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Journal of the Royal College of... 1996
Topics: France; Germany; History, 19th Century; Humans; Reflex, Babinski
PubMed: 8709074
DOI: No ID Found -
Annals of Indian Academy of Neurology Jan 2014Joseph Babinski (1857-1932) was born on November 17, 1857. He worked in a clinical arena dominated by Charcot and a focus on hysteria. His primary aim was in trying to...
Joseph Babinski (1857-1932) was born on November 17, 1857. He worked in a clinical arena dominated by Charcot and a focus on hysteria. His primary aim was in trying to find the reliable clinical signs to distinguish organic from non-organic disease of the nervous system. He was considered as masterly diagnostician, relying considerably less on neuropathological reports. Babinski's first attention to the reflex of the toes occurred during a chance observation of the contrasting responses between two female patients, one a hysteric and the other a hemiplegic. He first published description of his famous "sign" in 1896. Babinski's love for research works could be gauzed from his desire to publish and by the age of 27 years, he had to this credit, 12 important articles, mainly concerned with histological and neurological themes and one of his articles on the basic description of muscle spindles was considered to be a significant one. He was awarded the doctorate degree in 1885. Babinski introduced the concept of pithiatism, meaning "curable by suggestion." He anticipated the emergence of neurosurgery in France and only 6 days prior to his death he is on record to have said that his most vital contribution to the cause of neurosciences was not the sign he described, but that he could goad Clovis Vincent and Martel to take up neurosurgery as a specialty.
PubMed: 24753651
DOI: 10.4103/0972-2327.128522 -
Frontiers in Pediatrics 2022Charcot-Marie-Tooth (CMT) disease is a rare group of peripheral neuropathies with high clinical and genetic heterogeneity. CMT type 2N (CMT 2N) is a rare subtype of CMT...
INTRODUCTION
Charcot-Marie-Tooth (CMT) disease is a rare group of peripheral neuropathies with high clinical and genetic heterogeneity. CMT type 2N (CMT 2N) is a rare subtype of CMT with few clinical reports. The clinical presentation mimics that of other diseases, frequently leading to misdiagnoses. We present a case of CMT 2N with reversible white matter lesions (WMLs), which repeatedly mimicked stroke or encephalitis. We include a literature review to the improve management of this disease.
CASE DESCRIPTION
An 8-year-old boy was admitted to the hospital with slurred speech and limb weakness that had persisted for 1 day. Physical examination revealed lethargy, dysarthria, and a positive bilateral Babinski sign. Cerebrospinal fluid (CSF) analysis showed no abnormalities. Brain magnetic resonance imaging (MRI) revealed symmetrical abnormal signal areas in the paraventricular white matter and corpus callosum. The patient was suspected of having viral encephalitis and recovered rapidly after treatment.He was hospitalized 3 years later for limb weakness, barylalia, and facial paralysis persisting for 1 day. MRI showed an abnormal signal in the bilateral corona radiata. He was suspected of having a stroke or encephalitis. He was completely recovered after treatment.After a second 3-year span, he was admitted for another stroke-like episode. Physical examination revealed facial-lingual hemiparesis, mild atrophy of the left thenar muscle, decreased muscle strength in the extremities, and disappearance of the tendon reflex. MRI revealed more pronounced abnormal signals in the bilateral centrum semiovale and corpus callosum. Antibodies against autoimmune encephalitis were negative. A nerve conduction velocity (NCV) study showed motor and sensory four-limb nerve demyelination with axonal damage, most notably at the distal end. His symptoms were resolved after active treatment. A follow-up MRI showed the complete disappearance of the abnormal white matter signal. Whole exon sequencing showed a heterozygous mutation [c.2093C > T(p.Ser698Phe)] in the alanyl-tRNA synthetase 1 gene (). His mutation, clinical features, and electrophysiological testing led to a diagnosis of CMT 2N.
DISCUSSION
Early-Onset CMT 2N with reversible WMLs can often mimic stroke or encephalopathy. Affected individuals may show an atypical posterior reversible encephalopathy syndrome (PRES) on MRI. Careful family history assessment, physical examination, nerve conduction studies, MRIs, and genetic testing are essential for early diagnosis. Further studies are required to confirm these findings.
