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Ochronosis is a rare hereditary disorder of tyrosine metabolism. Severe degenerative arthritis and spondylosis occur in the later stages of this disease. Radiologic examinations may reveal changes considered almost pathognomonic for ochronosis. We present the radiologic features of the lumbar spine in two ochronotic patients who were diagnosed after radiologic examinations in the late stages of the disease.
Post-polio syndrome (PPS) is the term used for the new late manifestations that occur in patients 30 to 40 years after the occurrence of acute poliomyelitis. PPS has been recognized for over 100 years, but is more common at the present time because of the large epidemics of poliomyelitis in the 1940s and 1950s. PPS is manifested by neurologic, musculoskeletal, and general manifestations. Neurologic manifestations include new weakness, muscle atrophy, dysphagia, dysphonia, and respiratory failure. Musculoskeletal manifestations include muscle pain, joint pain, spinal spondylosis and scoliosis, and secondary root and peripheral nerve compression. General manifestations include generalized fatigue and cold intolerance. New muscle weakness of a mild-to-moderate degree responds well to a nonfatiguing exercise program and pacing of activity with rest periods to avoid muscle overuse. Generalized fatigue may be treated with energy conservation and weight loss programs and lower extremity orthoses. Pharmacologic agents also may be helpful, but have not been beneficial in controlled trials. Bulbar muscle weakness includes dysphagia, dysphonia, sleep disorders, and chronic respiratory failure. Dysphagia may be improved with instruction on compensatory swallowing techniques. Dysphonia is treated with voice exercise therapy and voice amplification devices. Sleep disorders are treated similarly to sleep disorders in non-PPS patients. Respiratory failure may be treated with continuous positive airway pressure, bilevel positive airway pressure, and nasal ventilation, or tracheotomy and permanent ventilation if necessary. Musculoskeletal (muscle and joint) pain is treated with weight loss, pacing of activities, use of assistive devices, and prescribing anti-inflammatory medications and physical therapy techniques. Cardiopulmonary conditioning can be improved without muscle overuse with cycle or arm ergometer exercise or dynamic aquatic exercise.
PURPOSE: Ossification of the posterior longitudinal ligament (OPLL) is a common, well-recognized cause of spinal stenosis and myelopathy in Japan. Although also common in whites, especially among the elderly, it has received little scientific attention. We wish to increase awareness of this important cause of myelopathy, and to determine if the clinical characteristics of OPLL are similar in non-Japanese and Japanese patients. PATIENTS AND METHODS: The clinical and radiologic features of eight cases of OPLL are presented. These cases combined with 73 non-Japanese cases gathered from the English literature are contrasted with 2,125 Japanese cases of OPLL. RESULTS: Similarities among non-Japanese and Japanese cases included: (1) male predominance; (2) peak age at onset of symptoms in the sixth decade; (3) clinical presentation, which ranged from asymptomatic to quadriplegia, with progressive or acute onset of neurologic deterioration; (4) greater than 95% localization to the cervical spine, spastic quadriparesis being the most common neurologic presentation; (5) an association with several rheumatic conditions including diffuse idiopathic skeletal hyperostosis (DISH), spondylosis, and ankylosing spondylitis; and (6) neurologic improvement with either conservative or surgical treatment in a significant proportion of patients. Differences between the two groups were minimal and included a higher mean age at onset (although onset in both groups occurred within the sixth decade) and a greater proportion of patients with DISH and with the continuous type of OPLL in the non-Japanese group. CONCLUSION: The clinical characteristics of OPLL are similar in Japanese and non-Japanese patient populations. Increased awareness of this condition, which has potentially devastating neurologic complications, will favorably influence diagnosis, treatment, and outcome.
Previous reports have attributed the development of premature cervical spondylosis to movement disorders such as torticollis and athetosis. This case report describes the clinical, electromyographic, and radiographic findings in a 34-year-old man who developed a myelopathy and cervical radiculopathy superimposed on a chronic dystonia of his neck and left arm. Cervical myeloradiculopathy should be suspected in any patient with a chronic movement disorder of the hand, neck, or arms, who presents with neurologic deterioration. Early diagnosis will lead to treatment that may improve symptoms. Anterior cervical bony fusion appears to be the preferred surgical treatment in these patients.
