Hypokalemic quadriparesis associated with renal tubular acidosis in a patient with Sjögren's syndrome

Sj?gren's syndrome is an autoimmune exocrinopathy that involves both glandular and extra-glandular systems. We report a 25-year-old woman who had rapidly progressive quadriparesis. Biochemical investigations showed severe hypokalemia with hyperchloremic metabolic acidosis diagnosed as distal renal tubular acidosis. Salivary gland biopsy revealed Sj?gren's syndrome as the underlying cause. She recovered following from quadriparesis potassium and alkali replacement.     

Transient Quadriparesis following neck injury: presentation of a Chiari 1 malformation

We present the case of a 17-year-old male who presented with quadriparesis following an acute neck injury from a tackle in a football match. MRI scans of cervical spine showed a Type-1 Chiari malformation. The quadriparesis resolved over 24 h. We also present an overview of the differing presenting features of this condition from the literature. Whether these patients have to be advised against returning to contact sports is debatable.     

Dysplasia of the odontoid process in Morquio's syndrome causing quadriparesis.

Eleven patients with documented Morquio's syndrome who had dysplasia of the odontoid process and resulting atlanto-axial instability were reviewed. They were found to be at risk for acute traumatic quadriparesis, chronic myelopathy of a variable and often rapid rate of progression, and sudden death by respiratory arrest. The evidence suggests that early prophylactic posterior cervical fusion is indicated for patients with this malformation and that once quadriparesis is established, recovery of function is limited.     

Successful Liver Transplantation in a Patient With Quadriparesis: A Case Report

Major abdominal surgeries, including liver transplantation, are considered high-risk procedures for patients with respiratory muscle dysfunction, such as patients with quadriparesis, due to possible fatal postoperative pulmonary complications. We report on a 57-year-old male patient with longstanding quadriparesis due to fifth cervical spine injury from a traffic accident who suffered from decompensated liver cirrhosis related to hepatitis C infection and hepatocellular carcinoma. A preoperative pulmonary function test showed forced expiratory volume in 1 minute (FEV₁) 1.06 L, which was a risk for pulmonary complications. The patient required respiratory training. Cadaveric liver transplantation was performed successfully without surgical complications. The patient was extubated on the fourth day after surgery and initially did well. However, on the eighth postoperative day, an episode of status epilepticus from metabolic derangement developed. After controlling seizure with anticonvulsive medication and sedation, the patient was reintubated due to hypoventilation. Chest radiograph showed upper lung atelectasis. Due to this complication, tracheostomy was performed. The patient's condition gradually improved. He was ultimately discharged on the 45th postoperative day. Two months after the transplantation, liver functions were normal and the patient could breathe spontaneously without tracheostomy and had good quality of life. In conclusion, this is, to our knowledge, the first report of liver transplantation in a patient with quadriparesis. It shows that even with a very high risk for postoperative pulmonary complications, liver transplantation can be performed successfully with careful patient selection and effective respiratory care.     

Complete recovery of severe quadriparesis caused by stab wound at the craniocervical junction

Non-missile penetrating spinal cord injuries are uncommon, and involvement of the craniocervical junction is even less frequent. The author reports a case of 42-year-old male who presented with quadriparesis immediately following stab injury inflicted with a kitchen knife to the back of his neck. The knife was retained in the patients neck. Neurological examination revealed spastic paraplegia and severe weakness of the left upper limb and the right-hand grip, and sensory disturbance from C2 and downwards. In addition to these symptoms, cerebrospinal fluid (CSF) was leaking from the wound. Computerised tomography (CT) scan showed the blade passed through the spinal canal and its tip reached the odontoid peg. After retrieval of the knife, his quadriparesis recovered. The management and outcome of the patient are described.     

Cervicomedullary junction decompression in a case of Marshall-Smith syndrome. Case report

The case is reported of a 2-year-old boy born with Marshall-Smith syndrome who had difficulty in swallowing and who exhibited spasticity and quadriparesis due to compression of the medulla and cervical spine. This is the first child with this rare condition reported to have brain-stem compression from bone abnormalities at the craniovertebral junction and who has required surgery.     

Scarring around cervical epidural stimulating electrode

Scarring around the electrically stimulating electrodes has been of concern since dorsal column stimulation was introduced. This concern resurfaced in the days of cerebellar electrodes and, with the advent of epidural stimulating techniques for the control of pain and spasticity, it again arises as a potential problem. We present a patient who underwent the placement of a C-2-C-4 electrode to treat torticollis; 3 months later, a mild spastic quadriparesis developed and the stimulation became ineffective. At reexploration, dense scar surrounded the electrode and confined the cervical spinal cord. With microdissection techniques, the scar was removed from the dura mater and the dura began to pulsate freely. The quadriparesis reversed. Examination of the scar tissue microscopically showed linearly arrayed fibroblastic nuclei, and we are uncertain whether the exuberant fibroblastic response is a response to electrical stimulation, the materials used in the electrode, or some technical aspects of the operation.     