PubMed: 35911843
DOI: 10.3389/fped.2022.935721 -
Scientific Reports Feb 2016Different clinical results have been reported in the repair of extensor mechanism disruption using fresh-frozen complete extensor mechanism (CEM) allograft, creating a... (Comparative Study)
Comparative Study
Different clinical results have been reported in the repair of extensor mechanism disruption using fresh-frozen complete extensor mechanism (CEM) allograft, creating a need for a better understanding of fresh-frozen CME allograft reconstruction. Here, we perform histological and biomechanical analyses of fresh-frozen CEM allograft or autograft reconstruction in an in vivo rabbit model. Our histological results show complete incorporation of the quadriceps tendon into the host tissues, patellar survival and total integration of the allograft tibia, with relatively fewer osteocytes, into the host tibia. Vascularity and cellularity are reduced and delayed in the allograft but exhibit similar distributions to those in the autograft. The infrapatellar fat pad provides the main blood supply, and the lowest cellularity is observed in the patellar tendon close to the tibia in both the allograft and autograft. The biomechanical properties of the junction of quadriceps tendon and host tissues and those of the allograft patellar tendon are completely and considerably restored, respectively. Therefore, fresh-frozen CEM allograft reconstruction is viable, but the distal patellar tendon and the tibial block may be the weak links of the reconstruction. These findings provide new insight into the use of allograft in repairing disruption of the extensor mechanism.
Topics: Allografts; Animals; Autografts; Biomechanical Phenomena; Bone-Patellar Tendon-Bone Grafting; Freezing; Models, Animal; Osteocytes; Quadriceps Muscle; Rabbits; Reflex, Babinski; Tibia
PubMed: 26911538
DOI: 10.1038/srep22106 -
Neurologia 2018Pyramidal signs (hyperreflexia, spasticity, Babinski sign) are essential for the diagnosis of amyotrophic lateral sclerosis (ALS). However, these signs are not always...
INTRODUCTION
Pyramidal signs (hyperreflexia, spasticity, Babinski sign) are essential for the diagnosis of amyotrophic lateral sclerosis (ALS). However, these signs are not always present at onset and may vary over time, besides which their role in disease evolution is controversial. Our goal was to describe which pyramidal signs were present and how they evolved in a cohort of patients with ALS, as well as their role in prognosis.
METHODS
Retrospective analysis of prospectively collected patients diagnosed with ALS in our centre from 1990 to 2015.
RESULTS
Of a total of 130 patients with ALS, 34 (26.1%) patients showed no pyramidal signs at the first visit while 15 (11.5%) had a complete pyramidal syndrome. Of those patients without initial pyramidal signs, mean time of appearance of the first signs was 4.5 months. Babinski sign was positive in 64 (49.2%) patients, hyperreflexia in 90 (69.2%) and 22 (16.9%) patients had spasticity. Pyramidal signs tended to remain unchanged over time, although they seem to appear at later stages or even disappear with time in some patients. We found no association between survival and the presence of changes to pyramidal signs, although decreased spasticity was associated with greater clinical deterioration (ALSFR scale) (P<.001).
CONCLUSION
A quarter of patients with ALS initially showed no pyramidal signs and in some cases they even disappear over time. These data support the need for tools that assess the pyramidal tract.
Topics: Aged; Amyotrophic Lateral Sclerosis; Female; Humans; Male; Muscle Spasticity; Prognosis; Reflex, Abnormal; Reflex, Babinski; Retrospective Studies; Spain
PubMed: 27340020
DOI: 10.1016/j.nrl.2016.04.012 -
Journal of Neurology, Neurosurgery, and... Feb 1982
Topics: Humans; Muscle Spasticity; Paraplegia; Pyramidal Tracts; Reflex, Abnormal; Reflex, Babinski
PubMed: 6978383
DOI: 10.1136/jnnp.45.2.184 -
The Journal of Emergency Medicine Jul 2019Acute ischemic stroke (AIS) in pediatric populations accounts for more than half of pediatric strokes and is associated with significant morbidity and mortality....
