Focal Nerve Research Articles

Deep and superficial sensory nerve conduction to the first dorsal web space of the foot

BackgroundPolyneuropathy tends to progress in a distal to proximal fashion beginning at the tips of the toes. Sensory nerve conduction testing in the distal foot may identify early polyneuropathic changes. Deep fibular (DFN) sensory conduction techniques have been described to assess the distal dorsal foot, however, there has been no accommodation for reported anatomic communication between the DFN and superficial fibular nerve (SFN) in the dorsal foot. This study aims to stimulate both the DFN and SFN at the ankle to evaluate the function of the observed anatomic sensory nerve variations in the first dorsal web space. Normal nerve conduction values will also be presented in a sample of young adults and sex and BMI effects will be evaluated. Materials and methodsThe study participants included 201limbs tested in 101 subjects (31 males and 70 females) between the ages of 18 and 47 (mean age 24.6 ± 4.8 years). The DFN and intermediate branch of the SFN (IDCN) were stimulated at the ankle 12 cm proximal to a surface recording electrode over the first dorsal web space of the foot. The obtained sensory nerve amplitude and latency values were recorded and velocity values were calculated. ResultsThe current study identified a first dorsal web space response in 78% of limbs tested. Web space responses were variable in origin with SFN only, DFN only and both SFN and DFN recorded responses in the web space. The mean DFN peak to trough amplitude was 5.8 ± 2.3 μV, while the peak velocity was 31.2 ± 2.9 m/s. The mean SFN-IDCN peak to trough amplitude was 5.9 ± 2.3 μV and the mean peak velocity was 32.1 ± 3.8 m/s. There was a statistically significant sex effect for SFN-IDCN onset latency and velocity with women demonstrating shorter onset latencies and faster velocities than the men. There was a significant SFN-IDCN BMI effect for onset latency and velocity, such that those with low BMI had shorter latency and faster nerve conduction values. A significant DFN sex effect was also identified for amplitude with males and females demonstrating a mean amplitude of 5.4 μV and 6.0 μV, respectively. ConclusionsThere is a high degree of anatomic and functional variability in sensory supply to the first dorsal web space of the foot requiring exploration of the DFN and SFN when assessing focal nerve entrapments or generalized polyneuropathy. A general dorsal foot sensory velocity and amplitude value may serve as an index for evaluation of early polyneuropathy.

Diagnostic controversies in recurrent painful ophthalmoplegic neuropathy: single case report with a systematic review

BackgroundOphthalmoplegic migraine, renamed "Recurrent Painful Ophthalmoplegic Neuropathy" (RPON) in 2013 by the International Headache Society is a rare neurologic disorder characterized by recurrent attacks of ophthalmoplegia associated to ipsilateral headache. The etiology is still unknown. Typical magnetic resonance imaging findings show a focal nerve thickening and contrast enhancement. In the majority of cases, there is a full recovery within days or weeks. There is no evidence supporting a specific treatment. The review defines the characteristics of the recurrent painful ophthalmoplegic neuropathy in patients within 2 years of age underlying the importance of the role of magnetic resonance imaging even in presence of the first attack. Thus, an emblematic case report is presented.Case presentationThe authors present a case of third cranial nerve paresis in a 17-month-old male child, presenting a neuroradiological pattern highly suggestive of schwannoma, aneurism or recurrent painful ophthalmoplegic neuropathy. Thus, a review of the literature with the pediatric casuistry of recurrent painful ophthalmoplegic neuropathy occurred within 2 years of age focusing on diagnostic considerations is presented. The authors highlight the importance to consider recurrent painful ophthalmoplegic neuropathy in presence of magnetic resonance imaging findings and clinical symptoms referable to aneurysm or schwannoma. Thus, the review defines the characteristics and the neuroradiological findings at the first RPON attack occurred under 2 years of age.ConclusionAlthough two attacks are necessary, the review strongly suggests to consider recurrent painful ophthalmoplegic neuropathy even at the first attack, in presence of described characteristics and the aforementioned magnetic resonance imaging findings.

Technical Update on MR Neurography.

