Primary intramedullary primitive neuroectodermal tumor of the cervical spinal cord. Case report

Primary intramedullary primitive neuroectodermal tumors (PNETs) of the spinal cord are rare. Only six cases have previously been reported, all involving tumors in the thoracic or lumbar spine. The authors report the case of a 54-year-old woman who presented with quadriplegia and bladder and bowel dysfunction. The patient had suffered symptoms of neck pain for 1 month and left shoulder weakness for 10 days. Magnetic resonance imaging of the cervical spine revealed an intramedullary mass extending from C-2 to C-5 with an exophytic component in the adjacent left subarachnoid space. Multiple biopsy specimens were obtained, and a partial excision was performed. Histological examination revealed nodular growth and neuronal differentiation, with a striking resemblance to desmoplastic medulloblastoma. A positron emission tomography scan did not reveal uptake at any site. These findings confirmed the diagnosis of a primary intramedullary PNET. Postoperatively, the patient was given craniospinal radiotherapy with a radiation boost to the tumor bed.     

Open stand-up MRI: a new instrument for positional neuroimaging

We present a 40-year-old man referred with complaints of neck pain, left arm pain, headaches, paresthesias in the index and middle fingers, with numbness in the C7 nerve root distribution. Conventional recumbent magnetic resonance imaging (MRI) was read by the radiologist as a small protrusion at C5-C6 that did not correlate with his symptoms. The patient had exhausted his treatment options. He underwent MRI in a weight-bearing, upright position with extension that revealed a positional cervical disc protrusion on the left at C6-C7. The protrusion was causing a proximal left C6-C7 neural foraminal stenosis and impingement that correlated with his symptoms. With this information, we were able to offer a targeted epidural block. Imaging the spine in the weight-bearing position with extension or placing the spine in the position of pain may increase the diagnostic accuracy for the neuroradiologist and neuroimagist, who then can provide the spine surgeon or neurosurgeon potentially with additional information to further improve patient care.     

Osteosarcoma arising from desmoplastic fibroma treated 16 years earlier: a case report

We present a rare case of malignant transformation of desmoplastic fibroma in a 37-year-old man. In 1984 we performed curettage and bone grafting for a bone tumor of the left distal femur. Histologically, we obtained a final diagnosis of desmoplastic fibroma. After this treatment, the patient had no particular symptoms but felt tension in the left knee in 2000 and consulted our department. We performed tumor curettage and bone grafting although malignant findings were recognized by imaging. The last pathology diagnosis was an osteosarcoma (in view of the formation of an osteoid). We performed caffeine-assisted chemotherapy and an additional wide exicision. In the literature, two reports of a high-grade sarcoma developing in a desmoplastic fibroma have appeared. It is sometimes difficult to distinguish desmoplastic fibroma from low-grade fibrosarcoma or intraosseous-type osteosarcoma, although the radiographic findings and aspects of the resected specimen in 1984 were typical of a desmoplastic fibroma. Moreover, from a clinical standpoint, because the recurrence came 16 years after the initial operation, and lung metastasis was not seen, it is difficult to believe this case was malignant originally. This extremely rare lesion is believed to be a desmoplastic fibroma transformed to osteosarcoma.     

Brachial plexus neuropathy

Branchial plexus neuropathy is characterized by acute onset of intense pain in the shoulder or arm followed shortly by focal muscle weakness. This presentation may mislead the clinician into diagnosing shoulder or cervical spine pathology. Although brachial plexus neuropathy is not common, it should be considered in the differential diagnosis of pain and weakness of the arm. We present a patient with brachial plexus neuropathy who was originally misdiagnosed as having a cervical disc herniation.     

