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We report a case of quadriplegia complicating ossification of posterior longitudinal ligament (OPLL) in a patient who was also found to have diffuse idiopathic skeletal hyperostosis (DISH). She also had osteomalacia (Vit. D deficiency) with secondary hyperparathyroidism. There could be a cause and effect relationship between the abnormal biochemistry and OPLL.
Tick-borne encephalitis (TBE) is an uncommon and potentially severe illness. TBE virus is transmitted to the humans by an infected tick and it spreads to the central nervous system determining various clinical pictures. A case in which TBE virus caused an encephalomyelitis with quadriplegia and respiratory insufficiency that persist one year after the diagnosis, is reported. Such a clinical manifestation of TBE has never been described in Italy till now.
A 14-year-old boy born at 39 gestational weeks with the birth weight of 3,250 g, had severe mental retardation and spastic quadriplegia of unknown etiology from early childhood. At 7 years of age, disturbance of night sleep appeared with several awakenings. Combined treatment with melatonin (MLT; 0.04 mg/kg) and flunitrazepam (FNZ; 0.025 mg/kg) was more effective than monotherapy with FNZ for the sleep disturbance. Difference of the effects of the combined therapy and the monotherapy for the sleep disturbance were assessed by polysomnographies (PSGs), consisted of EEG (C3, C4), bipolar EOG and surface EMG on the mentalis muscle. The PSGs were performed twice at 13 years 6 months old, and at 14 years 1 month old. In both trials, the number of awakening was fewer, total sleep time was longer, percent (%) stage I and II were lower and %REM sleep was larger in combined MLT and FNZ therapy than in FNZ monotherapy. The present study suggests that the combined MLT and FNZ therapy not only reduces awakening during sleep but also normalizes sleep architecture. This therapy is valuable because normalization of sleep is important for the developing brain as it reflects the normal functioning of the aminergic neurons of the midbrain and the brainstem which have important roles for morphological and functional development of the brain in immature brain.
This study was designed to test the hypothesis that non-ambulatory patients with spastic quadriplegia will have reduced bone mass which worsens with increasing age. Forty-eight patients (age 5 to 48 years, median age 15 years; 19 females and 29 males) were studied. Anticonvulsants were used in 29 patients (60.4%). Lumbar spine bone mineral density (LS-BMD) was markedly reduced compared with age-and sex-matched control individuals with a z score of -2.37 +/- 0.21. Twenty-eight (58%) had z scores of less than -2. A history of documented previous fracture was present in 19 patients (39%). Patients with a history of fracture had significantly lower (p = 0.05) LS-BMD z scores (-2.81 +/- 0.29) compared with those without a history of fracture (-2.11 +/- 0.26). Mean serum 25-OH vitamin D was 29.6 +/- 1.9ng/mL (normal 9 to 37.6ng/mL) with three patients having serum 25-OH vitamin D levels less than 15ng/mL. These findings indicate that BMD is markedly reduced in non-ambulatory children and adults with neuromuscular disease. Reductions in bone mass put them at greater risk for non-traumatic fractures.
A 36-year-old female was admitted to the intensive care unit after resuscitation diagnosed with diabetic ketoacidotic coma, which was the first manifestation of her diabetes mellitus. It may have been provoked by pulmonary or gastrointestinal coinfection. Five days following admission the patient regained consciousness and homeostasis returned to normal. One week after the stabilization of her cardiopulmonary state, weaning from the respirator turned out to be unsuccessful: flaccid tetraparesis developed with rapid muscle atrophy and absence of deep tendon reflexes. The sensory system and cranial nerves remained intact. Electrophysiological studies and muscle biopsy showed serious acute illness myopathy with mild demyelination owing probably to the latent diabetes. The course of acute quadriplegia was fluctuating and correlated mainly with the activity of the systemic inflammatory response syndrome mechanisms. Myopathy might have been aggravated by using high-dose glucocorticoid therapy. The patient's general condition improved quickly as a result of full recovery from sepsis, discontinuation of glucocorticoids and normoglicaemia maintained by subcutan insulin substitution. Eight months after admission almost full neuromuscular restitution was achieved showing the reversibility of this grave illness.
