Multiple Nerve Involvement Research Articles

Perineural invasion for risk stratification in cutaneous squamous cell carcinoma: a scoping review.

Microscopic perineural invasion (mPNI) is a histopathological characteristic that can be found in cutaneous squamous cell carcinoma (cSCC). In the eight edition of the American Joint Committee on Cancer (AJCC), mPNI defined as the involvement of nerves ≥0.1mm and nerves deeper than the dermis is included in risk stratification of cSCC. The question remains whether other mPNI features are important for optimal cSCC staging. We aim to summarize the evidence from published studies on the independent association between various mPNI features and the risk of recurrence, metastasis and disease-specific death in patients with cSCC. Embase, PubMed, and Web of Science were searched from January 2023 to February 2024 to identify studies that reported the prognostic impact of mPNI features in patients ≥18 years with histopathologically verified cSCC. Data on study and tumour characteristics were extracted. Nineteen studies met the inclusion criteria and evaluated one or more mPNI features in cSCC including nerve diameter, the extent of mPNI, the number of affected nerves, and depth of mPNI. Two studies provided evidence that 'mPNI ≥0.1mm' and 'mPNI deeper than the dermis' are significantly and independently associated with poor prognosis after correction for other mPNI features and high-risk factors. One of these studies additionally identified 'involvement of ≥3 nerves' as an independent and significant predictor of higher risk of local recurrence (HR, 2.17; 95% CI, 1.03-4.56; p=0.04). Besides 'nerve diameter of ≥0.1mm' and 'depth of mPNI involvement', 'involvement of multiple nerves' was found to be an independent risk factor for poor prognosis and should also be considered for appropriate risk stratification.

Impact of thyroid carcinoma invasion of recurrent laryngeal nerve on cervical lymph node metastasis.

Papillary thyroid carcinoma (PTC) has a favorable prognosis. However, involvement of the recurrent laryngeal nerve (RLN) significantly increases the risk of recurrence. RLN invasion was an important factor in determining the extent of thyroid surgery. The purpose of this study was to compare clinicopathologic features and characterize risk factors of central and lateral lymph node metastasis (LLNM) of RLN invasion in patients with PTC. A retrospective review was conducted of 130 patients with PTCs who had exclusive tumor involvement of the RLN at our institution between January 2014 and February 2019. All patients underwent total thyroidectomy and high-dose radioactive iodine (RAI) therapy. The clinicopathologic factors and prognostic outcomes of the patients with solitary and multiple RLN involvements were compared. Kaplan-Meier method was performed to compare the outcomes of tumor recurrence. Univariate and multivariate logistic regression analyses were used to identify risk factors associated with LLNM. The invasion of the RLN was similar on both sides, with 58.5% on the right, 40.0% on the left, and 1.5% on both sides. Significant differences were observed in tumor size (p < 0.001), lymph node metastasis classification (p = 0.002), RLN resection (p < 0.001), and thyroglobulin (p = 0.010) in the solitary and multiple groups. During the median follow-up of 67 months, 9 (6.9%) patients developed recurrence. There were no statistical differences in recurrence for age, tumor size, gender, multifocality, lymph node metastasis (LNM), and RLN resection. According to receiver operating characteristic (ROC) curve analyses, a cut-off of tumor size > 1.7 cm was identified as the most sensitive and specific predictor of RLN with multiple involvements or LNM invasion. Univariate and multivariate analyses revealed that central lymph node metastasis (CLNM) and RLN invasion by LNM can serve as independent risk factors for LLNM (p = 0.006 and p < 0.001, respectively). Our results indicate that recurrence was comparable in patients with solitary and multiple RLN involvements. Multiple RLN involvement was associated with pathological features such as larger tumors, RLN resection, and LLNM. The presence of LNM invading RLN and multiple nerve involvement increases the risk of intraoperative RLN resection. A higher risk of multiple invasion or LNM invasion should be considered when tumor size > 1.7 cm. The presence of CLNM and RLN invaded by LNM were independent predictors for LLNM, which could aid surgeons in deciding on lateral lymph node dissection.

