Acute Flaccid Myelitis: Lessons From Polio.

With the eradication of poliomyelitis in the United States, the appearance of acute flaccid myelitis outbreaks has raised questions regarding their causation. Review of the epidemiology, clinical aspects, and laboratory findings of bygone cases of poliomyelitis have revealed shows important similarities with those of newer cases of acute flaccid myelitis. Many occurrences are preceded by an apparent viral illness, and a number of viruses, particularly enteroviruses A71 and D68, can be isolated from respiratory or stool specimens. Our inability to detect these viruses in cerebrospinal fluid samples from these patients does not eliminate them as etiologic agents, because poliovirus is often not detected in cerebrospinal fluid samples of patients with paralysis caused by poliomyelitis.

Profiling and identification of cerebrospinal fluid proteins in a rat EAE model of multiple sclerosis.

The experimental autoimmune encephalomyelitis (EAE) model resembles certain aspects of multiple sclerosis (MScl), with common features such as motor dysfunction, axonal degradation, and infiltration of T-cells. We studied the cerebrospinal fluid (CSF) proteome in the EAE rat model to identify proteomic changes relevant for MScl disease pathology. EAE was induced in male Lewis rats by injection of myelin basic protein (MBP) together with complete Freund's adjuvant (CFA). An inflammatory control group was injected with CFA alone, and a nontreated group served as healthy control. CSF was collected at day 10 and 14 after immunization and analyzed by bottom-up proteomics on Orbitrap LC-MS and QTOF LC-MS platforms in two independent laboratories. By combining results, 44 proteins were discovered to be significantly increased in EAE animals compared to both control groups, 25 of which have not been mentioned in relation to the EAE model before. Lysozyme C1, fetuin B, T-kininogen, serum paraoxonase/arylesterase 1, glutathione peroxidase 3, complement C3, and afamin are among the proteins significantly elevated in this rat EAE model. Two proteins, afamin and complement C3, were validated in an independent sample set using quantitative selected reaction monitoring mass spectrometry. The molecular weights of the identified differentially abundant proteins indicated an increased transport across the blood-brain barrier (BBB) at the peak of the disease, caused by an increase in BBB permeability.

Delayed CSF enhancement associated with Todd's paresis.

We present a patient with Todd's paresis who had undergone lung biopsy and who manifested increased signal intensity in the subarachnoid space of the right cerebral hemisphere on 10-h delayed enhanced fluid-attenuated inversion recovery images. We think that this was attributable to gadolinium leakage into the CSF due to increased leptomeningeal microvascular permeability in this patient with Todd's paresis.

Heat shock proteins HSP70 and HSP27 in the cerebral spinal fluid of patients undergoing thoracic aneurysm repair correlate with the probability of postoperative paralysis.

An understanding of the time course and correlation with injury of heat shock proteins (HSPs) released during brain and/or spinal cord cellular stress (ischemia) is critical in understanding the role of the HSPs in cellular survival, and may provide a clinically useful biomarker of severe cellular stress. We have analyzed the levels of HSPs in the cerebrospinal fluid (CSF) from patients who are undergoing thoracic aneurysm repair. Blood and CSF samples were collected at regular intervals, and CSF was analyzed by enzyme-linked immunosorbent assay for HSP70 and HSP27. These results were correlated with intraoperative somatosensory-evoked potentials measurements and postoperative paralysis. We find that the levels of these proteins in many patients are elevated and that the degree of elevation correlates with the risk of permanent paralysis. We hypothesize that sequential measurement intraoperatively of the levels of the heat shock proteins HSP70 and HSP27 in the CSF can predict those patients who are at greatest risk for paralysis during thoracic aneurysm surgery and will allow us to develop means of preventing or attenuating this severe and often fatal complication.

Clinical diagnosis in Karwinskia humboldtiana polyneuropathy.