A prospective scintigraphic and radiographic study was carried out in 35 patients with a whiplash injury of the cervical spine. The scintigraphic findings were within normal limits in 31 of the patients, while four had either a focal or multifocal increase in activity. Plain radiography revealed skeletal changes secondary to spondylosis in three patients and a localized bony spur with avulsion of a barely visible fragment in one. Our investigation shows that skeletal lesions after whiplash injury, although very few, are shown by plain skeletal radiography. Scintigraphy is not indicated for screening purposes.
Between 1965 and 1991, 132 patients with vertebral dislocation following hyperextension injury of the cervical spine were treated at the Spinal Injury Department of the Metropolitan Rehabilitation Centre in Konstancin, Poland. The series accounted for over 25 per cent of patients with hyperextension injuries and for about 7 per cent of all cervical-spine-injured patients treated during these years. These changes are most commonly found in two situations: (1) Injuries of the upper cervical spine. (2) Injuries sustained by individuals with advanced pathological changes in the spine. Such conditions are found in advanced spondylosis and ankylosing spondylitis. Such changes were noted in nearly 65 per cent of the patients in the series analysed. The improved results seen in surgically treated patients encourage the wider application of surgery in such groups of patients.
The complexities of interpreting results of electrical stimulation of the motor cortex in pathological states are discussed and illustrated by reference to results from a variety of patients with diseases affecting the upper motor neurone (multiple sclerosis, cervical spondylosis and myelopathy, motor neurone disease, hemiparesis due to cerebral infarction, and hereditary spastic paraplegia). The abnormalities of the electromyographic (EMG) responses after anodal cortical stimulation consisted of delay in the latency to onset, dispersion or reduction in response size or even absence of EMG responses. These changes were not confined to any specific condition or pathology. Previous work has suggested that the sequence of events that follow anodal cortical stimulation involves repetitive excitatory inputs to spinal motoneurones and transmission across at least one central synapse. Accordingly, delayed latencies may not exclusively indicate slowing of motor conduction, while the absence of any response may not indicate complete failure of conduction in corticomotoneurone pathways.
The trend toward anterior diskectomy for median and paramedian cervical disk rupture has tended to obscure progressive development of the posterolateral approach to these lesions. Modifications of surgical technique from the classic posterior approach have allowed direct access to these lesions, provided for satisfactory decompression of the spinal cord, especially when there is associated spondylosis, and avoided all of the disadvantages of anterior disk surgery. Of 28 patients operated on since 1950, 26 have had significant preoperative myelopathy or myeloradiculopathy. Two patients with obvious spinal cord compression and massive myelographic defects had no neurologic deficit. Improvement has been observed in every patient; 16 patients have had full recovery, and 8 others have had minor residual symptoms and asymptomatic signs. Although four patients have been lost to follow-up, they were all seen at least once after operation. No instance of increased deficit has been seen postoperatively, in contrast to the author's experience with spondylotic myelopathy. Postoperative contrast studies, which have now been performed on eight patients, confirm satisfactory excision of these lesions and decompression.
Anterior fixation of cervical spine with screw-plates is gaining in popularity in the management of anterior cervical spine instability. We report on our initial experience with anterior cervical fixation using the titanium locking screw-plate (TLSP). Twelve cases, including cervical trauma, cervical spondylosis, and ossification of the posterior longitudinal ligament (OPLL), underwent multilevel corpectomies or spondylectomies with iliac bone or hydroxylapatite grafting combined with the TLSP system. All 12 patients fused or stabilized with improvement of their neurologic symptoms at a mean follow-up period of 13 months. There were no complications relating to this plating system. Three illustrative cases are presented. Different from previous anterior cervical planting systems such as the Caspar plate, the TLSP system does not gain purchase on the posterior vertebral cortex. It thus reduces possible spinal cord injury during screw placement. The TLSP with interbody fusion may provide reliable stabilization of the cervical spine in a safe and effective manner.
Cervical spinal cord compression is easily and accurately demonstrated by CT. Isolated atypical sensory symptoms in the saddle area and lower extremities may be the earliest and only manifestations of cervical spinal cord compression from cervical spondylosis. A case is presented manifesting such symptoms, which were reversed completely by surgical decompression of the cervical spine.
