Hirayama disease successfully treated by posterior fixation: a case report.

Introduction and importance: Hirayama disease is a rare form of motor neuron amyotrophy that usually presents with weakness and atrophy of the distal upper extremities in young males. It is believed that it is caused by spinal cord compression during neck flexion because of the widening of the posterior extradural space. This case has been brought to attention due to its extraordinary rarity, serving as an educational tool for medical professionals and to advocate for surgical intervention when deemed necessary. Case presentation: The authors present a case of a young male in his 20s who was diagnosed with Hirayama disease, had weakness and atrophy in both of his upper limbs, and has been successfully treated by posterior fixation at C4, C5, and C6 with lateral mass screws. Clinical discussion: The majority of cases stabilize after 2-3 years of progression; therefore, cervical collars are generally sufficient for therapy. However, in certain serious cases with progression even after that time, surgical intervention is an option. Because this is such an uncommon incidence, surgical therapy has not been explored and is controversial. Conclusion: The use of posterior fixation at C4, C5, and C6 with lateral mass screws as a therapy for Hirayama disease may be regarded as a successful approach.

Venous pathology targeted surgical management in Hirayama disease: A comprehensive case series of nine cases exploring this potential etiology.

Objective: Hirayama disease is a rare cause of cervical myelopathy predominantly affecting young individuals. The disease is classically characterized by muscle atrophy in the distal upper limbs. While various etiopathogenesis such as dural sac dysplasia, nerve root dysplasia, structural abnormalities of the spinal ligament, and venous dysplasia have been proposed, this study explores the potential role of venous pathology and surgical management on the basis of it. Methodology: This is a prospective descriptive case series of nine cases. The diagnosis was made based on the Huashan diagnostic criteria which includes clinical manifestation, imaging, and electrophysiology. In cases where magnetic resonance imaging (MRI) failed to demonstrate engorged veins, a computed tomography (CT) venogram of the cervical spine was used as an imaging tool. All patients underwent cervical laminectomy and coagulation of the posterior epidural venous plexus with or without laminoplasty. All the patients were followed up regularly; clinical improvement and neck disability index were assessed. Results: All nine patients were male and exhibited classical clinical features, electrophysiological abnormalities, and MRI findings except, in one patient where a CT venogram helped in establishing the diagnosis as the MRI was inconclusive. Postoperatively, all patients had neurological improvement and stabilization of the disease. All patients who underwent CT venogram and cervical spine X-ray in neutral and dynamic position demonstrated no recurrence of engorged venous plexus or significant instability except one patient developing kyphosis. One patient experiencing symptoms in the other limb underwent a second surgery. Conclusion: This comprehensive case series strongly supports venous pathology as a potential etiology of Hirayama disease. Surgical management with laminectomy and venous coagulation with or without expansile laminoplasty has delivered consistent improvement in neurological outcomes and long-term disease stabilization without the restriction of movements and lesser complications. However, further research is warranted to elucidate the mechanism underlying cervical venous dilatation.

Clinical Outcomes of Anterior Cervical Decompression and Fusion Therapy for Patients With Hirayama Disease.

OBJECTIVES: To investigate the clinical outcomes of anterior cervical decompression and fusion (ACDF) surgery for the treatment of Hirayama disease (HD). METHODS: In this study, 15 patients with HD who underwent ACDF operation between March 2022 and March 2023 with complete data were retrospectively analyzed. Following the diagnosis, conservative treatment was ineffective, and thus, disease progression severely affected the quality of life (QOL) of patients. ACDF was performed in the China-Japan Friendship Hospital, and patients were regularly followed up postoperatively. The cervical range of motion (ROM), the anteroposterior and transverse diameter of the spinal cord, and their ratio was measured before and after the operation. The neurologic function of patients before and after the last follow-up was evaluated using the selected brief-Michigan Hand Questionnaire (sb-MHQ), whilst the overall therapeutic effect after the operation was evaluated using Odom's criteria. RESULTS: All patients were followed up for an average of 12 ± 4.5 (6-18) months. Dynamic X-ray displayed that the ROM of cervical vertebrae decreased from 72.73 ± 12.72° (53-97°) to 33.53° ± 10.34° (15-54°) (P < 0.001). Moreover, flexion cervical magnetic resonance imaging (MRI) performed after the operation revealed that spinal cord compression was markedly relieved, and the ratio of the anteroposterior diameter of the spinal cord to the transverse diameter increased from 0.27 ± 0.09 to 0.43 ± 0.03 (P < 0.001). At the last follow-up visit, finger extension tremor symptoms were alleviated, although they did not completely disappear. Contrastingly, muscle atrophy showed no significant improvement. Finally, the sb-MHQ score significantly increased from 17.33±1.76 preoperatively to 24.80±1.78 at the last follow-up (P<0.001). CONCLUSIONS: Our results collectively highlighted the efficacy of ACDF for the treatment of HD. This procedure can limit excessive cervical flexion and repeated compression of the spinal cord during cervical movement and considerably improve upper limb functions.