Imaging evaluation of peripheral nerves (PNs) is challenging. Magnetic resonance imaging (MRI) and ultrasonography are the modalities of choice in the imaging assessment of PNs. Both conventional MRI pulse sequences and advanced techniques have important roles. Routine MR sequences are the workhorse, with the main goal to provide superb anatomical definition and identify focal or diffuse nerve T2 signal abnormalities. Selective techniques, such as three-dimensional (3D) cranial nerve imaging (CRANI) or 3D NerveVIEW, allow for a more detailed evaluation of normal and pathologic states. These conventional pulse sequences have a limited role in the comprehensive assessment of pathophysiologic and ultrastructural abnormalities of PNs. Advanced functional MR neurography sequences, such as diffusion tensor imaging tractography or T2 mapping, provide useful and robust quantitative parameters that can be useful in the assessment of PNs on a microscopic level. This article offers an overview of various technical parameters, pulse sequences, and protocols available in the imaging of PNs and provides tips on avoiding potential pitfalls.

Signs Indicative of Central Sensitization Are Present but Not Associated with the Central Sensitization Inventory in Patients with Focal Nerve Injury.

Objective: Carpal tunnel syndrome (CTS) is the most common focal nerve injury. People with CTS may show alterations in central processing of nociceptive information. It remains unclear whether the central sensitization inventory (CSI) is capable of detecting such altered central pain processing. Methods: Thirty healthy volunteers were matched with 30 people with unilateral CTS from the orthopaedic waitlist. Changes to central pain processing were established through psychophysical sensory testing (bilateral pressure pain thresholds (PPT), conditioned pain modulation, temporal summation) and pain distribution on body charts. Patients also completed pain severity and function questionnaires, psychological questionnaires and the CSI. Results: Compared to healthy volunteers, patients with CTS have lower PPTs over the carpal tunnel bilaterally (t = −4.06, p < 0.0001 ipsilateral and t = −4.58, p < 0.0001 contralateral) and reduced conditioned pain modulation efficacy (t = −7.31, p <0.0001) but no differences in temporal summation (t = 0.52, p = 0.60). The CSI was not associated with psychophysical measures or pain distributions indicative of altered central pain processing. However, there was a correlation of the CSI with the Beck Depression Inventory (r = 0.426; p = 0.019). Conclusion: Patients with CTS show signs of altered central pain mechanisms. The CSI seems unsuitable to detect changes in central pain processing but is rather associated with psychological factors in people with focal nerve injuries.

Focused Neuromuscular Ultrasound Approach for the Diagnosis of Chronic Inflammatory Demyelinating Polyneuropathy.

Previous ultrasonographic studies of individuals with chronic inflammatory demyelinating polyneuropathy (CIDP) have shown nerve enlargement at several sites. This prospective study compares only the bilateral median and ulnar nerves of individuals with CIDP with reference values to determine the clinical usefulness of this focused approach as a diagnostic tool. The cross-sectional area, echogenicity, and vascularity of the bilateral median and ulnar nerves of 25 subjects with CIDP were measured using ultrasound. Nineteen had typical CIDP based on the European Federation of Neurological Societies and the Peripheral Nerve Society guidelines, whereas six had atypical CIDP and were diagnosed based on clinical impression. Focal nerve enlargement was found in at least one segment in all subjects. Subjects with typical CIDP had larger cross-sectional areas compared with subjects with atypical CIDP. A focused ultrasound study, involving only the median and ulnar nerves, is sensitive for the detection of nerve enlargement in CIDP. Measuring the cross-sectional area of the median and ulnar nerves is clinically feasible and may help establish the diagnosis of CIDP.

Paraneoplastic Neuropathies: What's New Since the 2004 Recommended Diagnostic Criteria.

