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Open access

The paraneurium and the tumefactive appearance of peripheral nerve neurolymphomatosis: illustrative case

Damiano G Barone, Ryan W Kendziora, Stephen M Broski, David J Schembri Wismayer, and Robert J Spinner

BACKGROUND

Peripheral neurolymphomatosis (NL) is an often-misdiagnosed condition characterized by lymphomatous infiltration within the peripheral nerves. Its rarity and complexity frequently result in delayed diagnosis and suboptimal patient outcomes. This study aims to elucidate the role of the paraneurium (circumneurium) in NL, emphasizing its diagnostic and therapeutic significance.

OBSERVATIONS

A 72-year-old man presented with lesions on his right lower eyelid. Initial diagnostics were inconclusive until an excisional biopsy confirmed extranodal marginal zone lymphoma. Following a complete metabolic response to rituximab treatment, the patient relapsed 14 months later with progressive lymphoma and bilateral sciatic nerve involvement, as confirmed by positron emission tomography–computed tomography and magnetic resonance imaging.

LESSONS

This paper underscores the critical role of the paraneurium in NL, enhancing understanding of its pathophysiology. Integrating advanced imaging techniques have proved essential in accurately identifying neurolymphomatous involvement within the paraneurium. This study paves the way for more effective management strategies in NL and similar conditions, focusing on improving patient care and outcomes.

Open access

Phantom limb pain, traumatic neuroma, or nerve sheath tumor? Illustrative case

Patrick J Halloran, E. Antonio Chiocca, and Andres Santos

BACKGROUND

Phantom limb pain and traumatic neuromas are not commonly seen in neurosurgical practice. These conditions can present with similar symptoms; however, management of traumatic neuroma is often surgical, whereas phantom limb pain is treated with conservative measures.

OBSERVATIONS

A 77-year-old female patient with a long-standing history of an above-the-knee amputation experienced severe pain in her right posterior buttocks area for several years’ duration, attributed to phantom limb pain, which radiated down the stump of her leg and was treated with a variety of conservative measures. A recent exacerbation of her pain led to a prolonged hospitalization with magnetic resonance imaging of her leg stump, revealing a mass in the sciatic notch, at a relative distance from the stump. The anatomical location of the mass on the sciatic nerve in the notch led to a presumed radiological diagnosis of nerve sheath tumor, for which she underwent excision. At surgery, a neuroma of the proximal portion of the transected sciatic nerve that had retracted from the amputated stump to the notch was diagnosed.

LESSONS

Traumatic neuromas of transected major nerves after limb amputation should be considered in the differential diagnosis of phantom limb pain.

Open access

Extracranial arteriovenous malformation mimicking a peripheral nerve tumor: illustrative case

Rahul Rodrigues, M. Harrison Snyder, Wilson Chavez, Knarik Arkun, Ozden Bulut, and Julian Wu

BACKGROUND

Arteriovenous malformations (AVMs) are vascular malformations that are more commonly found intracranially, followed by the head, neck, limbs, and trunk. Extracranially, AVMs can mimic peripheral nerve tumors, leading to misdiagnosis.

OBSERVATIONS

A 19-year-old female, who presented with left lateral lower leg pain, was preoperatively thought to have a peripheral nerve tumor; at surgery, however, she was found to have an extracranial AVM. The distinct margins of the tumor on preoperative magnetic resonance imaging suggested that the patient might have a peripheral nerve tumor; however, the clinical symptoms of focal pain at rest and the absence of Tinel’s sign should have raised questions about this diagnosis.

LESSONS

This case highlights the difficulty in differentiating a peripheral nerve tumor from an extracranial AVM in certain clinical scenarios. It is important to use a multifaceted diagnostic approach to get a correct preoperative diagnosis and plan treatment appropriately.

Open access

Atypical cervical radiculopathy presenting with brachioradial pruritus: illustrative cases

Kareem Khalifeh, Janneinne Le, Basel Musmar, Joseph Maroon, Adam S Kanter, and Burak Ozgur

BACKGROUND

Brachioradial pruritus (BRP) is a rare form of chronic dysesthesia characterized by persistent itching, burning, or tingling commonly involving the dorsolateral upper extremities. Although the exact pathophysiology remains unclear, the condition may be a manifestation of atypical cervical radiculopathy.

OBSERVATIONS

The authors describe two patients with BRP, a 44-year-old female and a 51-year-old male, both of whom benefited from surgical intervention for atypical cervical radiculopathy, while also highlighting their presentation, diagnostic testing, treatment, and outcomes.

