Vincenzo Neri (1880-1960) and his sign to detect leg weakness due to corticospinal tract injury.

The Italian neurologist Vincenzo Neri (1880-1960), a pupil of Joseph Babinski (1857-1932), greatly contributed to refining the semiotics of neurological examination and was a pioneer in medical cinematography. In 1909, Neri proposed a sign to diagnose leg paresis due to a pyramidal tract lesion. According to Neri, if a patient standing with the legs apart and the arms crossed on the chest bends the trunk of the pelvis, when the trunk has almost reached the horizontal line, the leg on the paralyzed side flexes, whereas the unaffected leg remains extended. This sign reflects a spinal hyperfunctioning emerging after a pyramidal lesion, and should be interpreted as a part of a triple flexion reflex. Beyond the acute stage, it could reflect an unusual pattern of flexor spasticity involving the lower limb due to corticospinal tract injury. The sign described by Neri retains its validity in identifying this organic leg weakness due to pyramidal lesions, particularly when it is mild or in its early stages.

Camillo Negro (1861-1927) and his method for eliciting the extensor toe sign.

The "toe phenomenon", or extensor toe sign, is characterized by the extension (dorsiflexion) of the great toe elicited by plantar stimulation, and indicates pyramidal tract dysfunction. This phenomenon was first extensively described and studied by Joseph Jules François Félix Babinski (1857-1932), who introduced it in clinical practice. In 1912, the famous Italian neurologist Camillo Negro (1861-1927) proposed a new method of eliciting the extensor toe sign by inviting the patient, lying in bed in dorsal decubitus position, to raise the paretic limb with the leg extended on the thigh. This sign appeared during voluntary effort and could not be elicited by raising the unaffected lower limb. Negro was also the first to investigate the influence of cold upon the appearance of the "toe phenomenon" and to propose the use of (faradic) electrical stimulation to evoke it.

Adolph Seeligmüller (1837-1912) and the first graphic illustration of the "toe phenomenon" (later called "Babinski sign") in the medical literature.

The "toe phenomenon" refers to the extension (dorsiflexion) of the great toe, which occurs instead of the normal flexion following stimulation of the foot sole. Its clinical significance was not fully appreciated until Joseph Jules François Félix Babinski (1857-1932) described it in 1896. In 1881, Ernst Strümpell (1853-1925) had described a continuous (tonic) extension of the big toe, a finding that years later the French neurologist Jean-Athanase Sicard (1872-1929) recognized as an equivalent of the "toe phenomenon", also indicating pyramidal tract dysfunction. Previously, this phenomenon had been mentioned in patients only passingly and without providing a picture of it. In 1887, the German neurologist Adolph Seeligmüller (1837-1912) mentioned the tonic extension of the big toe among the characteristic clinical features of spastic infantile hemiplegia-a condition first described by the Austrian physician Moritz Benedikt (1835-1920) in 1868. Seeligmüller incorrectly attributed the tonic extension of the big toe to spastic contracture of the extensor hallucis longus muscle. However, he put great emphasis on this sign and considered it worth being illustrated. Adolph Seeligmüller therefore provided the very first graphic illustration of the (tonic) "toe phenomenon" in the medical literature. Of note, the first photographic illustration of this sign made by Babinski appeared only in 1900, when it had already been adopted by neurologists all over the world.

Would Francisco Soca have been the first to relate toe phenomenon as pyramidal disorders?

INTRODUCTION: The Uruguayan physician Francisco Soca, who specialized in neurology in Jean-Martin Charcot's clinic, defended a thesis at the Paris Faculty of Medicine in 1888 on Friedreich's ataxia in eleven patients. In this work he described the presence of toe phenomenon. OBJECTIVE: This historical note presents a toe sign described by the Soca eight years before Babinski's classic description. DISCUSSION: In the late 1800s Soca completed a specialization in neurology at the service run by Charcot in Paris. He defended an important thesis in 1888 assessing data from 11 Friedreich's ataxia. In his thesis, Soca also described the toe phenomenon and the presence of structural changes in the feet of these patients that were not described in the Friedreich study published in 1863. CONCLUSION: The Soca's thesis contained the description of toe extension associated with pyramidal tract lesions, eight years later described and further immortalized as Babinski's sign. Therefore, Soca had already publicized this sign as being representative of a pyramidal dysfunction before Babinski or any other neurologist.

The Babinski sign in the first Italian reports.