Outcome of patients with traumatic cranial nerve palsy admitted to a university hospital in Nepal

BackgroundCranial nerve palsy (CNP) is a common complication of traumatic brain injury (TBI). Despite a high incidence of TBI in Nepal (382 per 100,000), literature on the specific management and outcome of CNP is lacking. This study aimed to examine the outcomes of TBI patients involving single versus multiple CNP.MethodsA retrospective chart review of 170 consecutive TBI patients admitted to the tertiary neurosurgical center in Nepal between April 2020 and April 2022 was conducted. Demographic, clinical, and etiological characteristics; imaging findings; and management strategies were recorded, compared, and analyzed using descriptive statistics. The Glasgow Outcome Scale Extended (GOSE) was used to measure the outcomes in two groups of patients (single and multiple CNP) at 3 months.ResultsOut of 250 eligible patients, 80 were excluded and CNP was noted in 29 (17.1%) of the remaining 170. The median age was 34.9 years, and falls (60.6%) were the most common cause of trauma. TBI severity was categorized based on GCS: mild (82.4%), moderate (15.9%), and severe (1.8%). Cranial nerve involvement was seen in 29 (17.05%) patients: single cranial nerve involvement in 26 (89.65%) and multiple nerve involvement in 3 (10.34%). The most common isolated cranial nerve involved was the oculomotor nerve (37.9%). CT findings revealed a maximum of skull fractures with no significant association between CNP and CT findings.ConclusionsCNP is a common consequence of TBI with the most common etiology being falls followed by RTA. Single CNP was more common than multiple CNP with no significant difference in the outcome in the 3-month GOSE score. Further research is needed to determine the burden of traumatic CNP and establish specific management guidelines for different types of CNP.

A clinicoepidemiological study of leprosy among children and adolescents in a tertiary care centre in the post elimination era

Leprosy amongst younger age indicates active foci of transmission in the community. Our aim is to analyse the epidemiological profile, clinical spectrum, reactions and deformities of leprosy among children and adolescents. This is a prospective observational study conducted on all newly diagnosed leprosy patients under the age of 19 years for one year. Of 113 newly diagnosed leprosy cases, 38 patients were under 19 years of age. Male-female ratio was 5.3:1. Mean age was 16.6 years. Borderline tuberculoid (BT) was the most common type followed by borderline lepromatous (BL) and Indeterminate (I) leprosy most common manifestation was hypopigmented anaesthetic skin lesion over exposed parts. 79% had multiple nerve trunk involvement of which ulnar nerve was the most common. Reaction and deformity were found to be in 21% and 18.3%. Both were seen only in multibacillary cases and predominantly in 15-19years of age. Risk factors for reactions were male sex, increasing age, labourers, borderline spectrum, skin lesion involving the nerve trunk, multiple nerve involvement and smear positivity. Risk factors for deformities were migrants in lower socio-economic status, increased duration of active disease and delayed diagnosis. Leprosy, a disease of long incubation period, among adolescents may indicate missed cases of childhood leprosy. Training through integrated service in early diagnosis and its complication, introduction of an exclusive type of treatment (MB MDT) for all categories for a shortened duration and newer drugs for reactions, sustained commitment to follow-up care and health education among community is a need at this post elimination era.

Extensive Perineural Invasion vs Nerve Caliber to Assess Cutaneous Squamous Cell Carcinoma Prognosis

Perineural invasion (PNI) is an adverse risk feature in cutaneous squamous cell carcinoma (CSCC) that affects patient prognosis and disease management. However, research comparing different PNI patterns on patient outcomes is limited. To compare 4 assessments of PNI in CSCC, their associations with poor outcomes, and implications for their inclusion in the Brigham and Women's Hospital (BWH) staging system. This retrospective cohort study was performed at a single tertiary care institution and compared 4 PNI assessments: nerve caliber, number of involved nerves per section, PNI maximal depth, and PNI location with respect to tumor. Patients with primary, localized, invasive CSCC with PNI diagnosed between January 1, 2000, and December 31, 2017, were identified via an electronic in-house database. Available pathology slides were secondarily reviewed by study authors. Relevant patient and tumor characteristics and outcomes were abstracted from the medical record. Data analysis was performed between September 6 and October 20, 2022. Risks of recurrence, disease-specific death, and a composite end point (any poor outcome) were calculated via multivariable stepwise Fine and Gray competing-risks regression. Considered revisions to the BWH staging system were assessed via receiver operating characteristic curves and test characteristics. This study included 140 patients with CSCC, with a mean (SD) age of 75.1 (11.2) years. More than half of the patients were men (93 [66.4%]), and most identified as White (132 [94.3%]). Of the 4 PNI assessments studied, only involvement of multiple nerves was associated with poor outcomes. Perineural invasion of 5 or more distinct nerves (extensive PNI [ePNI]) was independently associated with local recurrence (subhazard ratio [SHR], 13.83 [95% CI, 3.50-54.62]; P < .001), disease-specific death (SHR, 6.20 [95% CI, 1.59-24.21]; P = .009), and any poor outcome (SHR, 10.21 [95% CI, 2.88-36.15]; P < .001). A revised BWH staging system with substitution of ePNI for large-caliber PNI resulted in improved area under the curve and test characteristics compared with current BWH staging criteria that use nerve caliber as the measure of PNI. The findings of this cohort study suggest that ePNI is the best prognostic measure of PNI. Because ePNI obviated the need for a micrometer and had superior prognostic capacity to nerve caliber in this cohort, ePNI should be considered for inclusion in CSCC tumor staging. Inclusion of ePNI as a high-risk factor in CSCC staging systems may optimize patient selection for primary treatment and adjuvant interventions.