Intoxication by Karwinskia humboldtiana presents a neurological picture similar to that for Guillain-Barré syndrome or other polyradiculoneuropathies. Clinical diagnosis in poisoned humans may be difficult if no evidence of previous fruit ingestion is available. We present our experience in the clinical diagnosis of Karwinskia humboldtiana polyneuropathy, as confirmed by toxin detection in blood. We designed an open trial at the Pediatric Neurology service and included all cases with acute ascending paralysis that were admitted to our hospital in the last two years. In all cases, we performed hematological, immunological and biochemical profiles, CSF analysis including immunological studies, oligoclonal bands and myelin basic protein determinations. Electrodiagnostic studies were performed, including motor conduction velocities, distal latencies, F-wave latency and compound muscle action potential (CAMP) amplitude. The presence of Karwinskia humboldtiana toxins in blood were determined by thin layer chromatography. In six cases, T-514 Karwinskia humboldtiana toxin was detected. These cases had a symmetric motor polyneuropathy with the absence of tendon reflexes and no sensory signs or cranial nerve involvement. Only one patient required assisted ventilation due to bulbar paralysis. In two of these cases, a sural nerve biopsy revealed a segmental demyelination with swelling and phagocytic chambers in Schwann cells and without lymphocytic infiltration. All six cases survived, with complete recovery in five. We conclude that this intoxication is common in Mexico. The availability of toxin detection in blood samples allows the clinician to establish an accurate diagnosis and should be included in the study of children with polyradiculoneuropathy, especially in countries where this poisonous plant grows.

Acute paralytic syndrome in three American men. Comparison with Chinese cases.

OBJECTIVE: To define clinically an unusual acute paralytic syndrome with features distinctive from those of the Guillain-Barré syndrome and similar to those described in Chinese children and young adults. DESIGN: Case series. SETTING: University medical centers. PATIENTS: Three North American men (mean age, 29 years) who presented with acute symmetric weakness and muscle cramps after a preceding gastrointestinal tract illness. These patients had no sensory symptoms, developed no respiratory insufficiency or facial weakness, and had normal to brisk muscle stretch reflexes. RESULTS: Results of serial nerve conduction studies were normal except for low-amplitude motor potentials. Needle electromyography revealed diffuse denervation potentials. Cerebrospinal fluid showed an elevated protein level and, in one case, a mild pleocytosis. A sural nerve biopsy specimen in one patient was normal; muscle biopsy specimens showed denervation atrophy. CONCLUSION: These cases resemble those described in Chinese children and young adults and may represent a postviral monophasic process affecting the anterior horn cell or distal motor nerve terminal. Further pathologic correlation will be required to identify the exact site of the lesion. Differentiation is important when considering modes of treatment.

A report of Froin's syndrome in five ovine thoracolumbar epidural abscess cases.

An epidural abscess involving the thoracolumbar spinal cord was considered the probable cause of pelvic limb paresis in those sheep where analysis of lumbar cerebrospinal fluid (CSF) samples revealed a significant increase in protein concentration (P less than 0.05) and some increase in total white blood cell concentration and neutrophil percentage. There was no significant increase in protein concentration in the corresponding cisternal samples. This phenomenon caused by blockage to CSF flow is not dissimilar to that of Froin's syndrome in man which has been reported as a result of localized spinal meningitis (Brain, 1985).

Intrathecal synthesis of antibodies to human T lymphotropic virus type I and the presence of IgG oligoclonal bands in the cerebrospinal fluid of patients with endemic tropical spastic paraparesis.

Tropical spastic paraparesis (TSP), a neuromyelopathy predominantly involving the pyramidal tract and commonly observed in tropical and equatorial areas, was recently found to be associated with human T lymphotropic virus type I (HTLV-I). We investigated sera and cerebrospinal fluid (CSF) from 19 patients with TSP who were from the Caribbean area, French Guiana, and Africa. Our results showed an elevated intra-blood-brain barrier IgG synthesis rate and an elevated IgG index, with an increased HTLV-I antibody-to-albumin ratio and the presence of CSF oligoclonal bands in the majority of the patients. These data, in association with similar HTLV-I antibody patterns between patients with TSP who were from these three regions, strengthen the probable etiologic role of HTLV-I in the pathogenesis of such chronic neuromyelopathies.

