1/29. Spinal arachnoid cyst with weakness in the limbs and abdominal pain.A 7-year-old male admitted with neck rigidity, severe pain in the abdomen, and progressive weakness in the lower limbs was diagnosed as having a spinal intramedullary arachnoid cyst. There was a dramatic and immediate recovery after fenestration of the cyst.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/29. Rigid spine syndrome. Two case-reports.Rigid spine syndrome is characterized by massive spinal rigidity, usually most marked in the cervical region. Stiffness of the peripheral joints is sometimes present. We report two cases. Patient 1 was a 12-year-old boy diagnosed at three years of age with Duchenne's muscular dystrophy because of delayed onset of walking. contracture of the Achilles tendons, flexion contracture of the elbows, and loss of motion of the cervical spine were the main findings during the current evaluation. Radiographs of the affected joints were normal. An electrocardiogram showed an incomplete left bundle branch block. Muscle enzyme activities were moderately elevated. A myopathic pattern was seen on the electromyogram. A muscle biopsy showed muscle fiber atrophy with peri- and endomysial fibrosis. Patient 2 was a 39-year-old man with a five-year history of isolated rigidity of the cervical spine thought to be due to a spondylarthropathy. Extension was the only movement possible at the cervical spine. The peripheral joints showed no motion range limitation. Findings were normal from radiographs of the spine and sacroiliac joints, an erythrocyte sedimentation rate determination, an electromyogram, and muscle enzyme activity assays. A muscle biopsy showed muscle fiber atrophy with peri- and endomysial fibrosis. DISCUSSION: Rigid spine syndrome is rare in rheumatological practice and can simulate a number of other muscle and joint diseases. Peri- and endomysial fibrosis may be strongly suggestive, although nonpathognomonic. Involvement of the heart governs the prognosis.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
3/29. encephalomyelitis with rigidity complicating human immunodeficiency virus infection.A 34-year-old man with human immunodeficiency virus type 1 (hiv-1) presented with axial rigidity, painful spasms, and delayed hemiparesis and dementia. cerebrospinal fluid analysis showed no antiglutamic acid dehydrogenase antibodies but viral genome from Epstein-Barr virus was detected by polymerase chain reaction. Clinical features and possible viral aetiology of progressive encephalomyelitis with rigidity are briefly discussed.copyright 2001 Movement Disorder Society- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
4/29. pseudotumor cerebri manifesting as stiff neck and torticollis.Stiff neck and torticollis are significant signs of neurologic disease. Nuchal rigidity is often associated with meningitis, subarachnoid hemorrhage, and posterior fossa tumor. torticollis may be encountered in inflammatory disorders, such as cervical lymphadenitis, or it can be a sign of spinal cord syrinx or of central nervous system neoplasm. We report on three prepubertal children in whom stiff neck and torticollis were the presenting signs of pseudotumor cerebri. In all, the removal of 6-7 mL of cerebrospinal fluid led to prompt relief of symptoms and signs. We suggest that in the presence of unexplained stiff neck or torticollis in children, the optic discs should be examined to exclude pseudotumor cerebri.- - - - - - - - - - ranking = 2keywords = spinal (Clic here for more details about this article) |
5/29. Progressive encephalomyelitis with rigidity: a paraneoplastic presentation of oat cell carcinoma of the lung. Case report.Progressive encephalomyelitis with rigidity and myoclonus (PEWR) is a rare neurological disorder, characterised by muscular rigidity, painful spasms, myoclonus, and evidence of brain stem and spinal cord involvement. A 73-year-old white man was admitted with a 10-day history of painful muscle spasms and continuous muscle rigidity on his left lower limb. He had involuntary spasms on his legs and developed encephalopathy with cranial nerves signs and long tract spinal cord symptomatology. Brain CT scan and spinal MRI were normal. The CSF showed lymphocytic pleocytosis and no other abnormalities. EMG showed involuntary muscle activity with 2-6 seconds of duration, interval of 30-50 ms and a frequency of 2/second in the left lower limb. Anti-GAD antibodies were detected in the blood. We detected radiological signs of lung cancer during the follow-up, which proved to be an oat cell carcinoma. The patient died two weeks after the diagnosis of the cancer.- - - - - - - - - - ranking = 3keywords = spinal (Clic here for more details about this article) |