The Babinski sign, named after Joseph François Félix Babinski (1857-1932), is one of the most important in clinical neurology. It refers to the upward movement of the great toe following stimulation of the foot sole and is associated with a dysfunction of the pyramidal tract. The BS was recognized and adopted by neurologists all over the world immediately after its first description. In 1899, this sign was first introduced to the Italian scientific community at two different medical meetings. Some opinions on its clinical value and underlying pathophysiology were discordant, possibly reflecting suboptimal diagnostic accuracy of the first observations. Giovanni Mingazzini (1859-1929) first suggested that the BS reflects a complex mechanism emerging in patients with pyramidal tract dysfunction, whereas Giovanni Boeri (1867-1946) emphasized that the BS can be elicited if the neuromuscular structures underlying it are intact. Although some of their opinions eventually proved wrong, early Italian neurologists further contributed to the advancement in the understanding of this phenomenon.

The Babinski sign in Renaissance paintings-a reappraisal of the toe phenomenon in representations of the Christ Child: observational analysis.

OBJECTIVE: To investigate systematically the presence of the Babinski sign in paintings of the Christ Child by the greatest painters of the Renaissance. DESIGN: Observational analysis. SETTING: Large collection of paintings depicting the Christ Child from Flemish, Rhenish, and Italian schools between 1400 and 1550 CE, searched using published catalogues and Google. STUDY SAMPLE: 302 Renaissance paintings (by 19 painters) depicting the Christ Child. MAIN OUTCOME MEASURE: Babinski sign, defined as a hallux extension with an amplitude greater than 30°. The presence of foot sole stimulation was also noted. RESULTS: An unquestionable upgoing toe was apparent in 90 (30%) of the 302 paintings. The Babinski sign was present in more than 60% of Christ Child paintings by Rogier van der Weyden, Hans Memling, Martin Schongauer, and Matthias Grünewald. A bilateral Babinski sign was observed in three paintings. Stimulation of the sole was noted in 48/90 (53%) paintings and was always present in paintings by Andrea del Verrocchio, Leonardo da Vinci, and Giorgione. No association existed between the presence of the Babinski sign and the period during which the painter was active. CONCLUSIONS: Four main factors were noted in relation to the representation of the Babinski sign in paintings of the Christ Child: the physiological toe phenomenon in infants, the representation of the nudity of the Christ by painters during the 15th century to demonstrate the incarnation, Renaissance painters' need for precise observation of anatomy, and the desire of some Rhenish and Flemish painters to depict very realistic details. Italian Renaissance painters, whether Mannerist or not, tended to idealise the beauty of human body, and they often did not reproduce the Babinski sign.

Rotator drift: A sign of upper motor neuron leg weakness.

OBJECTIVE: There are techniques for eliciting subtle arm weakness (pronator drift), but the accompanying abnormal reflex response (Hoffmann's sign) is of limited value; conversely, in the leg there are no techniques for eliciting subtle weakness equivalent to pronator drift, but there is a robust abnormal reflex response (Babinski's sign). Thus, there is a need to devise a simple and rapid technique for detecting leg weakness capable of being used in either cooperative or comatose patients. PATIENTS AND METHODS: Using three patient groups (discovery set, training set, test set) a technique for detecting upper motor neuron (UMN) lesion leg weakness was devised. RESULTS: With the patient supine, the examiner grasps both big toes, pointing them towards the ceiling with the long axis of the foot perpendicular to the bed; the patient is asked to maintain this position for 30 s. People with pyramidal tract weakness show external rotator drift on their weak side: on the normal side the foot is deviated 20-25° from the perpendicular, on the paretic side the foot is deviated more than 30°. CONCLUSION: This rotator drift sign is a simple method for detecting subtle UMN leg weakness. When combined with the pronator drift sign, these two signs constitute "pyramidal drift" signs for the bedside detection of UMN hemiparetic weakness.

A New Method of Eliciting Pyramidal Tract Impairment in Adults.

BACKGROUND: To suggest a new way of eliciting pyramidal tract dysfunction in adults since the most widely utilized plantar reflex, which is the Babinski reflex, has limitations with different reliability and consistency among different examiners. MATERIALS AND METHODS: 168 adult subjects were examined for the new sign in addition. It consists of just an observation of the patient's feet and toes in a conscious patient looking for the extension of the great toe along with fanning, spreading and plantar flexion of the small toes either at rest or when patient elevates one leg up at a time. RESULTS: We were able to observe the extension of the great toe along with fanning, spreading and plantar flexion of the small toes in patients with impairment of pyramidal tract. The specificity was 94% while the sensitivity was 96%. CONCLUSION: Pyramidal tract lesion in adults can be elicited by this new test that observes the extension of the great toe along with fanning, spreading and plantar flexion of the small toes in patients. We suggest this sign as a complement to established signs like Babinski reflex.

