1/29. amyotrophic lateral sclerosis in an adult following acute paralytic poliomyelitis in early childhood.About 30% of polio survivors develop a post-polio syndrome. Some of these patients develop slowly progressive muscle weakness known as post-poliomyelitis muscular atrophy (PPMA). We describe an unusual form of amyotrophic lateral sclerosis (ALS) in a patient with acute poliomyelitis in childhood. An 80-year-old woman had acute poliomyelitis at 2 years of age and developed weakness limited to the lower extremities. Residual weakness was stable until the age of 75 when she developed rapidly progressive weakness that first affected her left arm and subsequently the right arm. Neurological examination revealed both upper and lower motor neuron signs. These clinical features were more consistent with ALS than PPMA. At autopsy, there was marked atrophy of the precentral gyrus. Microscopic examination revealed a severe loss of all nerve cells and pronounced fibrillary astrocytosis of the lumbar ventral horns in the spinal cord, presumably a result of poliomyelitis. Superimposed on these spinal cord alterations were the pathological features of ALS, consisting of loss of Betz cells, corticospinal tract degeneration and loss of motor neurons of other levels of the spinal cord. The findings included some atypical features for ALS, namely, sparing of the hypoglossal nucleus, absence of Bunina bodies and absence of ubiquitin-immunoreactive inclusions. Although poliomyelitis and ALS may be coincidental, the unusual pathological expression of ALS raise the possibility that it is related to the antecedent poliomyelitis.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/29. Surgical treatment of poliomyelitic scoliosis.Between 1968 and 1973 forty nine patients suffering from poliomyelitic scoliosis were treated surgically at the Rizzoli Institute. They were due to asymmetrical paralysis and contracture in the muscles of the trunk and limbs. Associated pathological conditions were found, such as pelvic obliquity, and vascular and trophic changes due to ganglionic lesions. The differing incidence and combination of these factors gave rise to various clinical types of spinal deformity. The average severity of curve was 39 degrees, the localisation was predominantly central, the average extent was ten vertebrae, and there was a marked predominance of right convexity (twenty nine out of thirty six). The rate of progression was maximum during puberty and almost negligible after bony maturity. It was greater in males and was unfavourably affected by the severity and asymmetrical distribution of the paralysis, by the early appearance of the disease, by high localisation of the deformity, and by the erect posture in patients who were ambulant. The most frequent visceral complications were in the respiratory system (ten patients with a deficit over 50%), followed by cardiac changes. Surgical treatment was adopted in patients with progressive curves over 60 degrees, because of the inevitable deterioration in their general condition and the tendency of the deformity to become fixed. Pre-operative correction by Halo-traction results (52% correction) than Risser plasters (38%). Posterior arthrodesis by Harrington's method was carried out in all the more recent cases (forty four). Post-operative plaster was maintained for eight months and then replaced by an orthopaedic corset. At bony maturity there was an averaged improvement of 35% in the angle of curvature, and an average improvement of 6% in vital capacity. The best corrections were obtained in patients under fourteen (42%), in dorso-lumbar scoliosis (40%) and in patients with curves above 100 degrees (38%). There was an average increase in height of 9.1 cms and a reduction in the gibbus of 3.4 cms. The complications included one traumatic pneumothorax, eight pseudarthroses, and breakage of the distraction rod in two cases resulting in complete relapse of the deformity. In six cases the upper hooks became loos and there were two cases of postoperative staphylococcal infection. In the distally sited curves our present policy is towards combining posterior arthrodesis with Dwyer's anterior interbody fusion.- - - - - - - - - - ranking = 0.25keywords = spinal (Clic here for more details about this article) |