Which Components of the Simple Shoulder Test Show Improvement After Scapulothoracic Fusion for Recalcitrant Scapular Winging? Clinical Results at a Minimum of 5 Years of Follow-up.

Scapular winging is an uncommon but important cause of shoulder pain and disability. Surgical management can include soft tissue procedures such as split pectoralis major transfer, the Eden-Lange procedure, or a triple tendon transfer. When these procedures do not alleviate symptomatic winging or are inappropriate, scapulothoracic fusion is an alternative, but data on its longer-term effectiveness are sparse. (1) What changes in outcome scores were observed (VAS, Single Assessment Numeric Evaluation [SANE], and Simple Shoulder Test [SST] scores), and what proportion of the patients improved by an amount larger than the minimum clinically important difference (MCID) for the outcome tool in question? (2) Which components of the SST are patients able to perform at a minimum of 5 years? (3) What complications occurred after surgery? We performed a retrospective study in a single, large, urban referral medical center of patients who underwent scapulothoracic fusion. Between January 2011 and November 2016, 15 patients underwent scapulothoracic fusion to treat symptomatic scapular winging. Only patients with nondystrophic etiology were included in the analysis (n = 13). Of the 13 remaining patients, one patient was lost to follow-up and another patient died during data collection, leaving 11 patients for the final analysis. Six patients had brachial plexus injuries affecting multiple nerve roots and periscapular muscles, and five had persistent symptoms despite prior tendon transfer. The median age of the patients was 43 years (range 20 to 67 years), and there were six male and five female patients. All patients had a minimum of 5 years of follow-up. There was a median follow-up of 79 months (range 61 to 128 months). The VAS pain score (range 0 to 10, higher scores represent more pain; MCID = 2), SST score (range 0 to 12, higher scores represent less pain and better shoulder function; MCID = 2.3), and SANE score (range 0 to 100, higher scores represent better shoulder function; MCID = 28) were recorded before surgery and at the most recent follow-up. We compared scores from before surgery with those taken at the most recent follow-up and ascertained the proportion of patients whose improvement exceeded the MCID. The number of patients achieving fusion (as confirmed by a CT image), complications, and reoperations was recorded via a record review as well as direct patient query by telephone. The median VAS pain score improved from 7 (range 3 to 10) preoperatively to 3 (range 2 to 5) at the latest follow-up (p < 0.001). The median SANE score improved from 30 (range 0 to 60) preoperatively to 65 (range 40 to 85) at the latest follow-up (p < 0.001). The median SST score improved from 0 (range 0 to 9) to 8 (range 5 to 10) at the latest follow-up (p < 0.001). Ten of 11 patients had improvements exceeding the MCID for VAS, six of 11 had improvements for SANE scores, and nine of 11 had improvements for SST. Preoperative to postoperative improvements in these components of the SST were seen (responses of "yes"): comfort at rest (three of 11 improved to 11 of 11; p < 0.001), sleep comfortably (three of 11 improved to 11 of 11; p < 0.001), place coin on shelf (two of 11 improved to 10 of 11; p < 0.001), lift 1 pound above shoulder (two of 11 improved to eight of 11; p = 0.03), and carry 20 pounds with the arm at side (one of 11 improved to nine of 11; p < 0.001). All 11 patients had successful fusion noted on CT images. There were three complications (progression of glenohumeral arthritis, broken wires, and perioperative chest tube placement) and one reoperation for progression of glenohumeral arthritis with subsequent total shoulder arthroplasty. Patients with recalcitrant symptomatic scapular winging often undergo an exhaustive course of clinical examinations, diagnostic tests, physical therapy, and multiple surgical procedures. Those with brachial plexus palsy with involvement of multiple nerves may continue to have symptoms despite nonoperative management and subsequent soft tissue tendon transfers. Scapulothoracic fusion could be considered for patients with persistent pain and decreased function because of recalcitrant scapular winging who are either not candidates for the procedure or have persistent symptoms despite prior soft tissue procedures. Level IV, therapeutic study.