BACKGROUND
Acute ischemic stroke (AIS) in pediatric populations accounts for more than half of pediatric strokes and is associated with significant morbidity and mortality. Pediatric AIS can present with nonspecific symptoms or symptoms that mimic alternate pathology.
CASE REPORT
A 4-month-old female presented to the emergency department for fever, decreased oral intake, and "limp" appearance after antibiotic administration. She was febrile, tachypneic, and hypoxic. Her skin was mottled with 3-s capillary refill, her anterior fontanelle was tense, and she had mute Babinski reflex bilaterally but was moving all extremities. The patient was hyponatremic, thrombocytopenic, and tested positive for influenza A. A computed tomography scan of the brain revealed an acute infarction involving the right frontal, parietal, temporal, and occipital lobes in addition to hyperdensities concerning for thrombosed cortical veins. The patient was transferred for specialty evaluation and was discharged 2 weeks later on levetiracetam. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Pediatric AIS can present with nonspecific symptoms that mimic alternate pathology. A high level of suspicion is needed so as not to miss the diagnosis of pediatric AIS in the emergency department. A thorough neurologic assessment is warranted, and subtle abnormalities should be investigated further.
Topics: Female; Fever; Humans; Hypoxia; Infant; Influenza, Human; Levetiracetam; Nootropic Agents; Stroke; Tachycardia; Tomography, X-Ray Computed
PubMed: 31027989
DOI: 10.1016/j.jemermed.2019.03.015 -
Neurology and Therapy Dec 2013Listeria monocytogenes is an important opportunistic pathogen affecting patients with immunosuppression and shows a high tropism for the central nervous system. The...
INTRODUCTION
Listeria monocytogenes is an important opportunistic pathogen affecting patients with immunosuppression and shows a high tropism for the central nervous system. The clinical manifestations of central nervous system listerial infections are variable and represent a diagnostic challenge.
CASE REPORT
The authors report the case of a 59-year-old woman who was admitted for confusion, agitation, and right-lower extremity weakness. The patient was treated for 3 months with fludarabine and 2 months with corticosteroids for chronic lymphocytic leukemia and hemolytic anemia, respectively. At the time of admission, the neurological examination revealed grade 4 right-lower extremity weakness with reflex asymmetry and right-sided Babinski sign; no signs of meningeal irritation were detectable. Physical examination was notable for grade 1 obesity and subfebrility. The cerebral computed tomography scan demonstrated a hypodense lesion in the left frontal lobe. Cerebral magnetic resonance imaging revealed a hyperintense lesion in the left frontal lobe with extension toward the basal ganglia (T2 and Fluid-Attenuated Inversion Recovery [FLAIR] sequences), and small nodular enhancing lesions after gadolinium infusion in the affected territory. Blood analyses revealed pancytopenia and elevated liver enzymes. During the second day after admission, the patient developed fever and neurological examination revealed signs of meningeal irritation. The cerebrospinal fluid (CSF) analyses revealed: red blood cells 24 cells/mm(3); white blood cells 829 cells/mm(3) (76% lymphocytes, 22% neutrophils, 2% monocytes); protein level 111.2 mg/dL; glucose level 10.2 mg/dL. Empiric anti-infection treatment was started with intravenous ceftriaxone, ciprofloxacine, aciclovir, and fluconasole. Both blood cultures and CSF cultures were positive for L. monocytogenes. The antimicrobial regimen was changed to ampicillin. The clinical and imaging outcome was excellent.
CONCLUSION
The supratentorial focal lesions secondary to Listeria meningoencephalitis are rare. The cases with focal neurological signs without fever at onset can resemble stroke.
PubMed: 26000217
DOI: 10.1007/s40120-013-0009-y -
The Journal of Neuroscience : the... Jul 1997Resistive force and electromyograms from triceps surae muscles were measured during dorsiflexion of both ankles of awake cats before and after interruption of one...