Long term quality of life follow-up and functional impairment study in patients with Hirayama disease.

Hirayama Disease (HD) is a focal motor neuron disorder generally affecting young adults with a male predominance who experience weakness and atrophy in distal upper extremity muscles in an asymmetric or unilateral pattern. Progression is insidious though significant weakness occurs during a progressive phase of the disease over 2-5 years. The long-term outcome of HD is not as well-known and, thus, this study presents self-reported outcomes from HD patients years after a diagnosis. Thirty HD patients reported quality of life (QOL) and other functional outcome measures after a mean of just over 11 years from diagnosis. Variables that predicted better or worse outcome were analyzed. Overall, QOL was affected by HD though most patients were functional with limitations. No clear attributes of patients or their disease predicted outcome.

Anesthetic challenges in a patient with Hirayama disease with quadriparesis and autonomic dysfunction undergoing cervical spine surgery.

Hirayama disease is a rare neurological disorder, characterized by muscular atrophy of the distal upper extremities. The occurrence of spastic quadriparesis and autonomic dysfunction is rarely reported and has important perioperative considerations during cervical spine surgery for the treatment of this disorder. The role of the anesthesiologist is vital in the thorough assessment of the patient for the involvement of the pyramidal tract, autonomic dysfunction, gastroparesis, hyperreactive airway disease, and documentation of neurological deficits. Intraoperative concerns include safe manipulation of the airway during mask ventilation and the use of a flexible fibreoptic bronchoscope during endotracheal intubation to prevent neck flexion. It is also essential to avoid drugs, leading to histamine release. The use of multimodal monitoring including bispectral index and neuromuscular monitoring is crucial to prevent delayed recovery. Anticipation and management of exaggerated hypotension in response to anesthetic induction agents and prone position is the key to a successful outcome in patients with autonomic dysfunction.

Hirayama's disease associated with cervical deformity and spinal cord compression: a case report from Sweden.

BACKGROUND: Hirayama's disease (HD) is most common in young males, and previous studies are predominantly from Asian countries. The cause of HD is unknown but the most common theory about the pathology speculates on forward bending that causes a compression of the dura mater and the anterior horn of the spinal cord against the vertebra during an overstretch flexion that may result in myelopathy. Both anterior and posterior cervical surgical approaches have been shown to be effective in stopping the disease and improving function; however, HD is also reported to be a self-limited disease, and treatment with a cervical collar may be an alternative for these patients. CASE REPORT: We report HD in a 17-year-old male from Sweden who underwent surgical treatment with a 2 level anterior cervical discectomy and fusion (ACDF) due to neurological progression from HD after conservative treatment. CONCLUSION: HD is rare and is easily overlooked. Surgical intervention shows promising results for neurological progression, but HD is also reported to be a self-limited disease.

Anterior cervical discectomy and fusion for the treatment of pediatric Hirayama disease.