3/29. Acute care pediatric electromyography.The recognition of uncommon pediatric motor unit disorders or unusual clinical presentations of common illnesses, such as guillain-barre syndrome (GBS), have increased the need for electromyography (EMG) in childhood critical care units. There are two different clinical sets, one appropriate to newborns and infants and the other to older children. Some illnesses that present as an acute floppy infant are not found in the differential diagnosis of motor unit disorders in the older child or adult. These include spinal muscular atrophy, postvaccine poliomyelitis, intrauterine GBS, infantile botulism, and severe myopathies, such as myotonia dystrophy, and some glycogen storage diseases. An appreciation of the neurophysiological maturational norms is essential to an effective pediatric EMG consultation for children ages 0-3 years. Additionally, the neuromuscular complications of extended intubation and sepsis in children are gaining broader recognition. An increased dialogue between clinical neurophysiologists and pediatric neurologists and intensivists in both neonatal and pediatric intensive care units is essential.- - - - - - - - - - ranking = 0.25keywords = spinal (Clic here for more details about this article) |
4/29. Nonpoliovirus poliomyelitis simulating guillain-barre syndrome.BACKGROUND: Paralytic poliomyelitis due to the wild-type poliovirus has been eradicated in the united states because of effective immunization programs. In the postvaccination era, most cases are caused by other rna viruses, such as coxsackievirus or echovirus. The condition usually begins with a fever and upper respiratory tract or gastrointestinal tract symptoms that progress to a "paralytic" phase characterized by limb weakness, areflexia, and, occasionally, respiratory failure that superficially resemble guillain-barre syndrome. OBJECTIVE: To describe 2 patients with nonpoliovirus poliomyelitis and highlight the findings on magnetic resonance imaging of the spinal cord to distinguish these cases from variants of guillain-barre syndrome. DESIGN AND SETTING: Case series from an academic medical center. patients: Following a viral illness, the patients, aged 35 and 50 years, had painless, progressive, asymmetrical weakness in the arms followed by respiratory failure in one patient, and generalized limb weakness in the other patient, reaching a nadir in 1 week. Both patients had fevers but no signs of meningitis at onset. Tendon reflexes were absent or reduced in affected regions. The cerebrospinal fluid findings were as follows: mononuclear leukocyte counts of 100 000 cells/mm(3) and 700 000 cells/mm(3), respectively, and the protein level was above 10 g/dL in both patients. Compound muscle action potential amplitudes were reduced in some nerves with active denervation in clinically affected muscles, and F-responses were absent but there were no other demyelinating features. magnetic resonance imaging showed discrete T2-weighted signal changes of the ventral horns of the spinal cord, and one had elevated coxsackievirus titers in the serum. There was little recovery and significant atrophy in weak muscles after 3 years. CONCLUSIONS: The poliomyelitis syndrome still occurs in adults in developed countries. It has superficial similarities to a motor axonal variant of guillain-barre syndrome but can be distinguished by clinical, cerebrospinal fluid, and, perhaps specifically, magnetic resonance imaging characteristics.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
5/29. Histopathologically proven poliomyelitis with quadriplegia and loss of brainstem function due to west nile virus infection.Recent electrophysiological and histopathological reports point to motor neurons in the anterior horn of the spinal cord and the brainstem as targets of severe west nile virus (WNV) infection. We report histopathological confirmation of this poliomyelitis-like syndrome in a patient with WNV infection in massachusetts.- - - - - - - - - - ranking = 0.25keywords = spinal (Clic here for more details about this article) |
6/29. Calf pseudohypertrophy in a patient with double neurogenic pathology.A pseudohypertrophy of the calf can be rarely associated with neurogenic pathologies as S-1 radiculopathy, poliomyelitis, spinal muscular atrophy, traumatic lesions of peripheral nerves, intraspinal neurinoma. The causes of this particular phenomenon are unknown. The authors present the case of a 52-year-old man with an enlargement of the left calf suffering from a mild form of spinal paralytic poliomyelitis in the early childhood and episodes of severe left sciatica in the last four years. electromyography demonstrated a pattern of denervation in both legs and an h-reflex absent when the left tibial nerve was stimulated. An open muscle biopsy of the left calf was performed. light microscopic and ultrastructural examination of the muscle confirmed the presence of a pattern of "neurogenic type" pseudohypertrophy. Our results could be interesting for the understanding of the mechanism of neurogenic pseudohypertrophy. This case suggests that timing of stimulus or "dose" of denervation may be important factors in such a phenomenon.- - - - - - - - - - ranking = 0.75keywords = spinal (Clic here for more details about this article) |