LESSONS

The cases demonstrate the potential relationship between cervical spondylosis and BRP while examining the role of surgical intervention as a treatment option.

Open access

Minimally invasive resection of a prominent transverse process in neurogenic thoracic outlet syndrome: new application for a primarily spinal approach. Illustrative case

Marc Hohenhaus, Johann Lambeck, Nico Kremers, Jürgen Beck, Christoph Scholz, and Ulrich Hubbe

BACKGROUND

The optimal surgical approach to treat neurogenic thoracic outlet syndrome (nTOS) depends on the individual patient’s anatomy as well as the surgeon’s experience. The authors present a minimally invasive posterior approach for the resection of a prominent transverse process to reduce local muscular trauma.

OBSERVATIONS

A 19-year-old female presented with painful sensations in the right arm and severe fine-motor skill dysfunction in the right hand, each of which had been present for several years. Further examination confirmed affected C8 and T1 areas, and imaging showed an elongated C7 transverse process displacing the lower trunk of the brachial plexus. Decompression of the plexus structures by resection of the C7 transverse process was indicated, owing to persistent neurological effects. Surgery was performed using a minimally invasive posterior approach in which the nuchal soft tissue was bluntly dissected by dilatators and resection of the transverse process was done microscopically through a tubular retractor. The postoperative course showed a sufficient reduction of pain and paresthesia.

LESSONS

The authors describe a minimally invasive posterior approach for the treatment of nTOS with the aim of providing indirect relief of strain on brachial plexus structures. The advantages of this technique include a small skin incision and minor soft tissue damage.

Open access

Adult neuromuscular choristoma, a rare peripheral nerve pathology: illustrative case

Ashley Ricciardelli, Alex Flores, Hsiang-Chih Lu, Ekin Guney, Arie Perry, and Joey Grochmal

BACKGROUND

Neuromuscular choristomas (NMCs) are rare tumors involving aberrant intercalation of muscle fibers into peripheral nerves, most commonly the sciatic nerve. Although benign, these lesions risk developing into NMCs with desmoid-type fibrosis (NMC-DTFs), aggressive lesions potentially requiring amputation. Currently, information on NMCs and the link between NMCs and NMC-DTFs is limited in adults, with the majority of cases reported in children. We present the case of a 66-year-old male with a sciatic NMC alongside a Preferred Reporting Items for Systematic Reviews and Meta-Analyses–based systematic review of similar cases to better characterize this lesion in the adult population.

OBSERVATIONS

A male presented with 10 years of progressive left lower-extremity weakness and paresthesia, and a mildly enlarged proximal sciatic nerve was discovered on magnetic resonance imaging. He underwent left sciatic fascicular nerve biopsy, with histopathological examination identifying the lesion as an NMC. The literature review revealed that our case, alongside other cases of adults with NMCs, shared characteristics similar to NMCs in the pediatric population.

LESSONS

More comprehensive studies of adults with NMCs are needed, as the current literature contains limited information concerning disease course, diagnostic characteristics, and treatment. Furthermore, NMCs in adults should be handled with care because of the increased likelihood of transformation to NMC-DTF after surgical intervention.

Open access

Exuberant circumferential fibroproliferative neuromas in lipomatosis of nerve: a unifying theory. Illustrative case

Andres A Maldonado, Mark A Mahan, Jodi M Carter, Kimberly K Amrami, Tomas Marek, and Robert J Spinner

BACKGROUND

Lipomatosis of nerve (LN) is a rare disorder characterized by the massive enlargement of peripheral nerves, frequently accompanied by generalized fibroadipose proliferation and skeletal overgrowth.

OBSERVATIONS

The authors have been routinely following a 20-year-old male for lipomatosis of median nerve at the wrist noted shortly after birth. He had undergone resection of the lesion accompanied by sural nerve grafting at another institution. Clinically, although his neurological loss of function has been stable, he has had continued soft tissue growth. Serial magnetic resonance imaging has revealed persistent LN proximal to the repair sites with evidence of fatty proliferation in the sural grafts and continued LN and fatty proliferation distally. There has been a progressive circumferential pattern of fibrosis around the proximal and distal suture lines, which has a similar radiological pattern to desmoid type fibromatosis (a pattern recently described in neuromuscular choristoma [NMC] desmoid-type fibromatosis).