PURPOSE: Hirayama disease, a rare cervical myelopathy in children and young adults, leads to progressive upper limb weakness and muscle loss. Non-invasive external cervical orthosis has been shown to prevent further neurologic decline; however, this treatment modality has not been successful at restoring neurologic and motor function, especially in long standing cases with significant weakness. The pathophysiology remains not entirely understood, complicating standardized operative guidelines; however, some studies report favorable outcomes with internal fixation. We report a successful surgically treated case of pediatric Hirayama disease, supplemented by a systematic review and collation of reported cases in the literature. METHODS: A review of the literature was performed by searching PubMed, Embase, and Web of Science. Full-length articles were included if they reported clinical data regarding the treatment of at least one patient with Hirayama disease and the neurologic outcome of that treatment. Articles were excluded if they did not provide information on treatment outcomes, were abstract-only publications, or were published in languages other than English. RESULTS: Of the fifteen articles reviewed, 63 patients were described, with 59 undergoing surgery. This encompassed both anterior and posterior spinal procedures and 1 hand tendon transfer. Fifty-five patients, including one from our institution, showed improvement post-treatment. Eleven of these patients were under 18 years old. CONCLUSION: Hirayama disease is an infrequent yet impactful cervical myelopathy with limited high-quality evidence available for optimal treatment. The current literature supports surgical decompression and stabilization as promising interventions. However, comprehensive research is crucial for evolving diagnosis and treatment paradigms.

Laminoplasty with tented duraplasty for Hirayama disease.

BACKGROUND: Hirayama disease (HD) is a characterized by progressive amyotrophy of the upper limbs due to a forward displacement of the cervical dura during neck flexion. METHODS: Unlike other treatment options aiming at preventing cervical flexion (e.g., collar or arthrodesis), laminoplasty with tented duraplasty addresses dural dysplasia. Technically, the procedure consists in enlarging the dural sac by performing an expansile duraplasty that is secured to the yellow ligaments, in association with an open-book laminoplasty. CONCLUSION: Laminoplasty with tented duraplasty is a surgical option addressing the cause of HD to prevent further neurological deterioration while preserving cervical motion.

Successful treatment of respiratory failure in Hirayama disease.

Hirayama disease is a self-limiting cervical motor neuron disease, usually affecting the spinal cord at level C7-T1. We share an unusual case of Hirayama disease in a young man affecting roots C4-C6. He presented in coma due to diaphragm weakness and hypercapnic respiratory failure. Diagnosis was achieved via clinical presentation, neurophysiological examination, ultrasonography of the diaphragm and dynamic MR-imaging. Conservative treatment with a cervical collar resulted in remarkable improvement in respiratory and motor function.

Monomelic Amyotrophy/Hirayama Disease: Surgical Outcome in a Large Cohort of Indian Patients.

BACKGROUND: Hirayama disease (HD) is a cervical compressive myelopathy. Anterior cervical discectomy and fusion (ACDF) is identified as the best surgical approach. We evaluated surgical outcomes and factors influencing ACDF in HD. METHODS: Between 2015 and 2019, 126 patients with HD underwent ACDF. Contrast magnetic resonance imaging of the cervical spine in full flexion was performed. Clinical examination and preoperative/postoperative assessment of hand function using Fugl-Meyer assessment, Jebsen-Taylor hand function test, and handheld dynamometry were performed at 3-monthly intervals for 1 year. Surgical outcomes were assessed as per the Odom criteria and Hirayama outcome questionnaire. RESULTS: Age at onset and duration of illness were 12-31 years (mean, 18 ± 2.7) and 1-96 months (32.7 ± 24.4), respectively. All patients had progressive weakness and wasting of the affected limb. Cord atrophy was seen in 97.1%, with epidural detachment and engorgement of the posterior epidural venous plexus in all. All patients underwent ACDF. Of these patients, 54% had an excellent/good outcome and 39% had a satisfactory outcome as per the Odom scale at last follow-up (mean, 44.9 ± 16.5 months) after surgery. Handheld dynamometry showed improvement from preoperative values to 1 year follow-up. Duration of illness and age at onset had a negative correlation and the preoperative Fugl-Meyer score had a positive correlation with improvement. CONCLUSIONS: ACDF resulted in remarkable improvement or stabilization in neurologic deficits in many patients with HD. Because motor disability ensues over time, early surgical intervention during the progressive phase is advocated.

Brachioradialis Involvement in Hirayama's Disease: An Atypical Presentation of a Rare Cervical Myelopathy.