Serum lipocalin-2 levels are decreased in patients with leprosy.

Background Leprosy is an infectious disease caused by Mycobacterium leprae affecting the skin, peripheral nerves and mucosae. Lipocalin-2 is a key component of the immune system's antimicrobial defence - it prevents iron uptake by binding and sequestering iron-scavenging siderophores and thus inhibits bacterial growth. Aim We evaluated serum lipocalin-2 levels in leprosy patients and its relationship to the pathogenesis and prognosis of the disease. Materials and methods In this case-control study, serum lipocalin-2 levels were measured by ELISA in 20 patients with leprosy and 20 healthy controls. Results Serum levels of lipocalin-2 were significantly reduced (P < 0.001) in leprosy patients as compared to controls. The levels were significantly higher (P < 0.014) in patients with multibacillary leprosy than in those with paucibacillary leprosy. Although the levels of lipocalin-2 were higher in patients with multiple nerve involvement as compared to those with involvement of 1 or 2 nerves, the results were not statistically significant. Limitation of the study The small sample size and the lack of different ethnic groups in the study were the major limitations of this study. Conclusion The lower lipocalin-2 concentrations in leprosy patients point to the importance of the protective functions of lipocalin-2. The elevated levels of lipocalin-2 observed in leprosy patients with neural involvement may be related to the reported neurodegenerative role of lipocalin-2.

P.216 Two interesting and unique cases of acquired neuropathies in pediatrics

A 2-year-old boy known for an abdominal neuroblastoma was admitted after his fourth cycle of chemotherapy for generalized deterioration and pain when weight bearing. Multiple investigations and medication trials were made without finding the cause or relieving the patient's pain. While in hospital, the pain worsened and he developed gastroparesis, psychomotor regression and respiratory distress. A sensory polyneuropathy was confirmed by NCS, and the diagnosis of anti-Hu syndrome was subsequently made by CSF and serum paraneoplastic antibody panel. He received high-dose steroids, plasmapheresis, rituximab and cyclophosphamide, without clear improvement. After a few weeks, he underwent surgical resection of his neuroblastoma and was started on daratumumab bimonthly, mycophenolate mofetil and monthly IVIG after which he finally mildly improved. In summary, anti-Hu syndrome, while extremely rare in pediatrics, must be included in the differential diagnosis of refractory pain in children with a tumor diagnosis. A 15-year-old boy with no previous health issue presented with a subacute onset paraparesis. He had been recently diagnosed with diabetes of unknown type after a weight loss of 45 kg and started on insulin. He presented with progressive bilateral leg weakness, and the physical exam showed asymmetric weakness and decreased reflexes in the lower limbs, as well as sensory loss in a distal-to-proximal gradient. Investigations, such as a spine MRI and blood tests, showed no abnormality except for high HbA1c. The EMG and NCS confirmed involvement of multiple nerves and nerve roots with axonal loss. The final diagnosis was radiculo-plexo-neuropathy related to diabetes type II. The patient refused immunotherapy, and then completely recovered when his treatment was adjusted for diabetes type II.