Clinicians need to know recovery of neurologic function in the upper extremities after traumatic quadriplegia to prognosticate function in self-care, to determine the effectiveness of various interventions, and to develop a comprehensive rehabilitation plan. This study was undertaken to determine the extent of recovery of key muscles of the arms in motor complete quadriplegic subjects. The hypothesis stated that patients with some motor power (grades 1.0 to 2.5/5) in muscles in the zone of partial preservation would recover at an earlier time and to a greater extent than those with no motor power (grade 0/5). One hundred fifty subjects, C4, C5, and C6 motor complete, were entered in the study within one week of injury from four centers. Serial muscle examinations of the biceps, wrist extensors, and triceps on the right and left sides were performed up to 24 months after spinal cord injury. The pattern of recovery in the key muscles of the 67 subjects with some motor power in the zone of partial preservation to grade 3/5 was significantly greater than the 83 subjects with no motor power (68% to 82% vs 14% to 36%, p less than .001) at three to six months postinjury. The plateau of the median manual muscle test score determined the extent of recovery and reached grade 4/5 in subjects with some motor power at three to six months. The pattern of recovery revealed more subjects with some motor power improved to grade 3/5 at all intervals earlier than those with no motor power (p less than .005).(ABSTRACT TRUNCATED AT 250 WORDS)
We report a case of hypokalemic flaccid quadriplegia with sudden respiratory arrest in a 38-year-old woman discovered to have distal renal tubular acidosis which lead to the diagnosis of primary Sjögren's syndrome. This case is compared to 8 similar cases previously described in the literature.
Fifty-one patients who had complete motor quadriplegia secondary to a fracture or dislocation of the cervical spine were managed by anterior cervical decompression and arthrodesis with iliac bone grafts between 1973 and 1983. In all patients, myelography demonstrated that displaced fragments of bone and disc were compressing the anterior aspect of the spinal cord. Decompression was performed in an attempt to gain further improvement of the motor-roots in the upper extremities and thereby to improve the ability of the patients to care for themselves. The average interval from the injury to the decompression was fifteen months (range, one month to eight years). Two patients died within two months after the operation, one had a respiratory arrest that resulted in brain damage one day after the operation, and two died from cardiovascular disease more than one year after the operation. The remaining forty-six patients were followed for an average of five years (range, two to thirteen years). Neurological improvement of at least two new functional motor-root levels was documented in seven patients and of one level, in eighteen. Increased motor strength by two or three grades was seen in six patients. Noteworthy motor improvement did not occur in the remaining twenty patients. The mean modified Barthel index (used to measure improvement in the ability to perform activities of daily living) increased from 17 to 33 (of a possible 100) points. Functionally important improvement of the caudad part of the cord occurred in only one patient. In one patient, neural injury, with loss of one motor-root level, occurred, with only partial improvement. At the latest follow-up examination, the result was poor in nine of eleven patients who had decompression eighteen months or more after the injury. Only two of these patients had any improvement in the Barthel index, and then of only 5 points each. The result also was poor in the five patients who were more than fifty-three years old; two had no improvement in the Barthel index, one improved by 5 points, and two died.
Lesions of the middle and lower part of the cervical spine during rheumatoid arthritis are less frequent than those of the cervico-occipital joint. They concern the discs on the one hand, the posterior inter-apophyseal joints, on the other hand. The authors report a case of rheumatic spondylodiscitis of C5-C6 with dislocation, complicated by quadriplegia, and review other published cases of rheumatoid lesions of the middle and lower cervical spine with neurological complications. The mechanisms of these lesions and their complications are then discussed.