Juvenile monomelic amyotrophy (JMA), also known as Hirayama's disease, is a rare cervical myelopathy that predominantly affects young Asian males. It is characterized by degeneration of anterior horn cells due to compression by the redundant dural sac. This study presents an atypical case of a 23-year-old Indian male who exhibited uncommon symptoms of JMA. The patient displayed progressive weakness and atrophy in the left forearm, including the usually spared brachioradialis muscle. Electrophysiological tests and MRI scans solidified the diagnosis of Hirayama's disease. After wearing a cervical collar for one year, the patient's condition stabilized, reinforcing the diagnosis. Unlike most JMA cases, this instance highlights the involvement of the brachioradialis muscle, underlining the variability in JMA presentations. A precise diagnosis is contingent upon clinical criteria, dynamic MRI, and electrophysiological findings. Recognizing these variations is crucial for early detection and appropriate management of the disease.

The role of leisure-time physical activity in maintaining cervical lordosis after anterior cervical fusion and its impact on the motor function in patients with hirayama disease: a retrospective cohort analysis.

BACKGROUND: Surgical treatment has been increasingly performed in Hirayama disease (HD) patients to limit excessive neck flexion and restore cervical lordosis. However, postoperative recurrence of cervical lordosis loss may restart the progress of HD. Many studies have demonstrated a relationship between neck muscle strength and cervical lordosis, and it is widely accepted that leisure-time physical activity (LTPA) can increase muscle strength. However, there are few reports about the correlation between LTPA and maintenance of postoperative cervical curvature. OBJECTIVE: To quantify the cervical lordosis and motor function before and after operation in HD patients and to analyze the impact of postoperative LTPA levels on the changes in these measurements. METHODS: C2-7 Cobb were measured in 91 HD patients before, 2-5 days and approximately 2 years after operation. Motor unit number estimation (MUNE) and handgrip strength (HGS) were performed in all patients before and approximately 2 years after operation, and both cross-sectional area and fatty infiltration of posterior cervical muscles were measured in 62 patients. Long-form international Physical Activity Questionnaire and its different domains was administered to all patients at postoperative 2-year assessments. RESULTS: The C2-7 Cobb was larger immediately and approximately 2 years after operation than that at preoperative assessment (P < 0.05). The preoperative to postoperative change in C2-7 Cobb was associated with postoperative changes in the symptomatic-side HGS and bilateral MUNE measurements (P < 0.05). Importantly, the patients performing LTPA had greater improvements in C2-7 Cobb from immediate to approximately 2 years after operation and greater C2-7 Cobb at last follow-up than those without LTPA, and postoperative improvements in both symptomatic-side MUNE measurements and symptomatic-side HGS were also greater in the former than in the latter (P < 0.05). CONCLUSIONS: Postoperative LTPA has a positive effect on recovery/maintenance of cervical lordosis after operation, which may alleviate the motor unit loss of distal upper limbs in HD patients. Therefore, postoperative LTPA may be beneficial for postoperative rehabilitation or early conservative treatment of HD patients.

[Monomelic amyotrophy]. / Monomelicheskaya amiotrofiya.

Monomelic amyotrophy, also known as Hirayama disease, is a rare neurological disorder characterized by focal and latent onset of upper limb weakness and atrophy in the absence of sensory deficits, bulbar or pyramidal signs. It usually occurs in young patients. The disease usually begins unnoticeably and progresses slowly, and can manifest itself as unilateral or asymmetrical weakness, as well as atrophy of the distal upper limb. Sensory disturbances, reflex changes and signs of lesions of lower motor neurons are rare. This article describes a case of a patient with complaints of weakness not only in the upper but also in the lower extremities.

Changes in Self-Esteem in Patients with Hirayama Disease and its Association with Prognosis After Anterior Cervical Fusion Procedures.