Cervical myelopathy with ankylosing hyperostosis of the spine

We report the case of a man with ankylosing hyperostosis of the spine who developed spastic quadriparesis due to ossification of the posterior longitudinal ligament. The related literature is discussed.     

Destructive spondyloarthropathy mimicking spondylitis in long-term hemodialysis patients

A 63-year-old man with end-stage renal disease (ESRD) who had been undergoing hemodialysis for 18 years suffered persistent neck pain, progressive quadriparesis, and a deteriorating ataxic gait during the 6 months before admission. A sudden onset of aggravating quadriparesis and an inability to ambulate occurred during his trip to Sydney, Australia, 1 week before this admission. Vertebral tuberculosis osteomyelitis of the C5/6 segment was considered and treated in a hospital there. Findings from cervical magnetic resonance imaging (MRI; low signal intensity on both T1- and T2-weighted images) were diagnostic of destructive spondyloarthropathy (DSA) and distinguishable from spinal osteomyelitis preoperatively. Amyloid masses, mainly composed of B-2 microglobulin, filled in disc and paradiscal ligaments, with adjacent endplate destruction by cytokine-mediated reactive inflammation, and appeared to be mostly related to the pathogenesis of DSA. The cervical spine, especially C5/6, is the most common site of DSA. Spinal instability and neurologic compression cause the clinical symptoms and signs. Adequate decompression and successful cervical fusion ensure the best therapeutic results. Received: 8 February 1999     

Cervical instability in an achondroplastic infant

Cervical spine instability is a rare finding in achondroplasia. We present the previously unreported case of C2-C3 subluxation producing progressive quadriparesis in an achondroplastic infant. Operative treatment with appropriate fusion was performed, and an excellent result was obtained.     

Spinal shock in spontaneous cervical spinal epidural haematoma

Summary. A young man presented with quadriparesis and spinal shock because of a spontaneous cervical spinal epidural haematoma was reported. Immediate MRI diagnosis followed by emergency decompression with six hours of presentation resulted in complete recovery.     

Synovial cyst at the craniovertebral junction

A case of synovial cyst of the upper cervical spine that resulted in spastic quadriparesis and sensory loss is reported. Radiographic evaluation included a computed tomography scan after myelography and a magnetic resonance imaging scan. The synovial cyst was removed by a laminectomy at C-1 and C-2, combined with a foramen magnum craniectomy. The patient had a good recovery.     

Ureterosygmoidostomy-associated quadriparesis, non-traumatic rhabdomyolysis, and tetany in an adult

We report an adult case of ureterosigmoidostomy-associated quadriparesis, rhabdomyolysis, and tetany which may be the first such case in the literature. A 32-year-old female patient was brought to the emergency room of our hospital, having been unable to walk or use her arms for 24 h. Neurological examination revealed quadriparesis. She had severe hypokalemia (1.27 mmol/l) and metabolic acidosis (pH = 7.05). Creatine kinase value was 2,590 U/l on the third day. She received intensive therapy to correct the hypokalemia and acidosis. On the third day of hospitalization tetany was detected in her upper extremities. The patient regained full muscle power after intravenous potassium chloride, bicarbonate, and calcium replacement therapy.     

Injury to the spinal cord without radiological abnormality (SCIWORA) in adults

Injury to the spinal cord without radiological abnormality often occurs in the skeletally immature cervical and thoracic spine. We describe four adult patients with this diagnosis involving the cervical spine with resultant quadriparesis. The relevant literature is reviewed. The implications for initial management of the injury, the role of MRI and the need for a high index of suspicion are highlighted.     

Superior mesenteric artery syndrome: An unusual cause of intestinal obstruction in brain-injured children

Superior mesenteric artery syndrome is a rare cause of upper intestinal obstruction in both adults and children. Sixteen children with severe traumatic brain injury and spastic quadriparesis developed small intestinal obstruction while undergoing a rehabilitation programme between 1981 and 1990. Five patients met the roentgenographic diagnostic criteria. The presenting symptom was post-prandial bilious vomiting. The mean age was 13 (10-16) years. The mean time elapsed from injury to diagnosis was 53 days and from rehabilitation admission to diagnosis 22 days. The mean delay in diagnosis after onset of symptoms was 4 days. All patients were of disproportionately lower body weight in relation to height, with a mean weight loss of 7 kg. The mean percentile for weight was 18 and height 58, with a difference of 30 between height and weight percentiles. The patients were receiving nasogastric or gastrostomy tube feedings at the onset of the symptoms. All patients were treated non-surgically with gastric aspiration, nasojejunal or gastrojejunal feeding by passing a feeding tube distal to obstruction. No patient required intravenous hyperalimentation. There was no recurrence in any patient during the follow-up period of 1-5 years. Though rare, superior mesenteric artery syndrome can develop in brain-injured children with spastic quadriparesis, prolonged recumbency and recent weight loss. Increased awareness of occurrence of this condition and timely management will decrease morbidity and complications that may interfere with recovery.     