Isolation of an HTLV-1-like retrovirus from patients with tropical spastic paraparesis.

Tropical spastic paraparesis (TSP) is a slowly progressive myelopathy associated with increased serum and cerebrospinal fluid antibodies to the human T-lymphotropic retrovirus type I (HTLV-I) (ref. 1), and has been observed in many regions of the world. A similar condition known as HTLV-I-associated myelopathy occurs in the Kagoshima prefecture of Japan. Recent but controversial reports suggest involvement of virus related to HTLV-I in multiple sclerosis. Magnetic resonance imaging and electrophysiological studies indicate that TSP lesions are like multiple sclerosis in that they are disseminated throughout the nervous system. Complete virus from patients with TSP has proved difficult to isolate using techniques successful in adult T-cell leukaemia cases associated with HTLV-I. Here we report the isolation of an HTLV-I-like virus from T-cell lines derived from the peripheral blood and cerebrospinal fluid of TSP patients. The monoclonal antibody OKT3 was used to generate non-transformed T-cell lines that express HTLV-I antigens. Infectious virus was demonstrated by co-cultivation and complete, replicating virions were visualized ultrastructurally.

Cerebrospinal fluid levels of angiotensin-converting enzyme in Alzheimer's disease, Parkinson's disease and progressive supranuclear palsy.

Angiotensin-converting enzyme (ACE, E.C. 3.4.15.1) has been identified as a normal constituent of human cerebrospinal fluid (CSF). ACE activity in CSF from adult subjects without known neurologic disorder correlated positively (P = 0.002) with age between 50 and 90 years. Patients with moderate degrees of senile dementia of the Alzheimer's type and comparably demented patients with Parkinson's disease or progressive supranuclear palsy exhibited mean levels of ACE activity that were decreased 41, 27 and 53% respectively, compared to the mean level in an age and sex-matched group of neurologically intact individuals. These results raise the possibility that ACE activity in CSF may be an index of neuronal dysfunction in certain central neurodegenerative disorders.

Progressive supranuclear palsy and In111-DTPA cisternography.

Three cases of progressive supranuclear palsy are reported in which In111-DTPA cisternography showed slow diffusion, ventricular reflux and failure of isotope clearance. The clinical diagnosis of progressive supranuclear palsy was confirmed histologically in two of these patients. The possible causes of the cisternographic changes and their relationship to the changes of CSF dynamics in progressive supranuclear palsy are discussed.

[Regressive multiple paralysis of the cranial nerves and lymphocytic meningitis. 8 cases]. / Paralysie multiple des nerfs crâniens et méningite lymphocytaire d'évolution régressive.

Eight cases of spontaneously regressive cranial nerve paralysis related to the meningo-radiculitis reported by F. Rohmer et al. in 1974 are described. The epidemiological, clinical features, and the CSF changes which should allow early diagnosis are detailed, but this can only be confirmed by the spontaneous regression of the condition. The problems in determining the etiology are emphasised.

[So-called idiopathic trigeminal paralysis. Presentation of the clinical syndrome in 3 case reports]. / Die sogenannte idiopathische Trigeminusparese--Darstellung des Krankheitsbildes an drei Fallbeobachtungen.

Idiopathic trigeminal neuropathy can affect the sensory or motor division of the fifth cranial nerve but also occurs as a combined lesion of both systems. This rare syndrome is discussed in light of three cases seen in our clinic. As the diagnosis can be secured by exclusion only, special care is necessary in the differentiation of other trigeminal lesions utilizing CSF examination, CAT scans and other radiological methods.

Isolation of a paramyxovirus from the cerebrospinal fluid of a dog with posterior paresis.

A paramyxovirus was isolated from cerebrospinal fluid of a dog with a history of incoordination and posterior paresis. The virus apparently was not related to canine distemper virus (CDV), considering the lack of virus neutralization with CDV-specific antibody, negative immunofluorescence with CDV-specific conjugate, and avirulence for ferrets. The virus was antigenically related to a prototype strain of canine parainfluenza virus, as determined by positive immunofluorescence with canine parainfluenza virus-specific conjugate and virus neutralization tests.