OBJECTIVE: To quantify self-esteem in patients with Hirayama disease (HD) and investigate the impact of this psychosocial factor on surgical outcomes in HD. METHODS: The Rosenberg Self-Esteem Scale (RSES) was measured in 58 patients with HD before anterior cervical fusion. These patients further underwent motor unit number estimation, handgrip strength, disabilities of the arm, shoulder and hand (DASH), Beck Anxiety Inventory (BAI) and Beck Depression Index (BDI) before and 18 months after operation. Furthermore, the International Physical Activity Questionnaire (IPAQ) was administered to all patients at postoperative 18-month assessments. RESULTS: Compared with the general population, patients with HD showed a relatively lower RSES, and RSES was negatively associated with both postoperative DASH (r = -0.431, P < 0.05) and preoperative to postoperative changes (r = -0.295, P < 0.05) and positively associated with IPAQ (r = 0.472, P < 0.05). Similar to the difference in postoperative DASH scores, more patients with low self-esteem felt postoperative aggravated motor dysfunction than those with high/normal self-esteem (P < 0.05). Postoperative BDI exerted a partial mediating effect on the relationship between RSES and DASH scores (B = -0.30, P < 0.05), and postoperative BAI played a partial mediating effect on the relationship between RSES and IPAQ scores (B = 0.30, P < 0.05). CONCLUSIONS: The self-esteem of HD patients may be below the population norms. Importantly, relatively low self-esteem in HD patients may cause/worsen postoperative depression and anxiety, thereby resulting in poor self-reported surgical prognosis and an inactive lifestyle after operation. Therefore, perioperative treatment and rehabilitation efforts in HD patients, especially those with low self-esteem, should account for both physiological and psychological symptoms.

Association of joint hypermobility with range of cervical motion and its impact on the motor unit loss in patients with Hirayama disease.

INTRODUCTION/AIMS: Some patients with Hirayama disease (HD) may have generalized joint hypermobility (GJH), which may excessively increase cervical range of motion (ROM) and then worsen the HD. The purpose of this study was to identify the frequency of GJH in HD patients and to analyze the effect of GJH on cervical ROM and the severity of HD. METHODS: The Beighton scoring system (≥4) was used to diagnose GJH in 84 HD patients. All patients underwent assessments of cervical-flexion/extension ROM; motor unit number estimation in bilateral abductor pollicis brevis (APB) muscles; handgrip strength; and the disabilities of the arm, shoulder, and hand assessments. RESULTS: Concomitant GJH was identified in 20 (23.8%) HD patients. The HD patients with GJH exhibited greater cervical-flexion (P < .001) and cervical-extension (P = .033) ROM than those without GJH. Both greater single motor unit potential amplitudes (symptomatic side: P = .005; less-symptomatic side: P = .011) and lower motor unit numbers (symptomatic side: P = .008; less-symptomatic side: P = .013) in bilateral APB, along with lower compound muscle action potential amplitudes on the symptomatic-side APB (P = .039), were observed in patients with GJH than those without GJH. There was a mild negative correlation between motor unit number and cervical-flexion ROM in HD patients (symptomatic side: r = -0.239, P = .028; less-symptomatic side: r = -0.242, P = .027). DISCUSSION: The frequency of GJH in HD patients may be higher than in the general population. Importantly, GJH may exacerbate excessive cervical-flexion ROM, thereby worsening motor unit loss in HD patients. A cautious approach should be taken when treating HD due to possible comorbid GJH.

Cervical Spine Magnetic Resonance Imaging Findings in Hirayama Disease.