A hospital-based cross-sectional study of clinico-epidemiological aspects of childhood leprosy with disability in the post-elimination era

Background Childhood leprosy is an important indicator of active foci of transmission in the community in the post-elimination era. Childhood leprosy cases with disabilities are still prevalent, but are often undertreated or misdiagnosed due to diminishing awareness and the available studies on the same are limited. Aims and objectives To study the clinical presentation, WHO grading, duration, relation with lepra reaction, smear positivity and neurological correlation of childhood leprosy cases with disability. Materials and methods A cross-sectional study was conducted in the dermatology department of a tertiary care center including all recent cases of childhood leprosy (less than or equal to 13 years) with WHO grade 1 and grade 2 disability from October 2018 to June 2020. Results Disability among childhood leprosy cases was detected in 18 (32.1%) out of 56 children. The most common type of disability observed was visible muscle wasting (26.7%). Based on WHO disability grading, 3 children (16.6%) had grade 1 disability and 15 children (83.4%) had grade 2 disability. Children with borderline tuberculoid lesions had maximum disability (17.8%). All had thickened peripheral nerves with increased incidence of lepra-reaction (27.7%) and slit skin smear positivity (38.8%). None of the children with disability had a second dose of BCG or rifampicin prophylaxis, even if leprosy cases were reported in the family earlier. Conclusion Development of deformity was dependent on various factors including increasing age, delay in accessing health care, multiple skin lesions, multibacillary disease, smear positivity, multiple nerve involvement, and lepra reaction at the time of presentation. Active school surveys and campaigns are needed for early detection of leprosy and prevention of disability, with appropriate distribution of post-exposure prophylaxis.

Redefining Perineural Invasion in Head and Neck Cutaneous Squamous Cell Carcinoma.

Perineural invasion (PNI) negatively affects disease-specific survival in patients with head and neck cutaneous squamous cell carcinoma (HNcSCC). We aim to analyze the prognostic implications of PNI-related features. Retrospective cohort study. Academic tertiary care hospital. Retrospective chart review was performed on 104 patients diagnosed with HNcSCC between January 2011 and October 2019 who underwent resection, parotidectomy, and neck dissection with more than 1 year of follow-up. PNI was classified as incidental (identified on histopathology alone) or clinical (present on radiography and/or physical exam). Primary outcome measures were overall survival and disease-free survival (DFS). Kaplan-Meier analysis, logistic regression, and Cox regression were performed. The overall 5-year DFS was 57.9%. Sixty-one patients had PNI. On histopathology, 28 lesions showed complete nerve encirclement, 10 involved >5 nerves, and 12 involved named nerves. Patients with facial weakness (P = .026) and positive margins (P = .0029) had a higher likelihood of histopathologic PNI, and positive margins retained significance on multivariable analysis (P = .0079). Worse DFS was seen in patients with PNI (P = .004), advanced tumor stage (P = .049), positive margins (P = .014), and >5 nerves involved (P = .0061). Furthermore, histopathologic PNI was a predictor of DFS (hazard ratio [HR], 3.07; 95% CI, 0.33-1.38; P = .0061) overall and in the clinical PNI cohort (HR, 3.43; 95% CI, 1.65-7.10; P = .00091). DFS was significantly worse in patients with PNI, facial nerve weakness, advanced T stage, positive margins, and multiple nerve involvement. Further characterization of PNI features may help improve prognostic predictions and identify patients who may benefit from more aggressive treatment.

Childhood-onset borderline tuberculoid leprosy with reversal reaction

Background:&nbsp; Leprosy is a chronic granulomatous infection caused by&nbsp; Mycobacterium leprae that predominantly affects the skin and peripheral nerves. Leprosy among children is still common in endemic countries.&nbsp; Case Illustration: A 12-year-old girl complained about a hypopigmented anesthetic patch on her face for 11 years, which became larger and spread slowly to her arms and legs. She had a history of close contact with her aunt, who was diagnosed with multibacillary leprosy. On&nbsp; slit-skin-smear&nbsp; test, acid-fast-bacilli (bacteriologic index +1) were found. She was diagnosed with multibacillary leprosy and treated with children&rsquo;s multidrug therapy-multibacillary (MDT-MB) regimen. After 2 months of MDT-MB treatment, she complained that the hypopigmented patches became reddish and swollen with enlarged peripheral nerves. She underwent a reversal reaction (RR) and was treated with 40 mg prednisone daily and continued the MDT regimen. Discussion:&nbsp; RR is found less frequently in children than the adult. Accurate diagnosis is vital because of its psychosocial impact on the family. One of the most prominent features of borderline tuberculoid leprosy is its susceptibility to RR. It is characterized by rapid changes from existing plaques to edematous lesions with or without abrupt neuritis. Conclusion:&nbsp; We reported a girl with borderline tuberculoid leprosy with developed RR after taking MDT-MB for 2 months. The risk factors for developing RR were being diagnosed with borderline tuberculoid leprosy, female, multiple and disseminated patches involving larger body areas and multiple nerve involvement, large facial patches, and starting treatment. These risk factors were found in our patient. &nbsp; Keywords :&nbsp; borderline tuberculoid, leprosy, reversal reaction

Leprous Neuropathy: Observational Study Highlighting the Role of Electrophysiology in Early Diagnosis.