Does the Babinski sign predict functional outcome in acute ischemic stroke?

OBJECTIVE: The aim of this prospective cohort study was to determine the incidence and neuroimaging risk factors associated with Babinski sign following acute ischemic stroke, as well as its relationship with the functional outcome of patients. METHODS: A total of 351 patients were enrolled in the study within 7 days of acute ischemic stroke. The Babinski sign along with other upper motor neuron signs were examined upon admission and between days 1 and 3 and days 5 and 7 after admission. Neuroimaging parameters included site and volume of infarction and white matter lesions. All patients were followed up at 3 months. Functional outcome was assessed with the Lawton Activities of Daily Living scale and modified Rankin Scale. RESULTS: Babinski sign was observed in 115 of 351 (32.8%) patients in the acute ischemic stroke. These patients had higher National Institutes of Health Stroke Scale (NIHSS) scores at admission and higher rates of atrial fibrillation and cardioembolism; higher frequencies of frontal, temporal, and limbic lobes and basal ganglia infarcts; and larger infarct volume. Higher NIHSS score and basal ganglia infarct were significant predictors of the presence of Babinski sign. After adjusting for confounds, the presence of Babinski sign did not predict poor functional outcome. CONCLUSION: The incidence of Babinski sign was 32.8% in the acute ischemic stroke. Severe infarction and basal ganglia infarct were independent predictors of Babinski sign. Although Babinski sign is common in acute ischemic stroke patients, it does not predict poor functional outcome 3 months later.

Case Report: Cognitive Impairment without Clinical Spinal Disease May Be the First Sign of HTLV-1 Neurological Alteration.

Human t-cell lymphotropic virus type 1 (HTLV-1)-associated myelopathy (HAM) is a progressive neurological disease whose diagnosis is defined by clinical manifestations and seropositivity for HTLV-1 infection. Cognitive impairment (CI) is considered to occur after spinal impairment. A 51-year-old HTLV-1-infected man classified as an asymptomatic carrier presented difficulties in listening comprehension and executive memory. He was assessed for central auditory processing (CAP), cognition (event-related auditory evoked potential [P300]), and otoneurological functions (galvanic vestibular-evoked myogenic potential [gVEMP]). Altered responses were found in CAP, P300, and gVEMP, but the neurological examination and cognitive screening were normal. After a 2-year follow-up, we disclosed a positive Babinski sign, a mild CI, worsened P300, and gVEMP latencies, and the patient reported progressive lumbar pain and difficulty running. He was, then, reclassified as HAM. The first examination, in 2016, had already shown abnormal results in P300 and gVEMP despite the HTLV-1-asymptomatic carrier status. Therefore, tests that provide subclinical measures of neurological disease progression can be useful tools for an early diagnosis and intervention in HTLV-1 patients. Electrophysiological results had worsened as well as the clinical status and the cognitive function and the progression from asymptomatic status to an HTLV-1-associated neurological disease occurred within 2 years. Thus, HTLV-1-infected individuals with complaints of CI, hearing, or otoneurological manifestations should be submitted to neuropsychological and electrophysiological tests, allowing them to be properly cared in case of HAM progression.

Cocaine as a rare cause of locked-in syndrome: a case report.

INTRODUCTION: In the United States, cocaine is a commonly used drug of abuse. It is also a recognized contributing factor for both hemorrhagic and ischemic strokes. However, cocaine-induced basilar artery thrombosis has rarely been reported in the literature. CASE PRESENTATION: Our patient was a 51-year-old African American woman with a history of polysubstance abuse who presented to the emergency department for acute behavior changes. Later, during admission, she had a dramatic decrease in motor strength in all extremities and a positive Babinski reflex bilaterally. The results of her toxicology reports were positive for cocaine; in addition, results of magnetic resonance angiography and magnetic resonance imaging were consistent with acute thrombosis and subsequent infarction of the basilar artery. Her mental status improved, but she was only able to communicate via movements of her eyes. CONCLUSION: Our patient developed locked-in syndrome after use of cocaine. Given the prevalence of its use in the United States, cocaine use should be included among the potential causes of locked-in syndrome.