Disappearance of quadriparesis due to a huge cervicothoracic aneurysmal bone cyst

A young man who had suffered from rheumatoid arthritis developed a huge cervicothoracic aneurysmal bone cyst and progressive quadriparesis. Complete recovery occurred after "incomplete therapy," which consisted of an open biopsy and a small dose of irradiation. This tumor was observed and characterized as a blood-filled cyst excavated from the bone. Because of the controversy in the literature over the benefit of surgery vs. irradiation, this report emphasizes the fact that even "partial" treatment may beneficially affect this tumor.     

Eosinophilic granuloma of the cervicothoracic junction. Case report

A case is reported of eosinophilic granuloma at the cervicothoracic junction presenting with profound quadriparesis preoperatively. The patient underwent excision via an anterior approach, with splitting of the sternum to gain access to the T-1 vertebra. Postoperatively, he has had an excellent return of function.     

Spinal cord vasculitis presenting as a spinal cord tumor in a heroin addict. Case report

Progressive quadriparesis in a heroin addict was diagnosed on the basis of clinical and myelographic findings as an intramedullary cervical spinal cord tumor. A biopsy of the enlarged cervical spinal cord showed myelomalacia, vasculitis, and many doubly refractile bodies in the parenchyma and vessel walls.     

Marginal indications for the Magerl method of fixation of C1-C2 (case report)

The authors present a 4-year old girl who had a car accident as a passenger and hurt her head, chest and limbs as well as upper cervical spine. The patient with multiple injuries was taken to the FTN Centre of Children's traumatology, Prague. Here the basic vital functions were ensured and a diagnosis was made of contusion of the brain with quadriparesis and inhibition of the respiratory centre, contusion of the chest, epiphysiolysis of the distal femur and later also instability of C1-C2. A censor for measuring or intracranial pressure was immediately inserted with a subsequent reduction of the distal femur and elastic fixation. External lumbar drainage was performed in the next week instability of C1-C2 was not found out and therefore not treated. Three months after the injury a ventriculoperitoneal shunt for intracranial hypertension was inserted. MRI showed stenosis in the region of occipitocervical passage and dorsal decompression of craniocervical passage was performed which consisted in the removal of the posterior arch of C1 and a significant extension of foramen magnun dorsally and laterally to both sides. Due to persisting ligamentous instability of C1-C2 with a spastic quadriparesis and inhibition of the respiratory centre a surgical atlantoaxial stabilization was indicated, i.e. causal treatment of instability. Seven months after the injury Magerl fixation of C1-C2 was performed by 2.7 mm titanicum screws (Synthes). Preoperative stability of C1-C2 in the reduced position was satisfactory but with regard to iatrogenic instability the C0-C1 fixation was combined with occipitocervical fussion by Ransford loop extending over C0-C3. Further, the triangular flap of periost was overturned from the external occipital protuberance to C3 and all this was bridged by cortical cancellous bone grafts from iliac crest. After two months a check simple and functional x-ray examination showed a stable fusion of C0-C2. The neurological finding remained the same even after one year, i.e. a severe quadriparesis with the inhibition of the respiratory centre requiring artificial lung ventilation.     

Whole cerebrospinal axis infection after lumbar epidural injection: a case report

Purpose

Lumbar epidural injection is a popular treatment for degenerative lumbar disease. Although post-procedural epidural infection is rare, meningitis and epidural abscess are life-threatening conditions, and need additional medical and surgical intervention. The purpose of this article is to report a patient with fatal whole cerebrospinal axis infection after lumbar epidural injections.

Methods

A 64-year-old female patient presented with septic shock and quadriparesis. In the past, this patient had received lumbar epidural injections several times for degenerative spondylolisthesis at L4-5 in another hospital. The magnetic resonance imaging showed epidural abscess, a compressed dura and spinal cord from C1 to S2, and cerebral meningitis. We performed laminectomies and removal of the abscesses. Her mental status was diminished to a deep, drowsy state after three postoperative weeks. Brain computed tomography scans revealed hydrocephalus. Therefore, ventriculoperitoneal shunting was performed.

Results

Methicillin resistant Staphylococcus epidermidis was cultured from blood samples. The patient’s infection was completely controlled and her mental status improved to alert; however, her quadriparesis remained.

Conclusions

Although lumbar epidural injection is an effective procedure to treat lumbar radicular pain, this procedure can induce fatal complications such as sepsis and epidural abscess.

Level of evidence

5.