Neck Disability Index,short form-36 physical component summary,and pain scales for neck and arm pain: the minimum clinically important difference and substantial clinical benefit after cervical spine fusion

Background ContextThe Neck Disability Index (NDI), the short form-36 (SF-36) physical component summary (PCS), and pain scales for arm and neck pain are increasingly used to evaluate treatment effectiveness after cervical spine surgery. The minimum clinically important difference (MCID) is a threshold of improvement that is clinically relevant to the patient. However, the true goal is to provide the patient with a substantial clinical benefit (SCB).PurposeThis study determines the MCID and SCB using common anchor-based methods for NDI, PCS, and pain scales for arm and neck pain in patients undergoing cervical spine fusion for degenerative disorders.Study Design/SettingThe study setting is a longitudinal cohort in a multisurgeon spine specialty clinic.Patient sampleThe sample comprises 505 patients who underwent a cervical fusion for degenerative spine conditions and who have prospectively collected outcome scores with a minimum 1-year follow-up.Outcome MeasuresThe outcome measures of the study were NDI, SF-36, and numeric rating scales for arm and neck pain.MethodsThe MCID and SCB values for NDI, PCS, and pain scales for arm and neck pain were determined using receiver operating characteristic (ROC) curve analysis with the Health Transition Item of the SF-36 as an anchor. The Health Transition Item asks a patient “Compared to one year ago, how would you rate your health in general now?” with answers ranging from “Much Better,” “Somewhat Better,” “About the Same,” “Somewhat Worse,” to “Much Worse.” An ROC curve was constructed for each measure. The ROC curve–derived MCID was the change score with equal sensitivity and specificity to distinguish the “Somewhat Better” from the “About the Same” patients. The ROC curve–derived SCB was the change score with equal sensitivity and specificity to distinguish the “Much Better” from the “Somewhat Better” patients. Distribution-based methods including the standard error of the mean and the minimum detectable change were also used to calculate MCID.ResultsThe calculated MCID is 7.5 for the NDI, 4.1 for SF-36 PCS, and 2.5 for arm and neck pain. The calculated SCB is 9.5 for the NDI, 6.5 for SF-36 PCS, and 3.5 for arm and neck pain.ConclusionsPatients with an eight-point decrease in NDI, a 4.1-point increase in PCS, and a three-point decrease in arm or neck pain can detect a minimally clinically important change. Patients with a 10-point decrease in NDI, a 6.5-point increase in PCS, and a four-point decrease in arm or neck pain can detect an SCB after cervical spine fusion.     

Thoracic outlet syndrome of pectoralis minor etiology mimicking cardiac symptoms on activity: a case report

Thoracic outlet syndrome is the result of compression or irritation of neurovascular bundles as they pass from the lower cervical spine into the arm, via the axilla. If the pectoralis minor muscle is involved the patient may present with chest pain, along with pain and paraesthesia into the arm. These symptoms are also commonly seen in patients with chest pain of a cardiac origin. In this case, a patient presents with a history of left sided chest pain with pain and paraesthesia into the left upper limb, which only occurs whilst running. The symptoms were reproduced on both digital pressure over the pectoralis minor muscle and on provocative testing for thoracic outlet syndrome. The patient’s treatment therefore focused on the pectoralis minor muscle, with a complete resolution of symptoms. This illustrates that not all cases of chest pain with associated arm symptoms that occur on physical activity are of cardiac origin.     

Occult fracture-dislocation of the cervical spine

Summary Occult injuries of the cervical spine in certain patients may lead to misdiagnosis or delayed diagnosis and treatment. The authors present a patient with an occult cervical spinal fracture-dislocation and review the literature. A 37 year-old male was involved in a motor vehicle accident. At his admission, the patient was alert and denied any pain in the cervical region, as well as neurologic symptoms. Physical examination revealed painless range of motion of his neck and no sensory or motor deficits. Plain radiographs of the cervical spine showed unilateral dislocation of C₄ on C₅ vertebra with fracture of the left facet of C₅ vertebra. Computed tomography scanning showed no neural compression. Operative reduction, stabilization and arthrodesis of the spine were advised, but the patient refused operative treatment. One year after his initial injury, the patient presented with torticollis and no neurologic symptoms.     