UNLABELLED: This study proposes a new type of complicated form of hereditary spastic paraplegia (HSP) and some problems on a clinico-pathological classification of HSP. The present study includes three male and two female patients from two families (A and B). In the family A, four siblings (two males and two females) were affected. Spastic paraplegia developed as an initial symptom. In the family B, a man was affected with spastic paraplegia which had started at the age of eleven. His two half-sisters are normal. All parents in two pedigrees are healthy. The parents in the family B are first cousins. CLINICAL FEATURES: Their physical development was normal, but all of them showed mild mental retardation. Gait disturbance due to spastic paraplegia and mental deterioration developed at the age of high teens. At the age of high 20's, they became unable to walk, because of progressive spastic paraplegia and other complicated neurological impairments including pyramidal disorders in the upper limbs, generalized neurogenic muscular atrophy, sensory disturbance and bradykinesia. Some cases showed rigidity and/or spasticity. At the age of high 30's, they became bed-ridden, because of quadriplegia with generalized muscular atrophy. Three cases in the family A suffered from convulsions which started at the high 30's. Mild athetoid movement in the face and neck was observed in three cases in the family A. All patients became apathetic and indifferent at least by the age of 40. Laboratory findings and data: CT scan yielded brain atrophy and dilatation of the lateral ventricles, atrophy of the corpus callosum and hyperostosis of the cranium.(ABSTRACT TRUNCATED AT 250 WORDS)
The purpose of this study was to determine whether maximal oxygen uptake (VO2max) is protocol dependent during arm cycle ergometry (ACE) for quadriplegic males with spinal cord injuries (SCI). Twenty-four non-ambulatory subjects (aged 20-38 yr) with cervical SCI were divided into two groups based on wheelchair sports classification (IA group = 14; IB/IC group = 10). They underwent three different, continuous graded exercise tests spaced at least 1 wk apart on an electronically braked arm cycle ergometer. Following a 3-min, unloaded warm-up at 60 rpm, the work rate was increased 2, 4, or 6 W.min-1 for the IA group and 4, 6, or 8 W.min-1 for the IB/IC group. Ventilation and gas exchange were measured breath-by-breath with a SensorMedics 4400 computerized system. Repeated-measures ANOVA showed no significant difference among the three protocols for VO2max in the IA group (P greater than 0.05). The mean (+/- SD) VO2max values (ml.kg-1.min-1) were 10.8 (+/- 3.4), 11.0 (+/- 2.7), and 10.2 (+/- 2.9) for the 2, 4, and 6 W.min-1 protocols, respectively. In contrast, the IB/IC group showed a significant difference among the protocols for VO2max (P less than 0.05). The mean (+/- SD) VO2max values (ml.kg-1.min-1) were 16.8 (+/- 4.5), 15.3 (+/- 4.3), and 14.6 (+/- 4.3) for 4, 6, and 8 W.min-1, respectively. Post hoc analysis revealed a difference between the 4 and 8 W.min-1 protocols. Our results suggest that graded exercise testing of SCI persons with quadriplegia, using ACE, should employ work rate increments between 2-6 W.min-1 and that work rate increments of 8 W.min-1 or greater will underestimate VO2max.
We present the first reported case of Candida pyelonephritis in a spinal cord injured patient. In addition to multiple courses of empiric antibiotics, the neurogenic bladder and alteration in cell-mediated immunity found in spinal cord injured patients may have increased this patient's susceptibility to fungal disease. A 50-year-old patient with C5 motor functional quadriplegia developed Candid albicans pyelonephritis while undergoing rehabilitation. The patient had several surgical procedures and multiple courses of antibiotic therapy during acute hospitalization. He continued to have a hectic fever curve, leukocytosis with increased band forms, lethargy, and progressive uremia during rehabilitation. Successful investigation of the patient's condition included assessment of serologic tests for Candida precipitin antigen; multiple blood and urine cultures; exclusion of other causes of hectic fever; abdominal computerized tomogram, which revealed a left kidney hypodensity with irregular margins; and a retrograde pyelogram, which demonstrated multiple renal pelvic-filling defects. Cystoscopically placed ureteral stents, which relieved the genitourinary obstruction, drained gross pus from which Candida albicans was cultured; the patient was treated with amphotericin B and showed clinical improvement. Pathogenesis, presentation, diagnosis, and treatment of Candida pyelonephritis are reviewed.
Superior mesenteric artery syndrome is a condition in which the third portion of the duodenum is intermittently compressed by the overlying superior mesenteric artery, resulting in gastrointestinal obstruction. Predisposing factors include rapid weight loss, prolonged supine positioning, and using a spinal orthosis, all of which are common among acute traumatic quadriplegic patients. This paper presents three patients, aged 24, 16, and 20 years, with traumatic quadriplegia treated with supine positioning and cervical orthoses, who had postprandial nausea and emesis, bloating, and abdominal pain during rehabilitation. Upper gastrointestinal radiographic series demonstrated abrupt duodenal obstruction to barium flow in all three patients. Two of the patients had complete relief of symptoms with conservative management, and one required surgical duodenojejunostomy. Enhanced awareness of this condition may result in improved recognition of this disease as a cause of persistent, unexplained gastrointestinal disturbances in quadriplegic persons, thereby optimizing its treatment and reducing its potential morbidity.