Background Worldwide leprosy is a common cause of peripheral neuropathy. Electrophysiology is underutilized in its diagnosis. Objective This study aims to evaluate the usefulness of electrophysiological study in the diagnosis of leprous neuropathy. Materials and Methods Clinical and electrophysiological abnormalities of 36 histopathology proven leprosy patients from January 2015 to January 2017 were studied. Statistical Analysis Proportions were compared by Chi-square test. Results Total patients were 36. Thirty-four patients had abnormal electrophysiology and 34 had neurological deficits like weakness, sensory changes, and thickening. By clinical examination, multiple nerve involvement (motor weakness, sensory changes, and nerve thickening) occurred in 29, single nerve in 5, and no nerve involvement in 2. With electrophysiology, multiple nerve involvement (mononeuritis multiplex) was present in 32, single nerve in 2, and normal conduction parameters in 2. From the 36 patients, a total of 1,008 nerves were subjected to clinical examination and 132 were picked up clinically as affected, (13.1%). Electrophysiological study was done in 504 nerves, and 215 were found to be involved, (43%). Nerve abnormality detected by electrophysiology is significantly higher than clinical detection. (Chi-square =164.4054; p = 0.0000). Clinically, the most commonly affected nerve was unar (27) and the least affected was median (2) nerve. Electrophysiology detected 69% of nerves with demyelination and 35% of nerves with axonal features (mosaic pattern). Discussion There was subclinical neuropathy with electroclinical dissociation, as evidenced by more abnormality in electrophysiology than clinical examination. The nerve involvement was mononeuritis or mononeuritis multiplex pattern, both clinically and electrophysiologically. Electrophysiology showed both axonal and demyelinating nerve involvement (mosaic pattern). All the three features are present in leprous neuropathy. In corollary, if a patient has these electrophysiological features, he should be thoroughly investigated for leprosy. Conclusion Triple findings, such as subclinical neuropathy with electroclinical dissociation, mononeuritis multiplex, and mosaic pattern of demyelination and axonopathy, suggest leprous neuropathy

Atypical variants of chronic inflammatory demyelinating polyneuropathy with benign course: a clinical observation for 8 patients without pathogenic therapy

Introduction. Atypical variants of chronic inflammatory demyelinating polyneuropathy are marked by its clinical heterogeneity and variable disease course.Aim of the study. To describe clinical, anamnestic and neurophysiological features of patients with atypical variants of chronic inflammatory demyelinating polyneuropathy, characterized by benign disease course, minimal motor involvement and not required pathogenic therapy.Materials and methods. 8 patients (7 men (87 %) and 1 woman (13 %) at the age of 52–77 years) with atypical variants of chronic inflammatory demyelinating polyneuropathy were analyzed: 5 patients (62.5 %) with asymmetric variant – multifocal acquired demyelinating sensorimotor neuropathy and 3 patients (37.5 %) with sensory variant. All patients were observed at the Research Center of Neurology for the period of 2016– 2019. In each patient the proper clinical and laboratory evaluation was performed along with nerve conduction study and nerve ultrasound.Results. The disease duration at the time of first visit was 1–8 years. By INCAT disability score 3 (37.5 %) patients had 0 points (normal), 3 (37.5 %) patients – 1 point and 1 patient had 2 (25 %) points. Nerve conduction study showed multifocal, asymmetric demyelinating changes in motor nerves. For the whole period of observation all patients were stable, so no one required pathogenic therapy.Conclusion. Chronic inflammatory demyelinating polyneuropathy is a clinically heterogeneous disorder, required clinical suspicion in all patients over 50 years with features of multiple nerve involvement; nerve conduction study helps to detect typical changes, including subclinical ones. The primary strategy of management typical and atypical disease variants in stable course and minimal symptoms is a case follow-up with precise assessment of advantages and disadvantages of pathogenic therapy.