Cervical pneumatocyst

The authors present the case of a 56-year-old right hand-dominant woman who was referred for chronic neck pain and a second opinion regarding a cervical lesion. The patient's pain was localized to the subaxial spine in the midline. She reported a subjective sense of intermittent left arm weakness manifesting as difficulty manipulating small objects with her hands and fingers. She also reported paresthesias and numbness in the left hand. Physical and neurological examinations demonstrated no abnormal findings except for a positive Tinel sign over the left median nerve at the wrist. Electromyography demonstrated bilateral carpal tunnel syndrome with no cervical radiculopathy. Cervical spine imaging demonstrated multilevel degenerative disc disease and a pneumatocyst of the C-5 vertebral body. The alignment of the cervical spine was normal. A review of the patient's cervical imaging studies obtained in 1995, 2007, 2008, and 2010 demonstrated that the pneumatocyst was not present in 1995 but was present in 2007. The lesion had not changed in appearance since 2007. At an outside institution, multilevel fusion of the cervical spine was recommended to treat the pneumatocyst prior to evaluation at the authors' institution. The authors, however, did not think that the pneumatocyst was the cause of the patient's neck pain, and cervical pneumatocysts typically have a benign course. As such, the authors recommended conservative management and repeated MR imaging in 6 months. Splinting was used to treat the patient's carpal tunnel syndrome.     

Changes in the cervical spine in juvenile rheumatoid arthritis

One hundred and twenty-one patients with juvenile rheumatoid arthritis were studied clinically and roentgenographically for evidence of disease of the cervical spine. None of the fifty-seven patients with pauciarticular-onset juvenile rheumatoid arthritis had cervical symptoms or signs, and only one had minor roentgenographic changes of disease in the cervical spine. In contrast, clinical stiffness and roentgenographic changes in the cervical spine occurred commonly in the fifty-one patients with polyarticular-onset disease and in the thirteen patients with systemic-onset disease. Despite extensive roentgenographic involvement of the cervical spine, however, pain in the neck was not a common complaint. Neither severe pain in the neck nor torticollis, occurring either separately or concomitantly, is frequently found in patients with juvenile rheumatoid arthritis, and its presence may suggest an intercurrent problem such as a fracture or infection. As patients with juvenile rheumatoid arthritis rarely have disease in the cervical spine alone, the patient should be carefully examined for involvement of multiple joints.     

Chondromyxoid fibroma of the cervical spine: case report

OBJECTIVE AND IMPORTANCE: The clinical, pathological, and imaging findings in a patient with a chondromyxoid fibroma involving the C2 vertebra are reported. Seven cases that involve the cervical spine have previously been reported in the literature; the posterior vertebral body and posterior elements are the most frequently involved. To our knowledge, this is the first reported case of C2 involvement and craniocervical instability. CLINICAL PRESENTATION: A 20-year-old man presented with intermittent neck pain resulting from a fall 4 years earlier. X-rays and magnetic resonance imaging demonstrated a lytic lesion in the body of C2, as well as instability at C1-C2. INTERVENTION: Preoperative transcatheter angiography and embolization were performed. An expansile tumor of the C2 vertebral body was resected via a transoral approach. Because of craniocervical instability, fusion from the occiput to posterior elements of C1 through C4 was performed. CONCLUSION: Chondromyxoid fibroma is a rare benign bone tumor that is uncommonly found in the spine and may mimic other lesions, particularly radiographically. We report the first known involvement of C2 by this tumor in the modern English-language literature. The patient has had excellent clinical and radiographic resolution of the prior instability with no neck pain, instability, or local recurrence.     