Osteitis deformans has hitherto been regarded as a bone disease of unknown aetiology, but epidemiological studies have raised the possibility of an environmental factor. From the epidemiological studies, X-Rays of 1864 patients with Paget's disease were available for examination of the skeletal distribution of the disease. The epidemiological surveys were based upon examinations of X-Rays covering the lumbar spine, pelvis and upper ends of the femora, and all other X-Ray examinations on the patients showing Paget's disease were also studied for evidence of the disease outside the primary sites. There is a preference for the lower half of the body, and a distribution of the disease in the lumbar and dorsal vertebrae similar to spondylosis; a peri-articular origin of the disease is apparent in long bones, and there is a tendency for the right side of the body to be affected more than the left. The radiological distribution has been supported by radio-nucleide studies in a further 50 patients. In general, the vascular axial skeleton is affected, and it is postulated that an environmental factor is being transported by the blood to bone. Bone stress may then stimulate the changes of osteitis deformans.
A Short TI Inversion Recovery (STIR) sequence with spin-echo data collection was compared to a conventional cardiac gated long TR spin-echo (SE) sequence for detecting intramedullary signal abnormalities. The cervical (n = 48), cervico-thoracic (n = 1), and thoraco-lumbar (n = 18) spinal cord was imaged in a sagittal plane with a 0.5 T (n = 61) or 1.5 T (n = 6) MRI unit in 67 patients with clinical evidence of myelopathy of different etiologies (e.g., multiple sclerosis, trauma, herniated intervertebral disk, spondylosis, etc.). In all patients, ungated double or quadruple echo STIR images (TR 1000-1400 msec, TI 100 msec, TE 30-60 or 30-60-90-120 msec) were compared with cardiac gated long TR (1400-2100 msec), double echo (30-100, 50-100, or 50-150 msec) SE images with first order flow compensation for the second echo. Although STIR images appeared "noisier" than long TR SE images, they showed fewer ghost artifacts. In 55 patients, single or multiple, focal or diffuse, hyperintense areas within the spinal cord were observed on both long TR SE and (magnitude reconstructed) STIR images. Lesion conspicuity was better on the STIR images in 25 patients, better on the SE images in 14 patients, and equal in 16. STIR sequence provides a valuable alternative to gated long TR SE sequence for the MRI investigation of intramedullary spinal lesions.
Nine patients with unrecognized incomplete cervical spinal cord injury are discussed. Four were sent home as normal, three were called hysterical, and two went undiagnosed during stupor or coma. A literature search revealed 28 additional cases. Eighty percent of these were males, two thirds were over 50 years old, and most had central or posterior cord syndromes. Falls with hyperextension, spondylosis, or disc disease, and motor vehicle accidents were the most common causes. Only one of the 37 had a cervical fracture. In some the neurologic problem was missed altogether; in others it was attributed to hysteria, intoxication, or to other neurologic or systemic diseases. Minor injuries without cervical fracture or dislocation, advanced age, unusual or changeable neurologic deficits, intoxication, and psychiatric problems all contributed to the confusion.
Vertebral artery compression from head turning has been described in adults, especially in those who have spondylosis of the spine and osteophytes. There are no reports that demonstrate vertebral artery compression due to head turning in children without vertebral abnormalities. A child is reported with thalamic stroke in whom cerebral arteriography revealed vertebral artery compression on head turning. Blood flow in the vertebral artery, however, could be demonstrated by Doppler ultrasonography with the head in the midline. It is concluded that the vertebral artery is vulnerable to mechanical compression in the neck even in the absence of bony abnormalities.
We measured calcium-dependent phospholipase A2 (PLA2) activity and immunoreactive group II PLA2 levels of 54 normal discs obtained from cadavers and 73 disc samples surgically obtained from patients with spinal disorders, including intervertebral disc herniations, spondylosis, and spondylolisthesis. Both cadaveric and surgical disc specimens contained about two-fold greater PLA2 activity than the ileal mucosa, one of the richest sources of group II PLA2. Discs of middle-aged cases had significantly higher activity than those of younger and elder cases. In cadaveric normal discs, calcium-dependent PLA2 activity was significantly higher in females than in males. Annulus fibrosus and nucleus pulposus contained the same PLA2 levels. In diseased disc, herniated fragments that had extruded or protruded out of the discs possessed lower activity than other parts of discs in the intervertebral space. Immunoreactive group II PLA2 levels of intervertebral discs closely correlated with PLA2 enzymatic activity. We purified a PLA2 from human intervertebral disc to homogeneity to further identify the isozymic nature of discal PLA2. Its NH2-terminal amino acid sequences and molecular weight were identical to those of human group II PLA2. Immunohistochemical analysis using a monoclonal anti-group II PLA2 antibody showed that in both annulus fibrosus and nucleus pulposus chondrocytes contained intense group II PLA2 immunoreactivity in their cytoplasm, and that the matrix contained no substantial immunoreactivity. These results suggest that group II PLA2 in chondrocytes has important physiological roles in discal ordinary metabolism, maintaining discal homeostasis.