Fibrocartilaginous Embolism of the Spinal Cord in Children: A Case Report and Review of Literature.

Fibrocartilaginous embolism is assumed to be caused by fibrocartilaginous nucleus pulposus component migration through retrograde embolization to the spinal cord artery. Fibrocartilaginous embolism is currently not well recognized among pediatricians because of its rarity. We describe a previously healthy 15-year-old soccer player who, after kicking a ball, developed progressive weakness in both legs and urinary retention the next day. Magnetic resonance imaging revealed T2 hyperintensity in the anterior horn of the spinal cord at the Th12/L1 level with Schmorl node at the level of L1/2. We also review the previous literature on fibrocartilaginous embolism of the spinal cord in children (less than18 years age); a total of 25 pediatric patients, including our patient, were identified. The median age was 14 years, and 64% of the reviewed patients were female. The most common trigger event was intense exercise or sports. The neurological symptoms started within one day in most cases, and the time to symptom peak varied from a few hours to two weeks. The most common initial neurological symptoms were weakness or plegia (100%), followed by paresthesia or numbness (48%). Affected areas of the spinal cord were distributed evenly from the cervical to thoracolumbar regions. Although steroids and anticoagulants were most commonly used, the prognosis was quite poor (mild to severe sequelae with three deaths). Although fibrocartilaginous embolism is a very rare condition, physicians should be aware of the characteristics and include fibrocartilaginous embolism of the spinal cord in their differential diagnosis, especially for physically active patients.

Acoustic Analysis of Crying Signal in Infants with Disabling Hearing Impairment.

OBJECTIVE: Crying is a multimodal, dynamic behavior and the first way to communicate. Early identification of hearing impairment is critical for prevention of speech and language disorders. The present study aimed to assess the acoustic features of infant's cry signals to find possible differences between two groups including hearing-impaired (HI) infants and normal hearing (NH) control. METHODS: The data were collected from 34 (17 HI, 17 NH) infants under 2 months of age. Recording of the infant cry signals was collected during the examination of the Babinski reflex and was subsequently submitted for acoustic analysis. The total duration of the recording for each infant was approximately 30 seconds. The acoustical features included fundamental frequency (F0), formants (F1, F2, and F3), intensity, jitter, shimmer, ratios of F2/F1 and F3/F1, ratio of harmonic to noise, and voice break. The recording device was an Olympus ws-321M voice recorder with 44,100 Hz sampling frequency in the stereo form. Praat analysis software (version 27, 3, 5) was used to analyze the crying signals. The data were then statistically analyzed using SPSS version 21. RESULTS: Acoustic analysis of the crying signals showed that HI infants have lower intensity and higher F0 and voice break than NH infants. However, the other differences were not statistically significant. CONCLUSION: The results of the present study demonstrated that the acoustic components including F0, intensity, and voice break may be used as indices to discriminate HI infants from NH infants under 2 months of age. These findings can be increased our knowledge concerning the functional mechanisms of the vocal organ in HI and NH infants.

Babinski's hand sign: many have tried .

Recognizing the semiologic importance of the second, and most famous, sign described by Joseph Babinski - the extension of the hallux after stimulation of the plantar region in order to differentiate organic from pithiatic paralysis- several authors have tried to find a comparable signal in the hand. After 122 years, no one has succeeded.

Late-onset Myasthenia Gravis Accompanied by Amyotrophic Lateral Sclerosis with Antibodies against the Acetylcholine Receptor and Low-density Lipoprotein Receptor-related Protein 4.

An 82-year-old woman developed neck weakness and dysarthria with antibodies against acetylcholine receptor (AChR) and low-density lipoprotein receptor-related protein 4 (LRP4). Myasthenia gravis (MG) was diagnosed by edrophonium and repetitive nerve stimulation tests. Her symptoms resolved completely by immunotherapy. One year later, she presented with muscle weakness and bulbar palsy accompanied by atrophy and fasciculation. Her tendon reflexes were brisk, and Babinski's sign was positive. She was diagnosed with probable amyotrophic lateral sclerosis (ALS). Immunotherapy did not improve her symptoms, and she ultimately died of respiratory failure. MG and ALS may share a pathophysiology, including anti-LRP4 antibodies at the neuromuscular junction.