Acute neck pain caused by calcium pyrophosphate dehydrate deposition in the transverse ligament of the atlas: a case report

We report a case of pseudogout manifested by severe posterior neck pain. Pseudogout of the neck, also known as the crowned dens syndrome, causes acute neck pain characterized by calcium pyrophosphate dehydrate deposition around the odontoid process. Crowned dens syndrome is typified clinically by severe cervical pain and stiffness, often in conjunction with raised inflammatory markers. A 71-year-old man presented with severe neck pain. On admission, elevation of serum CRP level was confirmed. Magnetic resonance images showed no responsible abnormalities except for degenerating change of the spine. The patient was diagnosed as having pseudogout caused by calcium pyrophosphate dehydrate deposition based on cervical computed tomographic imaging, which showed linear calcification in the transverse ligament of the axis. After administration of non-steroidal anti-inflammatory drugs, the fever and neck pain disappeared and the CRP level returned to within the normal range. Pseudogout of the cervical spine should be considered as a differential diagnosis when we examine patients with acute neck pain. Cervical spinal computed tomographic scan is a more sensitive and useful examination method to diagnose this disease rather than magnetic resonance images.     

Desmoplastic fibroma of the fibula. A difficult clinical, radiological and histological diagnosis

Desmoplastic fibroma of bone is a rare benign tumor that is made up of wavy fibroblasts and abundant collagenous tissue. The case of a 18-year-old patient is presented with a two months history of weightbearing pain in the left knee. Neither native x-ray, CT nor MRI could detect the kind of tumor. The histological findings lead finally to the diagnosis of a desmoplastic fibroma. Wide resection prevented recurrence of the tumor for 8 1/2 years until now. Considering the semi-malignant character of the desmoplastic fibroma and the recurrence rate marginal or wide resection for the primary treatment is recommended. The superior imaging quality of MRI facilitates preoperative planning.     

Diaphragm paralysis from cervical disc lesions

An opera singer, who "made her living with her diaphragm", developed a post-traumatic unilateral radiculopathy due to cervical disc lesions, C3 to C6. During one year of severe neck and left arm pain she gradually lost the ability to sing difficult operatic passages which brought an end to her music career. Following a three level anterior cervical decompression and fusion, the neck and arm pain was immediately relieved. One week later her voice and singing ability returned to its full strength and power permitting her to resume her activities as a vocalist. The diagnosis of paresis of the left hemi-diaphragm as part of the cervical disc syndrome was implied by postoperative retrospective inference.     

Adverse swelling associated with use of rh-BMP-2 in anterior cervical discectomy and fusion: a case study.

BACKGROUND CONTEXT: The use of recombinant bone morphogenetic protein-2 (rh-BMP-2) is effective in augmenting lumbar spinal fusions. A safe, effective dosage of rh-BMP-2 in the cervical spine has yet to be determined. Use of rh-BMP-2 is currently being evaluated and is only indicated for use in the cervical spine on Investigational Device Exempt cases. PURPOSE: To present a potentially serious adverse event that may occur when using rh-BMP-2 in cervical spine surgery. STUDY DESIGN: An emergent sequence of events including physical examination, radiographic studies, intubation, and surgical exploration were performed upon arrival to the emergency department. METHODS: We report a case of a 54-year-old male patient presenting with neck swelling and difficulty swallowing 5 days after anterior cervical discectomy and fusion (ACDF) using rh-BMP-2. RESULTS: The patient was found to have massive neck swelling including the pharyngeal tissue. The patient was admitted to the intensive care unit where parenteral steroids were administered for 24 hours during monitored intubation. The patient was extubated on the second hospital day and discharged home on the fourth hospital day after swelling subsided. CONCLUSIONS: Caution should be exercised with rh-BMP-2 use in ACDF surgery as the correct dose and technique for application is not yet determined. Respiratory distress and dysphagia may result secondary to rh-BMP-2 induced anterior neck swelling.     