Resistive force and electromyograms from triceps surae muscles were measured during dorsiflexion of both ankles of awake cats before and after interruption of one dorsolateral funiculus (DLF). DLF lesions produced ipsilateral increases in dynamic and static reflex force that persisted over 66 weeks. The increase in dynamic reflex force was velocity sensitive, as demonstrated by a greater effect for 60 degrees /sec than for 10 degrees /sec dorsiflexion. Also, the lesions increased dynamic force to a greater extent than static force (increased dynamic index). Background force (recorded immediately before each reflex response) was elevated ipsilaterally. However, increases in reflex force were observed when preoperative and postoperative background forces were matched within 10% and were associated with equivalent resting levels of electromyographic (EMG) activity. Resistive reflex force was significantly correlated with EMG responses to dorsiflexion and was not determined by nonreflexive mechanical stiffness of the muscles. Contralateral background and reflex force and associated EMG activity were decreased slightly, comparing preoperative and postoperative records. Clinical testing revealed ipsilateral postoperative increases in extensor tone, increased resistance to hindlimb flexion, hypermetria during positive support responses, and appearance of the Babinski reflex. However, the most reliable tests of DLF lesion effects were the quantitative measures of dynamic and static reflex amplitude. The enhancement of stretch reflexes is suggestive of spasticity. However, hyperactive stretch reflexes, hypertonicity, and the Babinski reflex were observed soon after interruption of the ipsilateral DLF, in contrast to a gradual development of positive signs that is characteristic of a more broadly defined spastic syndrome from large spinal lesions. Also, other signs that often are included in the spastic syndrome, including clonus, increased flexor reflex activity, and flexor spasms, did not result from DLF lesions. Thus, unilateral DLF lesions provide a model of spasticity but produce only several components of a more inclusive spastic syndrome.
Topics: Animals; Cats; Electromyography; Female; Hindlimb; Muscle, Skeletal; Reflex, Abnormal; Reflex, Stretch; Spinal Cord Diseases; Time Factors
PubMed: 9185538
DOI: 10.1523/JNEUROSCI.17-13-05004.1997 -
Open Forum Infectious Diseases Nov 2023Neurological opportunistic infections cause significant morbidity and mortality in people with human immunodeficiency virus (HIV) but are difficult to diagnose.
BACKGROUND
Neurological opportunistic infections cause significant morbidity and mortality in people with human immunodeficiency virus (HIV) but are difficult to diagnose.
METHODS
One hundred forty people with HIV with acute neurological symptoms from Iquitos, Peru, were evaluated for cerebral toxoplasmosis with quantitative polymerase chain reaction (qPCR) of cerebrospinal fluid (CSF) and for cryptococcal meningitis with cryptococcal antigen test (CrAg) in serum or CSF. Differences between groups were assessed with standard statistical methods. A subset of samples was evaluated by metagenomic next-generation sequencing (mNGS) of CSF to compare standard diagnostics and identify additional diagnoses.
RESULTS
Twenty-seven participants were diagnosed with cerebral toxoplasmosis by qPCR and 13 with cryptococcal meningitis by CrAg. Compared to participants without cerebral toxoplasmosis, abnormal Glasgow Coma Scale score ( = .05), unilateral focal motor signs ( = .01), positive Babinski reflex ( = .01), and multiple lesions on head computed tomography (CT) ( = .002) were associated with cerebral toxoplasmosis. Photophobia ( = .03) and absence of lesions on head CT ( = .02) were associated with cryptococcal meningitis. mNGS of 42 samples identified 8 cases of cerebral toxoplasmosis, 7 cases of cryptococcal meningitis, 5 possible cases of tuberculous meningitis, and incidental detections of hepatitis B virus (n = 1) and pegivirus (n = 1). mNGS had a positive percentage agreement of 71% and a negative percentage agreement of 91% with qPCR for . mNGS had a sensitivity of 78% and specificity of 100% for diagnosis.
CONCLUSIONS
An infection was diagnosed by any method in only 34% of participants, demonstrating the challenges of diagnosing neurological opportunistic infections in this population and highlighting the need for broader, more sensitive diagnostic tests for central nervous system infections.
PubMed: 37965640
DOI: 10.1093/ofid/ofad515