Twenty-nine C4 to C7 motor-complete spinal cord injured subjects were evaluated to determine whether the manual muscle test (MMT) performed within 24 hours of injury or at 72 hours after injury better predicts short-term functional muscle recovery. A single muscle in the zone of injury that had a muscle grade between 1 and 3, inclusive, was evaluated serially in each patient from admission to three months. Increase in muscle strength to grade 4 or better was defined as success. Outcomes were evaluated by the Fisher exact test. Using the less-than-24-hour MMT, 12 of 17 subjects with grade 3 muscle strength vs four of 12 subjects with grade 1 or 2 muscle strength achieved success (p greater than .05). Using the 72-hour MMT, all 11 subjects with grade 3 muscle strength vs five of the 18 subjects with less than grade 3 muscle strength achieved success (p less than .001). Short-term functional muscle recovery in the zone of injury in cervical motor-complete quadriplegia is better predicted by the 72-hour MMT than by the less-than-24-hour MMT.
Records and radiographs of 90 patients suffering complete or motor-complete quadriplegia and treated surgically were reviewed. There were 76 men or boys and 14 women or girls; ages ranged from 15 to 75. Eighty-one were complete quadriplegics and nine had some degree of sensory preservation. After surgery 30 were unchanged, 47 obtained root recovery, and 13 recovered cord function, including two who became ambulatory. Fifty-three of the 74 (71%) patients undergoing decompressive procedures showed neurological improvement while seven of the 16 (49%) patients with fusion and no root decompression had improvement (p less than 0.05). All 26 patients with dislocations underwent closed or open reduction as part of their operative procedures; this did not appear to improve the likelihood of nerve root recovery. Since independence and quality of life may be improved by cord and root recovery, decompression of all neural structures should be considered in cervical spinal cord injury.
The purpose of this article is to define as a distinct clinical entity, the syndrome of cervical spinal cord neurapraxia with transient quadriplegia. Sensory changes include burning pain, numbness, tingling, or loss of sensation, whereas motor changes consist of weakness or complete paralysis. The phenomenon of cervical spinal cord neurapraxia occurs in individuals with (1) developmental cervical spinal stenosis, (2) congenital fusions, (3) cervical instability, or (4) intervertebral disc protrusions when associated with a decrease in the anteroposterior diameter of the spinal canal. There is no evidence that the occurrence of cervical spinal cord neurapraxia predisposes an individual to permanent neurologic injury. However, patients with this syndrome and associated with cervical spine instability or acute or chronic degenerative changes should be precluded from further participation in contact sports. Those with developmental spinal stenosis or spinal stenosis associated with congenital abnormalities should be treated on an individual basis.
A new, computerized neuromuscular stimulation system was applied to the upper limbs of two patients with complete quadriplegia below the C4 level. The stimulation-generated movements were integrated and augmented by residual, voluntary shoulder girdle movements and mechanical splinting. Up to 12 muscles were stimulated individually with high-resolution surface electrodes; coordination and control of the stimulation was effected by microcomputer. Simple vocal commands to the computer triggered preprogrammed hand prehensions, arm motion, and other functions, giving the patient complete control over the system. In pilot clinical trials of six weeks, writing, eating, and drinking, including picking up and replacing the pen or cup, were achieved.
The diagnosis of septic discitis, or vertebral osteomyelitis, in able-bodied adults is difficult to make and often delayed. Here, the clinical findings and events leading to diagnosis and complications of septic discitis occurring in a patient with quadriplegia after urinary tract manipulation are presented. The diagnosis was delayed nine weeks from onset of fever (11 weeks after urologic manipulation), despite a variety of radiologic procedures and repeated blood and urine cultures. The patient's symptoms recurred five weeks after IV antibiotics were discontinued and while he was still taking oral cephalexin. He underwent open debridement and further IV and IM tobramycin, recovering without complication. The patient died five months later, reportedly of bowel obstruction and pneumonia. A review of the literature revealed only one other case report, of a patient with paraplegia, who also presented a diagnostic problem and died one year after diagnosis.