Cervical radiculopathy or Parsonage-Turner syndrome: differential diagnosis of a patient with neck and upper extremity symptoms

STUDY DESIGN: Resident's case problem. BACKGROUND: The signs and symptoms of cervical radiculopathy (CR) warrant the consideration of several other conditions in CR's differential diagnosis. One condition that may mimic CR, which is not well known amongst physical therapists, is Parsonage-Turner syndrome (PTS). PTS is characterized by an onset of intense pain that typically subsides within days to weeks. However, as pain subsides, weakness and/or paralysis may develop in upper extremity muscles. The purpose of this resident's case problem is to describe a patient who presented to our clinic with a diagnosis of CR, but had findings consistent with PTS. DIAGNOSIS: The patient was a 43-year-old male referred to physical therapy with a diagnosis of CR. He had a previous episode of CR 1 year ago that was treated successfully. He had positive magnetic resonance imaging findings of structural abnormalities suggestive of causative factors for CR. The patient was treated for CR with thoracic and cervical spine manipulations and intermittent cervical traction. The initial acute severe pain subsided, but weakness in the upper extremity worsened. Diagnosis of PTS was made upon exclusion of other potential confounding diagnoses and the findings of fibrillation potentials and positive waves in electrodiagnostic studies. DISCUSSION: CR and PTS are characterized by pain in the cervical spine, shoulder, and upper extremity. CR generally has an insidious onset, while PTS has a rapid onset of intense pain. Symptoms of CR are exacerbated with neck movements, while symptoms related to PTS should not be exacerbated with neck movements. In patients that do not respond to conventional therapy and have a progression of upper extremity muscle weakness, regardless of decreased pain, the diagnosis of PTS should be considered.     

Spontaneous cervical epidural hematoma presenting with hemiparesis following neck extension: a case report

A 42-year-old woman suddenly developed weakness in her left extremities when stretching her neck two days after the onset of a nuchal pain. Because computed tomography (CT) of the brain did not show any apparent lesion, the patient had initially been treated as having a cerebral infarction until magnetic resonance imaging (MRI) of the cervical spine revealed a presence of a cervical epidural hematoma the next day. She was therefore transferred to our hospital, and a neurological examination showed moderate left hemiparesis, dissociated sensory loss under C6 on the right side, urinary incontinence, and left miosis and ptosis. A CT of the cervical spine demonstrated an anteriorly located left-sided epidural hematoma extending from C4 to C7. The T2-weighted MRI revealed hyperintense lesions around the gray matter on the left side that were compressed by the epidural hematoma. The patient underwent an emergent laminoplasty from C3 to C7. Although her neurological signs were consistent with Brown-Sequard syndrome, which was associated with left-sided Homer's sign, they gradually and completely subsided following surgery. The authors therefore emphasize that cervical lesions should be considered in the differential diagnosis in patients with acute onset of hemiparesis.     

Cervicobrachialgia and pancoast tumor: value of standard anteroposterior cervical radiographs in early diagnosis

Ten patients diagnosed with Pancoast tumor were studied retrospectively. The definitive diagnosis was made between 2 and 24 months after the onset of pain. Pain localization was hard to pinpoint; some patients reported pain in four different sites (neck, shoulder, arm, and scapula). Five patients had previously been diagnosed with degenerative, inflammatory, or infectious diseases of the cervical spine or shoulder. In the remaining five patients, the diagnosis was made during the first clinical visit. In three patients, an orthopedic surgeon made the diagnosis by viewing a standard anteroposterior (AP) cervical radiograph. The radiographic evidence arousing suspicion of a Pancoast tumor was the lack of pulmonary air at the top of the affected lung. Furthermore, a parallel study was conducted on 100 consecutive patients seeking treatment for neck pain. By examining the AP radiographs of their cervical spines, the third rib and the top of both lungs were observed in all cases. This study stresses the value of standard AP cervical radiographs in the diagnosis of Pancoast tumor.     

Metastatic cardiac angiosarcoma of the cervical spine. Case report.

