowska

Keyword(s):

Schwann Cell ◽

Basal Lamina ◽

Cell Apoptosis ◽

Onion Bulb ◽

Bulb Formation ◽

Onion Bulb Formation ◽

Congenital Hypomyelinating Neuropathy

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Localized Hypertrophic Mononeuropathy Involving the Tibial Nerve

Neurosurgery ◽

10.1227/00006123-198808000-00015 ◽

1988 ◽

Vol 23 (2) ◽

pp. 218-221 ◽

Cited By ~ 18

Author(s):

Vasudeva G. Iyer ◽

Henry D. Garreston ◽

Ryland P. Byrd ◽

Steven J. Reiss

Keyword(s):

Differential Diagnosis ◽

Tibial Nerve ◽

Posterior Tibial Nerve ◽

Motor Weakness ◽

Posterior Tibial ◽

Bulb Formation ◽

Histological Appearance ◽

Single Nerve ◽

Onion Bulb Formation ◽

Localized Hypertrophic Neuropathy

ABSTRACT Localized hypertrophic neuropathy (LHN) is a disorder of unknown cause that presents with progressive, predominantly motor weakness in the distribution of a single nerve, with the histological appearance of the “onion bulb” formation. Because of its rarity, the condition is often ignored in the differential diagnosis of mononeuropathy. We report a case of LHN involving the posterior tibial nerve and review the literature.

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Neurology

The Pocketbook for PACES ◽

10.1093/oso/9780199574186.003.0014 ◽

2012 ◽

Author(s):

Gerry Christofi ◽

Guy Leschziner

Keyword(s):

Motor Neuron ◽

Nerve Root ◽

Time Of Day ◽

Sensory Loss ◽

Sensory Level ◽

Gait Ataxia ◽

Pes Cavus ◽

Charcot Marie Tooth Disease ◽

Neurological Problem ◽

Sensory Involvement

The neurology section of the PACES examination is often the major cause of (unnecessary!) anxiety for MRCP candidates. The key is to approach the patient in a logical fashion. Some neurology cases are simply an exercise in pattern recognition – noticing the frontal balding and ptosis of myotonic dystrophy, the distal wasting and pes cavus of Charcot–Marie–Tooth disease, for example. However, in those cases without obvious clues to the underlying diagnosis, a clear systematic approach will usually pay dividends. When faced with a neurological problem, the first question that should be posed is the site of the lesion. During the course of the examination, identify signs that might help in localization: • Cortex: signs of dysfunction of higher cognitive function. • Subcortical: upper motor neuron (UMN) signs (hypertonia, pyramidal pattern of weakness, hyper-reflexia, extensor plantars), slowness of thought. • Basal ganglia: cogwheel rigidity, resting tremor, bradykinesia, postural instability, dyskinesias, dystonias. • Brainstem: cranial nerve abnormalities with contralateral UMN signs. • Cerebellum: gait ataxia, nystagmus, finger-nose ataxia, past-pointing. • Spinal cord: bilateral UMN signs, presence of a sensory level. • Nerve root: lower motor neuron (LMN) signs (wasting, weakness, hyporeflexia, sensory loss) in a myotomal or dermatomal distribution. • Single or multiple nerve/plexus: LMN signs that are focal, and are not consistent with a nerve root lesion. • Polyneuropathy: LMN signs, more pronounced distally, affecting the legs more than the hands, diminished reflexes, sensory signs. • Neuromuscular junction: weakness without sensory involvement or significant wasting, usually but not invariably proximal, which fluctuates (either with time of day or during the course of the examination). • Muscle: wasting and weakness with normal reflexes and sensation. Once the lesion has been localized, consider the disease processes that commonly affect that site. Clues may be obtained from the history, if you are permitted to ask questions. The most helpful aspect of the history is usually the speed of onset: • Seconds: electrical disturbance (i.e. epilepsy), trauma. • <5 minutes: infarction. • > 5 minutes: migraine, haemorrhage. • Minutes–hours: infection, inflammation, drugs. • Hours–days: infection, inflammation, nutritional, drugs.