The diagnostic criteria published by the PNS (Paraneoplastic Neurological Syndromes) Euronetwork in 2004 provided a useful classification of PNS, including paraneoplastic neuropathies. Subacute sensory neuronopathy (SSN) was the most frequently observed peripheral PNS, whereas other forms of neuropathy, as sensory polyneuropathy, sensorimotor polyneuropathy, demyelinating neuropathies, autonomic neuropathies, and focal nerve or plexus lesions, were less frequent. At the time of publication, the main focus was on onconeural antibodies, but knowledge regarding the mechanisms has since expanded. The antibodies associated with PNS are commonly classified as onconeural (intracellular) and neuronal surface antibodies (NSAbs). Since 2004, the number of antibodies and the associated tumors has increased. Knowledge has grown on the mechanisms underlying the neuropathies observed in lymphoma, paraproteinemia, and multiple myeloma. Moreover, other unrevealed mechanisms underpin sensorimotor neuropathies and late-stage neuropathies, where patients in advanced stages of cancer—often associated with weight loss—experience some mild sensorimotor neuropathy, without concomitant use of neurotoxic drugs. The spectrum of paraneoplastic neuropathies has increased to encompass motor neuropathies, small fiber neuropathies, and autonomic and nerve hyperexcitability syndromes. In addition, also focal neuropathies, as cranial nerves, plexopathies, and mononeuropathies, are considered in some cases to be of paraneoplastic origin. A key differential diagnosis for paraneoplastic neuropathy, during the course of cancer disease (the rare occurrence of a PNS), is chemotherapy-induced peripheral neuropathy (CIPN). Today, novel complications that also involve the peripheral nervous system are emerging from novel anti-cancer therapies, as targeted and immune checkpoint inhibitor (ICH) treatment. Therapeutic options are categorized into causal and symptomatic. Causal treatments anecdotally mention tumor removal. Immunomodulation is sometimes performed for immune-mediated conditions but is still far from constituting evidence. Symptomatic treatment must always be considered, consisting of both drug therapy (e.g., pain) and attempts to treat disability and neuropathic pain.

Management of Recalcitrant Cubital Tunnel Syndrome.

Cubital tunnel syndrome is a common upper extremity compressive neuropathy. Recalcitrant cubital tunnel syndrome poses diagnostic and treatment challenges. Potential etiologies of persistent or recurrent symptoms after surgical treatment include an inaccurate preoperative diagnosis, incomplete nerve decompression, iatrogenic injury, postsurgical perineural adhesions, irreversible nerve pathology, or conditions associated with secondary nerve compression. Confirmation of recalcitrant ulnar nerve pathology relies on a thorough history to consider symptoms and chronology, careful examination to quantify nerve function and to assess for focal nerve provocation, and objective testing to highlight a possible nerve lesion such as ultrasonography and electrodiagnostic testing. Conservative treatment may provide symptomatic relief; however, surgical management such as revision neuroplasty, neurolysis, nerve reconstruction, and/or anterior transposition may be indicated. Optimizing the biology of the local nerve environment is critical. No surgical treatment procedure has shown superiority over another; however, individualized treatment is emphasized to improve symptoms and maximize nerve recovery potential.

Focal Structure-Function Relationships in Primary Open-Angle Glaucoma Using OCT and OCT-A Measurements.

PurposeTo evaluate the focal structure–function associations among visual field (VF) loss, optical coherence tomography angiography (OCT-A) vascular measurements, and optical coherence tomography (OCT) structural measurements in glaucoma.MethodsIn this cross-sectional study, subjects underwent standard automated perimetry, OCT-based nerve fiber thickness measurements, and OCT-A imaging. Mappings of focal VF test locations with OCT and OCT-A measurements were defined using anatomically adjusted nerve fiber trajectories and were studied using multivariate mixed-effects analysis. Segmented regression analysis was used to determine the presence of breakpoints in the structure–function associations.ResultsThe study included 119 eyes from 86 Chinese subjects with primary open-angle glaucoma (POAG). VF mean deviation was significantly associated with global capillary perfusion density (β = 0.13 ± 0.08) and global retinal nerve fiber layer thickness (β = 0.09 ± 0.02). Focal capillary density (FCD) was significantly associated with VF losses at 34 VF test locations (66.7% of 24-2 VF), with 24 of the 34 locations being within 20° of retinal eccentricity. Focal nerve layer (FNL) thickness was significantly associated with 16 VF test locations (31.4% of 24-2 VF; eight locations within 20° eccentricity). For VF test locations in the central 10° VF, VF losses below the breakpoint were significantly associated with FCD (slope, 0.89 ± 0.12, P < 0.001), but not with FNL thickness (slope, 0.57 ± 0.39, P = 0.15).ConclusionsFocal capillary densities were significantly associated with a wider range of visual field losses and in a larger proportion of the visual field compared to nerve fiber thickness.

Nerve Ultrasound in Traumatic and Iatrogenic Peripheral Nerve Injury.