Background Hirayama disease is an uncommon type of cervical cord myelopathy seen typically in young males due to trauma from flexion movements. This study aims to assess the clinical presentations and classify the extent of various cervical spine MRI findings for the local population. Methodology A retrospective study of 13 patients diagnosed with Hirayama disease on cervical MRI was performed from January 2017 to December 2022 at Dr. D. Y. Patil Medical College, Hospital and Research Center, Pune. Results Of the 13 patients, 12 (92%) were male, and one (8%) was female. Nine (69%) patients were in the 16-25-year age group, two (15%) were in the 26-35-year age group, and one (8%) each was in the 6-15-year and 66-75-year age groups. Upper limb weakness was the most common clinical symptom seen in 12 (92%) patients, followed by distal muscle atrophy in seven (54%) patients. Tremors in the hand were a rare symptom seen in two patients. Claw hand was an atypical symptom seen in one patient. On cervical MRI, all patients showed excessive forward shifting of the posterior dura on flexion, with resultant cord compression due to tightness of the dural sac. One (8%) patient had no signs of myelopathy, while 12 (92%) patients had developed chronic myelomalacia and showed abnormal cord hyperintensity and atrophy in the lower cervical cord. All 13 (100%) patients showed increased laminodural space on flexion; the mean thickness was 4.08 mm, with the minimum and maximum thickness being 2.4 mm and 6.7 mm, respectively. Classifying by length of the anterior bulging dura, one (8%) patient showed involvement of less than two vertebral body segments, eight (62%) patients showed involvement of two to four vertebral body segments, and four (30%) patients showed involvement of more than four vertebral body segments. Crescent-shaped post-contrast enhancement on flexion was seen in all eight (100%) patients who underwent a contrast study. Prominent epidural flow voids on flexion were seen in six (46%) patients. Conclusions Hirayama disease is an uncommon type of cervical myelopathy seen typically in juvenile males. The occult onset of distal upper limb weakness and atrophy during puberty, typical MRI features of lower cervical cord atrophy, and the presence of a crescent-shaped enhancing mass in the posterior epidural space are pathognomonic of the condition. A few atypical cases can also occur. Early diagnosis and treatment are crucial to avoiding serious dysfunction.

Correlation Study Between Spinal Cord Function, Spinal Cord Morphology and Cervical Spine Alignments in Patients With Hirayama Disease.

OBJECTIVE: This study aimed to explore the relationship between diffusion tensor imaging (DTI) parameters, cervical spine alignments, and spinal cord morphological parameters in patients with Hirayama disease (HD). METHODS: In this retrospective cohort study, 41 HD patients were recruited from the Huashan hospital from July 2017 to November 2021. Patients received X-rays, conventional magnetic resonance (MR), and DTI scans in flexion and neutral positions. The DTI parameters assessed were calculated using the region of interest (ROI) method. Paired t-tests were performed on the DTI parameters of neck flexion and neutral position. Cervical spine alignments, including flexion and neutral Cobb angles, were measured, and range of motion (ROM) was calculated. Spinal cord morphological parameters were measured, including spinal cord atrophy (SCA) and loss of attachment (LOA). Spearman's correlation analysis between DTI parameters, cervical spine alignments, and spinal cord morphological parameters was performed. RESULTS: In comparing DTI parameters, segments of the C3/4, C4/5, C6/7, and lower cervical spine were significantly different, while segments of C5/6 were not significantly different. In Spearman's correlation analysis, the flexion Cobb angle was significantly correlated with the fractional anisotropy (FA) value (R2 = .111, P = .033) and apparent diffusion coefficient (ADC) value (R2 = .119, P = .027). Flexion FA values were correlated with SCA in C4/5 (R2 = .211, P = .003), C5/6 (R2 = .454, P < .001), and C6/7 (R2 = .383, P < .001) while flexion ADC values were correlated with SCA in the C4/5 (R2 = .178, P = .006), C5/6 (R2 = .388, P < .001) and C6/7 segments (R2 = .187, P = .005). CONCLUSION: The DTI parameters were correlated with the flexion Cobb angle and the SCA. These data support the dynamic cervical flexion compression hypothesis and indicate that the degree of SCA may be used to assess the condition of HD patients quantitatively.

Chiropractic management of a U.S. Veteran with myofascial pain and concurrent distal bimelic amyotrophy (Hirayama disease): a case report.

Background: Distal bimelic amyotrophy (DBMA) also known as Hirayama disease, is a rare, self-limiting motor neuron disease manifesting as atrophy of C7-T1 innervated muscles. We present a case report describing the chiropractic management of neck and thoracic pain in a patient with known DBMA. Case presentation: A 30 year-old black male U.S. veteran with DBMA presented with myofascial pain of the neck, shoulder, and back. A trial of chiropractic care was undertaken involving spinal manipulation of the thoracic spine and cervicothoracic region, manual and instrument-assisted soft tissue mobilization, and home exercise prescription. The patient reported modest improvement in pain intensity and did not experience any adverse events. Summary: This case presents the first documentation of chiropractic services in musculoskeletal pain management of a patient with concurrent DBMA. At this time there is no guidance in the existing body of literature for the safety and effectiveness of manual therapy in this population.