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TrkA inhibitor promotes motor functional regeneration of recurrent laryngeal nerve by suppression of sensory nerve regeneration

Scientific Reports ◽

10.1038/s41598-020-72288-w ◽

2020 ◽

Vol 10 (1) ◽

Author(s):

Hiroshi Suzuki ◽

Koji Araki ◽

Toshiyasu Matsui ◽

Yuya Tanaka ◽

Kosuke Uno ◽

...

Keyword(s):

Recurrent Laryngeal Nerve ◽

Nerve Regeneration ◽

Vocal Fold ◽

Compound Muscle Action Potential ◽

Motor Nerve ◽

Sensory Nerve ◽

Laryngeal Nerve ◽

Sensory Nerves ◽

Nucleus Ambiguus ◽

Functional Regeneration

Abstract Recurrent laryngeal nerve (RLN) injury, in which hoarseness and dysphagia arise as a result of impaired vocal fold movement, is a serious complication. Misdirected regeneration is an issue for functional regeneration. In this study, we demonstrated the effect of TrkA inhibitors, which blocks the NGF-TrkA pathway that acts on the sensory/automatic nerves thus preventing misdirected regeneration among motor and sensory nerves, and thereby promoting the regeneration of motor neurons to achieve functional recovery. RLN axotomy rat models were used in this study, in which cut ends of the nerve were bridged with polyglycolic acid-collagen tube with and without TrkA inhibitor (TrkAi) infiltration. Our study revealed significant improvement in motor nerve fiber regeneration and function, in assessment of vocal fold movement, myelinated nerve regeneration, compound muscle action potential, and prevention of laryngeal muscle atrophy. Retrograde labeling demonstrated fewer labeled neurons in the vagus ganglion, which confirmed reduced misdirected regeneration among motor and sensory fibers, and a change in distribution of the labeled neurons in the nucleus ambiguus. Our study demonstrated that TrkAi have a strong potential for clinical application in the treatment of RLN injury.

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Neuropeptides and nasal secretion

AJP Lung Cellular and Molecular Physiology ◽

10.1152/ajplung.1991.261.4.l223 ◽

1991 ◽

Vol 261 (4) ◽

pp. L223-L235 ◽

Cited By ~ 7

Author(s):

J. N. Baraniuk ◽

M. Kaliner

Keyword(s):

Nasal Mucosa ◽

Defense Mechanism ◽

Sensory System ◽

Sensory Nerve ◽

Sympathetic Nerves ◽

Mucosal Injury ◽

Sensory Nerves ◽

Neurokinin A ◽

Sympathetic Nervous Systems ◽

Glandular Secretion

The nasal mucosa is innervated by the sensory, parasympathetic, and sympathetic nervous systems. Nociceptive sensory nerves are stimulated by mucosal injury, inhalation of irritants, or mast cell degranulation and release of the calcitonin gene-related peptide, the tachykinins substance P and neurokinin A, and other peptides by the axon response mechanism. Sensory nerve stimulation initiates systemic reflexes, such as the sneeze, and central parasympathetic reflexes which release acetylcholine, vasoactive intestinal peptide, and other peptides and lead to glandular secretion. In concert, these proinflammatory neural responses lead to vasodilation, vascular permeability, and glandular secretion. Sympathetic nerves release neuropeptide Y and norepinephrine, potent vasoconstrictors which act to decompress the nasal mucosa and produce nasal patency. The balance between the effects of parasympathetic and sympathetic neurotransmitters may regulate nasal homeostasis, whereas the nociceptive sensory system may be held in reserve as a defense mechanism. Dysfunction of these systems may lead to pathological nasal syndromes. In the future, specific neuropeptide agonists and antagonists may be useful for the treatment of human rhinitic diseases.

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