6/29. Downbeating nystagmus and muscle spasms in a patient with glutamic-acid decarboxylase antibodies.PURPOSE: To report the ophthalmic findings and response to treatment in a patient with glutamic-acid decarboxylase antibodies. DESIGN: Case report. methods: A 55-year-old woman developed progressive, painful, low back muscle spasms, vertical diplopia, downbeating nystagmus, and asymmetric appendicular ataxia. RESULTS: Downbeating nystagmus was present in primary gaze with an alternating skew deviation in lateral gaze. serum and cerebrospinal fluid GAD antibodies were detected. Treatment with diazepam led to resolution of spasticity, whereas repeated courses of intravenous immunoglobulin improved cerebellar function, including appendicular ataxia and downbeating nystagmus. CONCLUSIONS: patients with GAD antibodies may have elements of both stiff-person syndrome (muscle rigidity and spasms) and prominent cerebellar dysfunction. Treatment with diazepam rapidly improved Stiff-person symptoms, whereas IVIg was partially effective at the early stage of cerebellar dysfunction.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
7/29. Neonatal rigid-akinetic syndrome and dentato-olivary dysplasia.This report describes a male infant who presented since birth with rigidity and hypokinesia. Severe developmental delay, episodic central hypoventilation, and drug-resistant epilepsy progressively added to the extrapyramidal signs in the following months and led to the patient's death at 10 months of age. Neuroradiologic and neurometabolic evaluations were negative. Normal cerebrospinal metabolites excluded a defect in dopamine metabolism, and treatment with levodopa failed to improve his motor symptoms. Neuropathologic findings demonstrated dentato-olivary dysplasia. While isolated dentato-olivary dysplasia has been described in a few cases of Ohtahara syndrome, to our knowledge, the association with infantile parkinsonism has not been previously reported.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
8/29. Sudden death and paroxysmal autonomic dysfunction in stiff-man syndrome.Two women with typical stiff-man syndrome (SMS) developed increasingly frequent attacks of muscle spasms with severe paroxysmal autonomic dysfunctions such as transient hyperpyrexia, diaphoresis, tachypnea, tachycardia, pupillary dilation, and arterial hypertension. autoantibodies to GABA-ergic neurons were identified in the serum of both patients and in the cerebrospinal fluid of one. Both died suddenly and unexpectedly. General autopsy did not reveal the cause of death. Neuropathological studies revealed perivascular gliosis in the spinal cord and brain stem of one patient and lymphocytic perivascular infiltration in the spinal cord, brain stem, and basal ganglia of the other. The occurrence of a chronic inflammatory reaction in one of the two patients supports the idea that an autoimmune disease against GABA-ergic neurons may be involved in SMS. A review of the literature indicates that functional impairment in SMS is severe and prognosis is unpredictable because of the potential for sudden and unexpected death. Both muscular abnormalities and autonomic dysfunctions may result from autoimmunity directed against GABA-ergic neurons.- - - - - - - - - - ranking = 3keywords = spinal (Clic here for more details about this article) |
9/29. Selenoprotein N muscular dystrophy: differential diagnosis for early-onset limited mobility of the spine.Early spinal rigidity is a nonspecific feature reported in diseases such as neuromuscular and central movement disorders. We present a male patient with rigid spine muscular dystrophy caused by newly identified compound heterozygote mutations of the selenoprotein N gene and discuss this disease as a possible differential diagnosis for early-onset reduced spine mobility. Rigid spine muscular dystrophy is a rare myopathy presenting in childhood with a typical combination of stable or slowly progressive mild to moderate muscle weakness, limitation in flexion of the spine, and progressive restrictive ventilatory disorder. The clinical features of our patient include early-onset rigidity of his spine, scoliosis, mild muscular weakness predominantly of neck and trunk flexors, and restrictive ventilatory disorder. biopsy of the biceps muscle revealed nonspecific myopathic changes, and molecular analysis confirmed the diagnosis of rigid spine muscular dystrophy. Thus, neuromuscular diseases such as muscular dystrophy must be considered in all patients presenting with early spinal rigidity, and genetic determination is a possible way to determine the diagnosis.- - - - - - - - - - ranking = 2keywords = spinal (Clic here for more details about this article) |
10/29. Rigidity and painful muscle spasms in a patient with probable myelitis.We describe a case of continuous motor unit potential (MUP) activity of central origin (unlike stiff man syndrome and progressive encephalomyelitis) characterized clinically by rigidity, painful muscle spasms, abnormal postures and spinal myoclonus. The topography of the manifestations, the subacute and benign course, the presence of stable sequels 2 years after onset and a searching process of differential diagnosis lead us to attribute the condition to an inflammation of the cord, which makes the case of particular clinical interest.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
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