Peripheral nerve injury is a potentially debilitating disorder that occurs in an estimated 2–3% of all patients with major trauma, in a similar percentage of medical procedures. The workup of these injuries has traditionally been clinical, combined with electrodiagnostic testing. However, this has limitations, especially in the acute phase of the trauma or lack of any recovery, when it is very important to determine nerve continuity and perform surgical exploration and repair in the case of the complete transection or intraneural fibrosis. Ultrasound can help in those situations. It is a versatile imaging technique with a high sensitivity of 93% for detecting focal nerve lesions. Ultrasound can assess the structural integrity of the nerve, neuroma formation and other surrounding abnormalities of bone or foreign bodies impeding the nerve. In addition, this can help to prevent iatrogenic nerve injury by marking the nerve before the procedure. This narrative review gives an overview of why and how nerve ultrasound can play a role in the detection, management and prevention of peripheral nerve injury.

Brachial Plexus Neuropathies During the COVID-19 Pandemic: A Retrospective Case Series of 15 Patients in Critical Care.

ObjectiveThe utilization of the prone position to treat patients with COVID-19 pneumonia who are critically ill and mechanically ventilated is well documented. This case series reports the location, severity, and prevalence of focal peripheral nerve injuries involving the upper limb identified in an acute COVID-19 rehabilitation setting. The purpose of this study was to report observations and to explore the challenges in assessing these patients.MethodsParticipants were patients with suspected peripheral nerve injuries following discharge from COVID-19 critical care who were referred to the peripheral nerve injury multidisciplinary team. The patients studied had suspected peripheral nerve injuries following discharge from COVID-19 critical care and were referred to the peripheral nerve injury multidisciplinary team. Data were collected retrospectively on what peripheral neuropathies were observed, with reference to relevant investigation findings and proning history.ResultsDuring the first wave of the COVID-19 pandemic in the United Kingdom, 256 patients were admitted to COVID-19 critical care of Queen Elizabeth Hospital, Birmingham, United Kingdom. From March to June 2020, a total of 114 patients required prone ventilation. In this subgroup, a total of 15 patients were identified with clinical findings of peripheral nerve injuries within the upper limb. In total, 30 anatomical nerve injuries were recorded. The most commonly affected nerve was the ulnar nerve (12/30) followed by the cords of the brachial plexus (10/30). Neuropathic pain and muscle wasting were identified, signifying a high-grade nerve injury.ConclusionPeripheral nerve injuries may be associated with prone positioning on intensive care units, although other mechanisms, such as those of a neuroinflammatory nature, cannot be excluded.ImpactProning-related upper limb peripheral nerve injuries are not discussed widely in the literature and may be an area of further consideration when critical care units review their proning protocols. Physical therapists treating these patients play a key part in the management of this group of patients by optimizing the positioning of patients during proning, making early identification of peripheral nerve injuries, providing rehabilitation interventions, and referring to specialist services if necessary.

Utility of nerve ultrasound in ulnar neuropathy with abnormal non-localizing electrophysiology in diabetes mellitus

We studied the utility of ultrasound in the diagnostic workup of ulnar neuropathy with abnormal non-localizing electrophysiology (NL-UN) in patients with diabetes. Eighteen ulnar nerves (15 patients) were scanned from wrist to mid-upper arm. Ultrasound showed: (a) focal nerve enlargement at the elbow (8/18 nerves), either alone (6) or superimposed upon diffuse nerve abnormality (2); (b) diffuse nerve enlargement without focal abnormality (8/18); (c) segmental abnormality in upper-arm or forearm without extrinsic nerve compression (2/18). This study shows a pivotal role for ultrasound in the classification of NL-UN in patients with diabetes, which can facilitate critical therapeutic decisions.

Optimizing telehealth pain care after COVID-19.

Optimizing telehealth pain care after COVID-19.

Bilateral Suprascapular Nerve Injury and Novel Technique of Nerve Transfers for Restoration of External Rotation: Case Report

Complex brachial plexus and peripheral nerve injuries can be challenging to evaluate and treat, especially in the face of novel pathology. To describe the diagnosis of bilateral suprascapular nerve injury, the unique surgical anatomy, and the novel use of a distal nerve transfer to re-establish external rotation. To the best of our knowledge, bilateral suprascapular nerve injury has not previously been reported. The authors describe the physical examination and electrodiagnostic and imaging work-up for elucidating complex injury patterns and present a procedure for reinnervating the infraspinatus muscle. A 48-yr-old man presented with profound, right-sided, partial brachial plexus injury incurred 5 mo earlier in a motorcycle accident. Imaging demonstrated atrophy and severe edema within the bilateral supraspinatus and infraspinatus muscles. Needle electromyography depicted no motor units and insertional activities in bilateral infraspinatus muscles, consistent with bilateral suprascapular nerve injury. Along with brachial plexus repair, the patient underwent transfer of a distal branch of the spinal accessory nerve to the inferolateral branch of the suprascapular nerve to the infraspinatus muscle. At 16 mo, he had recovered strong restoration of shoulder external rotation. Systematic evaluation can assist in defining complex pathology, particularly with previously unreported types. In this case, predominant injury was inflicted upon the branch innervating the infraspinatus. Highly focal nerve transfers can achieve substantial restoration of external rotation.