Lipomatosis of Nerve: More than a Focal Distal Disease.

Lipomatosis of nerve is a rare, ill-defined, fibrofatty tumor intermingled among nerve fascicles. The classic presentation of lipomatosis of nerve describes focal distal involvement of the median nerve at the wrist. The purpose of this study was to determine the level of proximal lesion extension in cases of upper extremity lipomatosis of nerve using magnetic resonance imaging. The authors' study confirms that lipomatosis of nerve is more than a focal distal disease. Lipomatosis of nerve has the potential for multiple nerve involvement and for proximal nerve involvement extending to the nerve roots. Magnetic resonance imaging of the entire upper extremity may be indicated in patients with lipomatosis of nerve, especially when the ulnar nerve is affected.

Involvement of cranial nerves in a patient with secondary central nervous system lymphoma.

Neurolymphomatosis (NL) is a rare disease that can be caused by T cell, B cell or natural killer cell (NK) lymphomas, but most commonly by B cell lymphomas.1 NL is characterized by direct infiltration of the central nervous system (CNS), nerve roots/plexus or peripheral nerves by a hematological malignancy.2–4 Multiple nerve involvement is more common than single nerve involvement.1 Secondary NL occurs as relapse or the progression of lymphomas.5

Peripheral Nervous System Involvement in Diabetes and Role of Rehabilitation

Diabetes is well known to affect peripheral nervous system. We have summarized the multiple different presentations of peripheral nervous system involvement in this disease along with the medical and rehabilitation management. The clinical presentation depends on the localization of the damage which can involve nerve roots, plexus, focal nerves, diffuse involvement of multiple nerves and autonomic system. The process can be acute or insidious based on the pathology which can be variable.

Childhood leprosy in India over the past two decades

Clinico-epidemiological pattern of childhood leprosy in India over the past two decades were analysed from the Indian studies conducted during the years 1990-2009. Twelve studies on childhood leprosy were included. Ten were conducted in health institutions and one was a community-based survey. Voluntary reporting was the principal method of case detection; community survey was adopted in two studies. Occurrence of childhood leprosy in tertiary care hospitals varied from 5.1-11.43%, in one urban clinic and the three leprosy referral hospitals it was 9.81-31.3% and peripheral surveys recorded 7.06-35.5% cases. History of familial contact was present in 0.66-47% cases. Borderline tuberculoid was the commonest clinical type, majority with single lesion. Other types were indeterminate (3.48-10.1%), borderline lepromatous (1.9-19.4%), lepromatous (0.1 to 9.38%), and pure neuritic (3.48-10.1%). Single peripheral nerve trunk was involved in 13.63-40-62% cases and multiple nerve involvement was recorded in 4.54-59.38% cases. The majority of cases were paucibacillary (43.28-98%). Multibacillary (MB) cases ranged from 2-56.6%. Slit-skin smear positive cases ranged from 5.42-25%. Lepra reactions occurred in 0-29.7% cases. Relapse rate varied between 1.16-7.1%. Deformity occurred in 0-24% cases. Multibacillary cases were common among Indian children, some of whom were smear positive. Probably these cases were the source of many new cases. Pure neuritic leprosy was frequent among Indian children, so also the lepra reactions and deformities. The presence of familial and extra-familial contact with leprosy cases may be a cause of concern, as it implies continuing transmission of the disease.

Hourglass-Like Constrictions of Peripheral Nerve in the Upper Extremity

The development of an hourglass-like constriction in the nerve is rare, and its origin is unknown. Its clinical manifestations are not well documented, and the treatment protocol has not been established. To identify the cause, presentation, and possible treatment for patients with nerve palsies secondary to an hourglass-like constriction in the affected nerves. Patients presenting with peripheral nerve palsy caused by an hourglass-like constriction of nerves were retrospectively investigated in 2 hand centers. The patients' presentation and neurological findings were reviewed, and the immunohistochemistry of excised specimens was studied. Forty-two patients who presented with 47 nerve palsies were examined. Forty-one patients experienced a sudden onset of pain in the upper limb, followed by flaccid paralysis in the affected muscles. Ten patients had multiple nerve involvement. Surgical exploration found 1 or more hourglass-like constrictions in the nerve. The treatments included internal neurolysis, neurorrhaphy, and nerve grafting. Thirty-one of 42 patients (36 nerves) were followed up for a mean of 48 months (range, 8-157 months). Fifteen of 16 nerves treated by neurolysis, 10 of 13 nerves treated by neurorrhaphy, and 4 of 7 nerves treated by nerve grafting had good recovery. CD8-positive T-lymphocyte infiltration was observed in all the excised specimens. The clinical presentation of patients with hourglass-like constrictions in their nerves is similar to that of patients with neuralgic amyotrophy. Histochemical analysis suggests that the pathogenesis may be immunological in origin. The role of surgery in this condition is uncertain.