7/29. Fatal fulminant pan-meningo-polioencephalitis due to west nile virus.We report a case of fatal fulminant west nile virus (WNV) meningoencephalitis in an 87-year-old white male gardener. The pennsylvania patient presented with a 3-day history of flu-like symptoms. His hospital course was gravely precipitous with onset of coma, ventilator dependence, loss of cortical and brainstem functions within ten days of admission. Acute serum and cerebrospinal fluid samples revealed elevated levels of WNV IgM antibodies by ELISA as well as elevated CSF white blood cells, protein and glucose. A complete autopsy revealed a multifocal lymphocytic myocarditis and severe chronic tubulointerstitial nephritis. Viral culture and PCR analysis of post-mortem samples of the spleen, kidney and brain were positive for WNV. Histological sections from all regions of the brain and spinal cord demonstrated a severe, non-necrotizing, subacute, polio-meningoencephalitis. While both gray and white matter were inflamed, gray matter was much more severely involved. Many gray matter nuclei showed severe neuronal loss with residual dying neurons surrounded by activated microglia. Immunohistochemical stains revealed profuse infiltration of the meninges and cerebral parenchyma by CD8 t-lymphocytes and perivascular b-lymphocytes. Electron micrographs revealed diffuse intracellular and extracellular edema but no viral particles were identified. Immunohistochemical and immunofluorescent staining for WNV filled the cytoplasm of residual neurons. west nile virus mediates a predominantly polioencephalitis secondary to direct infection of neurons.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |
8/29. Report of five children with guillain-barre syndrome following a nationwide oral polio vaccine campaign in turkey.Five children with guillain-barre syndrome (GBS), following a national oral polio vaccination campaign to eradicate disease, are reported. Clinical examination, cerebrospinal fluid and electromyographic findings conformed to the classical description of GBS. Four of them received therapeutic dose of intravenous immunoglobulin g. Two children succumbed to the disease. It was observed that the number of cases of GBS in children increased during the period of the oral polio vaccination campaign in turkey, suggesting a causal relationship.- - - - - - - - - - ranking = 0.25keywords = spinal (Clic here for more details about this article) |
9/29. MRI in poliomyelitis-like syndrome.We report the MRI findings in a patient with poliomyelitis-like syndrome. MRI demonstrated a lesion extending from the dorsal region of the pons to the upper level of the thoracic spinal cord. The spinal cord was particularly affected in a region involving the bilateral anterior horns. Although its pathology is unknown, the lesion of the spinal cord seemingly reflects the pathological features of this disease.- - - - - - - - - - ranking = 0.75keywords = spinal (Clic here for more details about this article) |
10/29. Electrodiagnostic features of acute paralytic poliomyelitis associated with West Nile virus infection.west nile virus (WNV) infection is a potentially fatal disease, with meningoencephalitis being its most common neurological manifestation. guillain-barre syndrome (GBS) has also been described, but acute paralytic poliomyelitis has only recently been recognized. We report the clinical and electrodiagnostic findings of five patients with WNV infection, who presented with acute paralytic poliomyelitis. Three patients manifested focal asymmetrical weakness, and two had rapid ascending quadriplegia mimicking GBS. Electrodiagnostic studies during the acute illness showed normal sensory nerve action potentials, compound motor action potentials of normal or reduced amplitude, and no slowing of nerve conduction velocities. Depending on the timing of the examination, fibrillation potentials were widespread, including in those with focal weakness. Cervical magnetic resonance imaging in one patient showed abnormal T2-weighted signals in the spinal cord gray matter. On follow-up, signs of clinical improvement were seen in one patient, whereas two remained quadriplegic and ventilator-dependent 5 months after the onset. This report highlights the value of the electrodiagnostic studies in the diagnosis and prognosis of focal or generalized weakness due to acute paralytic poliomyelitis associated with WNV infection.- - - - - - - - - - ranking = 0.25keywords = spinal (Clic here for more details about this article) |
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