Canine cauda equina neuritis secondary to neospora

A five-year-old, female spayed, crossbreed dog was presented for lethargy, fever, hyporexia and right hindlimb lameness of two weeks’ duration. Examination revealed an intermittent weightbearing right hindlimb lameness with delayed...

Low incidence of vascular uptake during ganglion impar sympathetic nerve blocks for coccydynia.

Context:Focal sympathetic nerve blocks of the ganglion impar are often effective treatments for coccydynia (coccyx pain) and other pelvic pain syndromes. These injections are generally performed under contrast-enhanced fluoroscopic guidance. Vascular uptake may potentially occur during the injection and vascular uptake rates have been reported for other spinal injections, but never for ganglion impar blocks. Aims:The purpose of the study was to determine vascular uptake rates during fluoroscopy-guided ganglion impar blocks. Settings and Design:An academic/University-based Coccyx Pain Center. Methods and Materials:A total of 78 consecutive trans-coccygeal ganglion impar blocks were analyzed for vascular uptake of contrast as determined by intermittent fluoroscopy. Statistical Analysis Used:Direct calculation of incidence. Results:Only one patient (1.3%) demonstrated a vascular uptake pattern, which was readily recognized and corrected by slightly adjusting the position of the needle tip and thereby subsequently obtaining the desired contrast pattern at the ganglion impar. Conclusions:Vascular uptake incidence is low during ganglion impar blocks. This information can be one of the multiple factors considered when a physician is deciding whether or not to use contrast in an individual patient.

An Unusual Cause of Intracerebral Hemorrhage: Clinical Pearls Regarding Primary Angiitis of the Central Nervous System.

Primary angiitis of the central nervous system (PACNS) is a rare form of vasculitis. It is a diagnosis of exclusion and often diagnosed post mortem on pathologic evaluation. Cerebral angiography can be suggestive, but biopsy is required. Symptoms can vary from headache to focal cranial nerve deficits. On the more severe spectrum, patients can present with ischemic and vary rarely hemorrhagic stroke. We present in this case report key clinical pearls regarding suspected diagnosis. Younger patients with cortical hemorrhages may have PACNS instead of the more common cerebral amyloid angiopathy. Early suspicion may aid in initiating effective treatment as we highlight in the discussion.

Interocular asymmetry of the superonasal retinal nerve fibre layer thickness and blood vessel diameter in healthy subjects.

Optical coherence tomography is commonly used to measure the retinal nerve fibre layer thickness in both normal and diseased eyes; however, variation among normal eyes is common and may limit the usefulness of the results. The aim of this study was to explore the interocular asymmetries in retinal nerve fibre layer thickness in a group of normal eyes and to investigate the influence of blood vessel diameter on local retinal nerve fibre layer thickness. In this prospective study, retinal nerve fibre layer thickness and blood vessel diameter across 100 healthy participants were measured using two optical coherence tomography instruments. Individuals were categorised into two groups based on the presence or absence of interocular retinal nerve fibre layer thickness asymmetry beyond the 75th percentile of all participants. The superonasal sectoral retinal nerve fibre layer thickness was significantly greater in the left eye compared to the right, across all three sectors. Mean blood vessel diameter showed a corresponding difference in thickness at one of the superonasal sectors. Linear regression showed a positive and moderate correlation between blood vessel diameter and focal retinal nerve fibre layer thickness. This trend persisted across both arteries and veins, but veins showed larger variability between left and right eye in participants with marked superonasal retinal nerve fibre layer asymmetry. Retinal nerve fibre layer thickness and blood vessel diameter vary significantly between eyes even in healthy individuals. These asymmetries in a normal population should be taken into consideration when interpreting the retinal nerve fibre layer thickness measurements from optical coherence tomography to assist in distinguishing normal variations from disease.

Radiomics of peripheral nerves MRI in mild carpal and cubital tunnel syndrome.