LESSONS

Considering the similar reaction of nerve in both LN and NMC despite differing genetic cascades, the authors believe a unifying process occurs in both lesions. The pattern of circumferential fibroproliferation would be most consistent with neuron-mediated growth from unspecified trophic factors, supporting a previously reported a nerve-derived “inside-out mechanism.” The clinical consequences of this unifying process are presented.

Open access

Unrecognized sciatic lipomatosis of the nerve diagnosed by telehealth: illustrative case

Tomas Marek, Noah D Spinner, and Robert J Spinner

BACKGROUND

Lipomatosis of the nerve (LN) is a rare congenital hamartoma in which abundant fibroadipose tissue is found within the epineurium of peripheral nerves. It is frequently associated with nerve territory overgrowth affecting bones or soft tissues. The clinicoradiological features are well known and pathognomonic.

OBSERVATIONS

A 38-year-old female was evaluated via telehealth for worsened sciatica after the resection of a sciatic notch lipoma, after having been evaluated over a decade for symptoms. Virtual physical examination revealed previously unrecognized subtle limb discrepancy (i.e., overgrowth). Although preoperative radiographs were unavailable during the initial evaluation, postoperative magnetic resonance imaging studies showed evidence of sciatic nerve LN. A diagnosis of LN and nerve territory overgrowth was established.

LESSONS

Despite its limitations, telehealth can be an effective alternative or adjunct to in-person evaluations in making complex diagnoses. Dedicated examination without clinical suspicion can miss subtle findings. An iterative process prompted by a focused history, knowledge of clinical associations, and pattern recognition can facilitate an accurate diagnosis without obvious face-to-face visual clues. In patients with rare disorders, asynchronous evaluation allows in-person visits to be supplemented by subsequent telehealth virtual physical exam findings. This case illustrates how examination via telehealth can be used to effectively diagnose a rare syndrome.

Open access

“Winged” Eagle’s syndrome: neurophysiological findings in a rare cause of spinal accessory nerve palsy. Illustrative cases

Eric C Mitchell, Kitty Y Wu, Fawaz Siddiqi, John Yoo, Pavlo Ohorodnyk, Douglas Ross, and Thomas A Miller

BACKGROUND

Eagle’s syndrome (ES) classically describes dysphagia, globus sensation, and otalgia from an elongated and calcified styloid process or stylohyoid ligament. Compression of the spinal accessory nerve (SAN) has not been reported as an associated feature of ES or related variants.

OBSERVATIONS

The authors describe two cases of an atypical “winged” variant with SAN palsy resulting from compression by a posteriorly angulated or calcified styloid process. Both patients exhibited lateral scapular winging and atrophy of the trapezius and sternocleidomastoid muscles. Electrophysiological studies demonstrated motor unit preservation; therefore, surgical exploration, styloidectomy, and SAN decompression were performed through a transcervical approach. Postoperatively, both patients had improvements in pain and shoulder mobility, the return of muscle strength, and electrophysiological evidence of trapezius reinnervation.

LESSONS

Compression of the SAN, which can be identified both clinically and on electrodiagnostic testing, is an atypical finding that can result from a posteriorly angulated or calcified styloid process. This winged variant of ES should be included in the differential for SAN palsy, and a multidisciplinary approach is recommended for assessment and management.

Open access

Segmental extraneural lipomatosis of the superficial peroneal nerve: illustrative case

Kitty Y Wu, Aditya Raghunathan, and Robert J Spinner

BACKGROUND

Adipose lesions of nerve are rare tumors that can cause nerve symptoms from either intrinsic or extrinsic compression.

OBSERVATIONS

The authors present a case of a patient with a 10-year history of progressive, persistent leg pain and dorsal foot paresthesias/dysesthesias. Imaging revealed several nondistinct nodules of indeterminate significance along the course of the superficial peroneal nerve (SPN). Surgery demonstrated six distinct extraneural lipomas studded on a 10-cm segment of the main SPN and one of its muscular branches in the midleg. The lesions were adherent to the SPN, without an easy dissection plane; therefore, a neurectomy was performed. Histology revealed the nerve was associated with multiple extraneural lipomas with focal evidence of prior trauma. At 4 months postoperatively, the patient’s pain had resolved completely, and she was able to resume normal physical activities.

LESSONS

The current classification of adipose lesions of nerve includes intraneural and extraneural lipomas and lipomatosis of nerve (fibrolipomatous hamartoma). The unique features of the present case include the discrete and segmental nature of the extraneural lipomas adherent to the nerve. Its etiology is unknown, and the histology would be suggestive of either a traumatic or a degenerative process.