Outcomes of Primary Cutaneous Squamous Cell Carcinoma With Perineural Invasion

To identify factors associated with poor outcomes in perineurally invasive squamous cell carcinoma. Retrospective cohort study. Two academic hospitals in Boston, Massachusetts. Adults with perineural SCC diagnosed from 1998 to 2008. Hazard ratios (HRs) for local recurrence, nodal metastasis, death from disease, and overall death, adjusted for known prognostic factors. A total of 114 cases were included, all but 2 involving unnamed nerves. Only a single local recurrence occurred in cases with no risk factors other than nerve invasion. Tumors with large nerve (≥ 0.1 mm in caliber) invasion were significantly more likely to have other risk factors, including diameters of 2 cm or greater (P<.001), invasion beyond the subcutaneous fat (P<.003), multiple nerve involvement (P<.001), infiltrative growth (P=.01), or lymphovascular invasion (P=.01). On univariate analysis, large nerve invasion was associated with increased risk of nodal metastasis (HR, 5.6 [95% CI, 1.1-27.9]) and death from disease (HR, 4.5 [95% CI, 1.2-17.0]). On multivariate analysis, tumor diameter of 2 cm or greater predicted local recurrence (HR, 4.8 [95% CI, 1.8-12.7]), >1 risk factor predicted nodal metastasis (2 factors: HR, 4.1 [95% CI, 1.0-16.6]), lymphovascular invasion predicted death from disease (HR, 15.3 [95% CI, 3.7-62.8]), and overall death (HR, 1.1 [95% CI, 1.0-1.1]). Invasion beyond subcutaneous fat also predicted overall death (HR, 2.1 [95% CI, 1.0-4.3]). Squamous cell carcinoma involving unnamed small nerves (<0.1 mm in caliber) may have a low risk of poor outcomes in the absence of other risk factors. Large-caliber nerve invasion is associated with an elevated risk of nodal metastasis and death, but this is due in part to multiple other risk factors associated with large-caliber nerve invasion. A larger study is needed to estimate the specific prognostic impact of nerve caliber.

Clinical Reasoning: An unusual lung mass causing focal weakness

A 30-year-old right-handed man presented to the emergency room with right forearm numbness and elbow pain for 7 months. For the past 4 weeks he could not write or hold anything with his right hand. His primary care physician referred him to the hospital for further workup after noticing thenar atrophy in that hand. He also described decreased sensation over the ventromedial aspect of his right forearm. He denied any neck pain, changes in urinary or bowel habits, or other neurologic complaints. Initial inspection showed significant atrophy of the thenar muscles. Neurologic examination showed mild weakness in the right arm and wrist extension and in the abductor pollicis brevis, all interossei (including the first dorsal interosseus), lumbricals I–IV, and the abductor digiti minimi of the right hand. He had diminished pinprick and temperature sensation over the ventromedial aspect of his right forearm, from the elbow to the fourth and fifth digits, without splitting of the fourth digit. The right biceps reflex was normal, the brachioradialis was diminished, and the triceps reflex could not be elicited. The remainder of the neurologic examination, including mental status, cranial nerves, coordination, and gait, was normal. There was no Horner syndrome on the right. His general physical examination demonstrated a well-built man with no skin lesions, edema, or protrusions. The muscles involved and the sensory distribution implied involvement of multiple nerves, possibly by compression of the lower trunk of the brachial plexus. Subsequently, a chest X-ray was performed by the emergency room physician to screen for a mass lesion compressing the brachial plexus. The X-ray showed a large mass in the apex of the right lung (figure 1). Figure 1 Unusual finding on chest X-ray Chest x-ray shows a round circular mass (large arrow) in the apex of the right lung. ### Questions for consideration: 1. What are the possible etiologies of the mass? 2. What would …