To assess the discriminative power of radiomics of peripheral nerves at 1.5T MRI, using common entrapment neuropathies of the upper limb as a model system of focal nerve injury. Radiomics was retrospectively done on peripheral nerve fascicles on T1-weighted 1.5T MRI of 40 patients with diagnosis of mild carpal (n = 25) and cubital tunnel (n = 15) syndrome and of 200 controls. Z-score normalization and Mann-Whitney U test were used to compare features of normal and pathological peripheral nerves. Receiver operating characteristic analysis was performed. A total of n = 104 radiomics features were computed for each patient and control. Significant differences between normal and pathological median and ulnar nerves were found in n = 23/104 features (p < 0.001). According to features classification, n = 5/23 features were shape-based, n = 7/23 were first-order features, n = 11/23 features were classified as gray level run length matrix. Nine of the selected features showed an AUC higher that 0.7: minimum AUC of 0.74 (95% CI 0.61-0.89) for sum variance and maximum AUC of 0.90 (95% CI 0.82-0.99) for zone entropy. Features analysis demonstrated statistically significant differences between normal and pathological nerve. The results suggested that radiomics analysis could assess the median and ulnar nerve inner structure changes due to the loss of the fascicular pattern, intraneural edema, fibrosis or fascicular alterations in mild carpal tunnel and mild cubital tunnel syndromes even when the nerve cross-sectional area does not change.

Update on classification, epidemiology, clinical phenotype and imaging of the nonsystemic vasculitic neuropathies.

Single-organ vasculitis of the peripheral nervous system (PNS) is often designated nonsystemic vasculitic neuropathy (NSVN). Several variants or subtypes have been distinguished, including migratory sensory neuropathy, postsurgical inflammatory neuropathy, diabetic radiculoplexus neuropathies, skin-nerve vasculitides, and, arguably, neuralgic amyotrophy. NSVN often presents as nondiabetic lumbosacral radiculoplexus neuropathy (LRPN). This review updates classification, clinical features, epidemiology, and imaging of these disorders. A recent study showed the annual incidence of LRPN in Olmstead County, Minnesota to be 4.16/100 000:2.79/100 000 diabetic and 1.27/100 000 nondiabetic. This study was the first to determine the incidence or prevalence of any vasculitic neuropathy. In NSVN, ultrasonography shows multifocal enlargement of proximal and distal nerves. In neuralgic amyotrophy, MRI and ultrasound reveal multifocal enlargements and focal constrictions in nerves derived from the brachial plexus. Histopathology of these chronic lesions shows inflammation and rare vasculitis. Diffusion tensor imaging of tibial nerves in NSVN revealed decreased fractional anisotropy in one study. Single-organ PNS vasculitides are the most common inflammatory neuropathies. Neuralgic amyotrophy might result from PNS vasculitis, but further study is necessary. The usefulness of focal nerve enlargements or constrictions in understanding pathological mechanisms, directing biopsies, and monitoring disease activity in NSVN should be further investigated.

Clinical Evaluation of Melorheostosis in the Context of a Natural History Clinical Study.

ABSTRACTMelorheostosis is a rare dysostosis involving cortical bone overgrowth that affects the appendicular skeleton. Patients present with pain, deformities, contractures, range of motion limitation(s), and limb swelling. It has been described in children as well as adults. We recently identified somatic mosaicism for gain‐of‐function mutations in MAP2K1 in patients with melorheostosis. Despite these advances in genetic understanding, there are no effective therapies or clinical guidelines to help clinicians and patients in disease management. In a study to better characterize the clinical and genetic aspects of the disease, we recruited 30 adults with a radiographic appearance of melorheostosis and corresponding increased uptake on 18F‐NaF positron emission tomography (PET)/CT. Patients underwent physical exam, imaging studies, and laboratory assessment. All patients underwent nerve conduction studies and ultrasound imaging of the nerve in the anatomic distribution of melorheostosis. We found sensory deficits in approximately 77% of patients, with evidence of focal nerve entrapment in five patients. All patients reported pain; 53% of patients had changes in skin overlying the affected bone. No significant laboratory abnormalities were noted. Our findings suggest that patients with melorheostosis may benefit from a multidisciplinary team of dermatologists, neurologists, orthopedic surgeons, pain and palliative care specialists, and physical medicine and rehabilitation specialists. Future studies focused on disease management are needed. © 2019 The Authors. JBMR Plus Published by Wiley Periodicals, Inc. on behalf of American Society for Bone and Mineral Research.