1/25. Detection of anterior horn lesions by MRI in central European tick-borne encephalomyelitis.We report a case of central European tick-borne encephalitis with cervical myelitis presenting clinically as a lower motor neuron syndrome of the upper limbs with proximal asymmetrical pareses and atrophies. There were no sensory deficits nor signs of lesions of the spinal pathways or signs of encephalitis or meningitis. The affected motor fibers of the upper limbs were electrically inexcitable, but sensory findings were normal. electromyography of the paralyzed muscles revealed pathological denervation activity without voluntary activation. The initial magnetic resonance imaging (MRI) showed a large hyperdense lesion in the anterior part of the cervical cord from C3 to T1. Despite the fact that MRI changes disappeared completely within 6 weeks the patient showed only little improvement in the paralyzed muscles after 6 months. To our knowledge, these MRI changes in patients with tick-borne encephalitis, consistent with an isolated anterior horn lesion, have never been reported previously. The course may have been aggravated by an initial antibiotic treatment with cephalosporins.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
2/25. 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 = 554421.98972996keywords = rigidity (Clic here for more details about this article) |
3/25. Progressive encephalomyelitis with rigidity presenting as a stiff-person syndrome.diagnosis criteria of stiff-person syndrome (SPS) include progressive, fluctuating muscular rigidity and spasms with normal neurological examination. The presence of unusual features such as prominent limb rigidity with segmental signs and contracture, evidence of brainstem dysfunction, profound autonomic disturbances, CSF pleiocytosis or MRI abnormalities in patients with SPS presentation allows to classify these patients as progressive encephalomyelitis with rigidity (PER). We report a 50 year-old woman suffering from severe painful spasms of abdominal wall and limb muscles. Neurological examination showed pyramidal signs. EMG disclosed continuous muscle activity with superimposed discharges. Treatment with high doses of diazepam and baclofen led to moderate improvement of generalised stiffness. However, the right arm became more rigid with oedema and vasomotor changes. Subsequently, bilateral nystagmus and internuclear opthalmplegia appeared. There was mild CSF pleiocytosis. Associated auto-immune thyroiditis was found with positive anti-microsome antibodies and decreased thyroid hormones. Search for profound neoplasm was negative. The patient had three subacute bouts then she improved with methylprednisolone. The initial clinical presentation mimicking a SPS with subsequent diffuse involvement of the central nervous system and a striking localisation of a severe rigidity to one arm allowed to suspect the diagnosis of PER. The relationship between SPS and PER remains unclear because of the rarity of these disorders. The observation reported in this paper gives evidence that both the disorders are probably two clinical presentations of the same pathogenic process.- - - - - - - - - - ranking = 739230.31963995keywords = rigidity, muscle (Clic here for more details about this article) |
4/25. Pathological evidence of encephalomyelitis in the stiff man syndrome with anti-GAD antibodies.We report the case of a 57 year old woman with typical clinical features of the stiff man syndrome (SMS) and antibodies to glutamic acid decarboxylase (antiGADAb), who developed a supranuclear gaze palsy, ileus and died of bronchopneumonia eight years after the onset of illness. Post mortem examination revealed perivascular lymphocyte cuffing throughout the cerebral hemispheres, brainstem and spinal cord and neuronal loss in medial anterior horns of the cervical spinal cord. These findings support the notion that the SMS and progressive encephalomyelitis with rigidity (PER) form a clinical and pathologic continuum.- - - - - - - - - - ranking = 92403.664954994keywords = rigidity (Clic here for more details about this article) |
5/25. Pathological and virological assessment of acute HTLV-I-associated myelopathy complicated with encephalopathy and systemic inflammation.HTLV-I-associated myelopathy, also known as tropical spastic paraparesis (HAM/TSP), is a chronic inflammatory disease of the spinal cord. Acute cases are uncommon. We report the case of a 41-year-old woman with acute HAM/TSP complicated with encephalitis, an intense inflammatory reaction of the nervous system and lymphocytic infiltration of skeletal muscles, liver, salivary, adrenal and pituitary glands. The immunohistochemical studies of the lymphocytes surrounding blood vessels showed both B- and t-lymphocytes, in similar proportion, with both CD4- and CD8-positive cells. In addition, many perivascular and scattered macrophages were observed. adult T-cell leukemia/lymphoma (ATL) was ruled out. The marrow aspirate was normal. Serial cerebrospinal fluid (CSF) analysis showed presence of htlv-i antibodies, but without intrathecal synthesis of specific antibodies. Determination of HTLV-I viral loads demonstrated increased levels in the CSF relative to the peripheral blood and may be associated with widespread inflammation. The pathological and immunological findings may help understand the role of immune-reactive cells in the pathogenesis of HTLV-I-associated myelopathy.- - - - - - - - - - ranking = 0.5keywords = muscle (Clic here for more details about this article) |
6/25. 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 = 646827.15468496keywords = rigidity, muscle (Clic here for more details about this article) |
7/25. An autopsy case of mitochondrial encephalomyopathy with prominent degeneration in olivo-ponto-cerebellar system.We describe a sporadic case of adult-onset, complex I deficiency mitochondrial encephalomyopathy (MEM), the clinical and pathological features of which failed to fit any of the known subgroups of MEM, such as kearns-sayre syndrome, mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes or myoclonus epilepsy with ragged-red fibers. Clinically, this patient had only progressive cerebellar ataxia, generalized muscle weakness and hearing loss. The principal finding at autopsy was degeneration of the olivo-ponto-cerebellar system. This case suggests that mitochondrial disease could underlie some cases of olivo-ponto-cerebellar atrophy.- - - - - - - - - - ranking = 0.5keywords = muscle (Clic here for more details about this article) |
8/25. A case of progressive encephalomyelitis with rigidity and positive antiglutamic acid decarboxylase antibodies [corrected]A 50 year old woman developed progressive encephalomyelitis with rigidity over a three year period. Her CSF contained oligoclonal bands and both her serum and CSF contained antibodies directed against GABA-ergic synapses (antiglutamic acid decarboxylase [corrected] antibodies). These antibodies have recently been described in cases of stiff man syndrome. Both disorders may be part of a clinical spectrum that has an underlying autoimmune basis.- - - - - - - - - - ranking = 462018.32477497keywords = rigidity (Clic here for more details about this article) |
9/25. encephalitis lethargica-like illness in a five-year-old.A five-year-old boy presented with an encephalitis lethargica-like illness, characterised by somnolence, mutism and Parkinsonian rigidity two weeks after an acute exanthem. Investigation revealed increased serum measles antibody titre. He made a complete recovery. encephalitis lethargica associated with Parkinsonism in childhood is rare and usually carries a poor prognosis.- - - - - - - - - - ranking = 92403.664954994keywords = rigidity (Clic here for more details about this article) |
10/25. Progressive encephalomyelitis with rigidity: a case report with magnetic resonance imaging findings.A 52 year old woman presented with pain in the back and upper limbs and progressive weakness and sensory impairment of the upper and lower limbs. She developed frequent episodes of severe generalised muscle spasms associated with piloerection and hyperventilation. Nerve conduction studies and electromyography were normal. MRI demonstrated a lesion of the cervical spinal cord and lower brainstem. A biopsy of the cord revealed perivascular cuffing with mononuclear cells and inflammatory changes in the parenchyma, with increased numbers of microglia, reactive astrocytes and plasma cells. Following treatment with corticosteroids she showed substantial improvement.- - - - - - - - - - ranking = 369615.15981998keywords = rigidity, muscle (Clic here for more details about this article) |
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