A 10-year-old uncastrated male Dalmatian dog was referred for gait abnormalities consisting of chronic progressive stiffness and rigidity. Other symptoms were polyphagia associated with weight gain, polyuria and polydipsia, excessive panting, and an inspiratory stridor. The owner had noticed progressive thickening of the skin and enlargement of the tongue over the last 3 years. Physical examination revealed thickening of the skin, redundant skin folds, and enlargement of the tongue. The only remarkable abnormalities found on routine laboratory examination were mild anaemia and an increased serum fructosamine concentration. Circulating concentrations of total thyroxine, free thyroxine, and cTSH, and the results of an ACTH stimulation test were all within reference ranges. The basal serum growth hormone (GH) concentration was markedly elevated (23microg/l) and did not decrease during a glucose tolerance test or after somatostatin administration. The serum insulin-like growth factor-1 concentration was also markedly elevated (1254microg/l). Basal serum insulin concentration was high (95mU/l) and insulin concentrations increased considerably after glucose loading, consistent with insulin resistance. Abdominal ultrasonography showed no abnormalities. Survey radiographs of the vertebral column showed severe spondylosis deformans extending from the cervical to the lumbosacral spine. CT scanning of the skull showed an enlarged pituitary gland with normal enhancement pattern. On post-mortem examination, the entire vertebral column appeared as a single and inflexible structure due to the presence of multiple fused osteophytes. The pituitary gland contained an acidophilic adenoma that immunostained positively for GH (and negatively for ACTH and alpha-MSH). In conclusion, this Dalmatian dog with acromegaly and insulin resistance represents the first case of GH hypersecretion proven to be due to a somatotroph adenoma.
OBJECTIVE: To describe the chiropractic management of a patient with paresthesia on the entire left side of her body and magnetic resonance imaging (MRI)-documented cervical spinal cord deformation secondary to cervical spinal stenosis. CLINICAL FEATURES: A 70-year-old special education teacher had neck pain, headaches, and burning paresthesia on the entire left side of her body. These symptoms developed within hours of being injured in a side-impact motor vehicle accident. Prior to her visit, she had been misdiagnosed with a cerebrovascular accident. INTERVENTION AND OUTCOMES: Additional diagnostic studies revealed that the patient was suffering from cervical spinal stenosis with spinal cord deformation. Two manipulative technique systems (Advanced Biostructural Therapy and Atlas Coccygeal Technique) unique to the chiropractic profession and based on the theory of relief of adverse mechanical neural tension were administered to the patient. This intervention provided complete relief of the patient's complaints. The patient remained symptom-free at long-term follow-up, 1 year postaccident. CONCLUSION: There is a paucity of published reports describing the treatment of cervical spinal stenosis through manipulative methods. Existing reports of the manipulative management of cervical spondylosis suggest that traditional manual therapy is ineffective or even contraindicated. This case reports the excellent short-term and long-term response of a 70-year-old patient with MRI-documented cervical spinal stenosis and spinal cord deformation to less traditional, uniquely chiropractic manipulative techniques. This appears to be the first case (reported in the indexed literature) that describes the successful amelioration of the symptoms of cervical spinal stenosis through chiropractic manipulation. More research into the less traditional chiropractic systems of spinal manipulation should be undertaken.
Twenty-two patients with subaxial acute traumatic central cord syndrome (CCS) without fracture or dislocation who underwent surgery between 1995 and 2002 were reviewed, retrospectively. There were 13 males and nine females ranging in age from 24 to 84 years (mean 61.2). Falls were the most common injury (68%), followed by motor vehicle accidents (32%). All patients had dynamic cervical lateral radiographs and magnetic resonance imaging (MRI). Cord compression was present in all cases and cervical instability in 11. Associated pathology included disc herniation in seven patients, cervical spondylosis (CS) in 11 and ossification of the posterior longitudinal ligament (OPLL) in four. Anterior decompression and fusion was performed in 12 patients with 1- or 2-level lesions. Posterior decompression and fusion was performed for multilevel lesions in 11 patients, including one patient who required re-operation. The interval between injury and surgery ranged from 1 to 37 days (mean 8.0). Postoperatively, all patients improved clinically. We conclude that surgical management of subaxial acute traumatic CCS without fracture or dislocation improved neurological status and prevented delayed neurological deterioration in our patients.