STUDY DESIGN: A case report of metastatic cardiac angiosarcoma of the cervical spine. OBJECTIVES: To show that this rare spine tumor behaves in the same manner as an arteriovenous malformation and embolization, which can allow for successful spine surgery, and to discuss the natural history and rarity of this tumor. SUMMARY OF BACKGROUND DATA: Primary angiosarcoma of the heart is a very rare tumor, with fewer than 200 reports in the English literature and nothing reported in the spine literature. RESULTS: The patient in this study initially sought treatment for neck pain, left arm pain, and weakness 17 months after cardiac surgery and subsequent chemotherapy. A cervical computed tomography scan demonstrated a C5 lytic vertebral body tumor with intracannilicular extension and cord compression. An anterior cervical approach was made, but the tumor was too vascular to resect, and surgery was aborted. The C5 vascular vertebral body metastasis subsequently was embolized successfully by an interventional neuroradiologist. Reoperation via an anterior approach with corpectomy, cadaveric fibula, and anterior locking plate internal fixation was successful, producing marked improvement in the patients' symptoms. CONCLUSION: Spinal involvement by primary cardiac angiosarcoma is very rare, and this is only the second operative case ever reported. The vascular nature of this tumor makes it behave in a manner similar to that of a high-flow arteriovenous malformation. Surgery should not be undertaken before preoperative angiography and embolization. The dismal prognosis for this rare malignancy is discussed.     

Clinical decision making associated with an undetected odontoid fracture in an older individual referred to physical therapy for the treatment of neck pain

STUDY DESIGN: Resident's case problem. BACKGROUND: The purpose of this paper is to provide the examination of and decision-making process for a patient referred to physical therapy for the treatment of neck pain following trauma. She was found to have an underlying odontoid fracture that precluded physical therapy intervention. DIAGNOSIS: This case involved a 73-year-old woman who had a sudden onset of neck and left upper extremity pain after a fall 15 days prior to her initial physical therapy visit. Conventional cervical spine radiographs completed 1 day prior to her initial physical therapy visit were negative for a fracture. However, several components of this patient's history and physical examination were consistent with a condition for which physical therapy intervention would not be indicated until more definitive cervical spine diagnostic imaging had been completed; more specifically, the physical therapist was primarily concerned about the possibility of an undetected fracture. The referring physician was contacted and immediate magnetic resonance imaging was requested, which revealed a type II fracture of the odontoid. Thirty-four days after her fall, the patient underwent a C1-C2 fusion. DISCUSSION: When evaluating patients with neck pain who have a history of cervical spine trauma, it is important that physical therapists understand the clinical findings associated with cervical spine fractures, as these findings provide guidance for the use of cervical spine diagnostic imaging and medical referral prior to implementing physical therapy interventions. LEVEL OF EVIDENCE: Diagnosis, level 4.     

Septic arthritis of the atlantoaxial joint: case report

Bacterial epidural abscess formation of cervical spine was rarely seen and all these cases were associated with osteomyelitis of the odontoid process. To the author's knowledge, only 1 case of septic arthritis of the C1-C2 lateral facet joint has been reported in the English literature. A 76-year-old woman presented with progressive neck pain and stiffness in the left side of her neck with fever. Coronal computed tomography clearly demonstrated destructive change of the atlantoaxial joint. Contrast magnetic resonance imaging demonstrated infection of the atlantoaxial joint resulting in epidural abscess formation. Because of lack of neurologic deficit, we treated her with halo-fixation together with appropriate antibiotics administration. The patient was successfully treated with antibiotics and immobilization. Septic arthritis of the lateral atlantoaxial joint should be added to the differential diagnosis of severe neck pain with fever, although it is very rare clinical entity. Early analysis of computerized tomography and magnetic resonance imaging although maintaining a high suspicion for this disease is mandatory to avoid delayed diagnosis and subsequent morbidity.