Contexte: La myélopathie cervicale basse, également connue sous le nom de maladie d'Hirayama, est une maladie rare et spontanément résolutive du motoneurone qui se manifeste par une atrophie des muscles innervés C7-T1. Nous présentons un rapport de cas décrivant la prise en charge chiropratique de douleurs cervicales et thoraciques chez un patient atteint d'une maladie d'Hirayama connue. Présentation du cas: Un vétéran américain noir de 30 ans, atteint de myélopathie cervicale basse, s'est présenté avec des douleurs myofasciales au cou, aux épaules et au dos. Un essai de soins chiropratiques a été entrepris comprenant des manipulations vertébrales de la colonne thoracique et de la région cervicothoracique, des mobilisations manuelles et instrumentales des tissus mous, et la prescription d'exercices à domicile. Le patient a fait état d'une amélioration modeste de l'intensité de la douleur et n'a pas ressenti d'effets indésirables. Résumé: Ce cas présente la première documentation des services chiropratiques dans la gestion de la douleur musculo-squelettique d'un patient souffrant d'une myélopathie cervicale basse. À l'heure actuelle, il n'existe pas d'orientation dans la littérature existante sur la sécurité et l'efficacité de la thérapie manuelle dans cette population.

Surgical management of Hirayama disease in a pediatric patient presenting with severe cervical kyphosis and focal myelopathy: illustrative case.

BACKGROUND: Hirayama disease (HD) is a rare, nonfamilial neuromuscular disease causing cervical myelopathy and deformity, most commonly effecting pubertal Asian males. Patients whose nonoperative treatment fails and who cannot tolerate long-term cervical immobilization, experience relapse after arrest of symptoms, or present with severe features warrant surgical treatment. Here, the authors present an unusual case of HD that resulted in rapid progression of severe cervical kyphosis and discuss surgical management strategies. OBSERVATIONS: A 15-year-old male presented with unprovoked neck pain, progressive chin-on-chest phenomenon, and cervical myelopathy. Imaging revealed a severe subaxial cervical kyphosis of 88° and severe spinal cord compression secondary to changes within the thecal sac, ligaments, and bony elements. He underwent a multistage surgery involving halo gravity traction, C3-6 anterior cervical discectomy and fusion, and C2 to T2 posterior instrumented fusion with C3-5 Smith-Petersen osteotomies. Cervical subaxial pedicle screws facilitated deformity correction through a cantilever technique. LESSONS: HD is rare and often self-limited. For severe or refractory cases of HD, guidelines for surgical management have been suggested, with a variety of approaches deemed efficacious. This is the first case of a patient presenting with such severe cervical deformity; early diagnosis and recognition is the first step toward prompt, adequate management.

Management of dynamic cervical kyphosis with dorsal epidural lipomatosis: a Hirayama disease variant? Illustrative case.

BACKGROUND: Hirayama disease, a cervical myelopathy characterized most commonly by a self-limiting atrophic weakness of the upper extremities, is a rare entity, scarcely reported in the literature. Diagnosis is made by spinal magnetic resonance imaging (MRI), which typically shows loss of normal cervical lordosis, anterior displacement of the cord during flexion, and a large epidural cervical fat pad. Treatment options include observation or cervical immobilization by collar or surgical decompression and fusion. OBSERVATIONS: Here, the authors report an unusual case of a Hirayama-like disease in a young White male athlete who presented with rapidly progressive paresthesia in all 4 extremities and no weakness. Imaging showed characteristic findings of Hirayama disease as well as worsened cervical kyphosis and spinal cord compression in cervical neck extension, which has not previously been reported. Two-level anterior cervical discectomy and fusion and posterior spinal fusion improved both cervical kyphosis on extension and symptoms. LESSONS: Given the disease's self-limiting nature, and a lack of current reporting, there remains no consensus on how to manage these patients. Such findings presented here demonstrate the potentially heterogeneous MRI findings that can be observed in Hirayama disease and emphasize the utility of aggressive surgical management in young, active patients whereby a cervical collar may not be tolerated.