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The Babinski reflex.

The plantar response is a reflex that involves not only the toes, but all muscles that shorten the leg. In the newborn the synergy is brisk, involving all flexor muscles of the leg; these include the toe 'extensors', which also shorten the leg on contraction and therefore are flexors in a physiological sense. As the nervous system matures and the pyramidal tract gains more control over spinal motoneurones the flexion synergy becomes less brisk, and the toe 'extensors' are no longer part of it. The toes then often go down instead of up, as a result of a segmental reflex involving the small foot muscles and the overlying skin, comparable to the abdominal reflexes. With lesions of the pyramidal system, structural or functional, this segmental, downward response of the toes disappears, the flexion synergy may become disinhibited and the extensor hallucis longus muscle is again recruited into the flexion reflex of the leg: the sign of Babinski. A true Babinski sign denotes dysfunction of the pyramidal tract, and should be clearly distinguished from upgoing toes that do not belong to the flexion synergy of the leg. Correct interpretation of the plantar response depends only to a minor degree on the method or site of stimulation of the foot. It is therefore most important to assess the response in the entire leg.

Humans↗

The Babinski reflex.

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Central Nervous System Diseases↗

Determinants of prognosis of acute transverse myelitis in children.

BACKGROUND: Acute transverse myelitis (ATM) is a severe disorder; recovery requires several months and often leaves neurologic residua. To determine what features of patients with acute transverse myelitis significantly influence prognosis, the authors reviewed reports of ATM in Japanese children published in the last 15 years (from 1987 to 2001). METHODS: The authors studied reports of 50 Japanese patients (17 boys, 26 girls, 7 children of unspecified sex; mean age +/- SD, 8.0 +/- 3.8 years). Acute-phase and demographic features including age, increased deep tendon reflexes, Babinski reflex, sex, preceding infection, decreased deep tendon reflexes, time course of peak neurologic impairment, treatment with prednisolone and/or high-dose methylprednisolone, and the day of illness when treatment was started were used as independent variables in a regression analysis. The dependent variable was long-term persistence of neurologic deficits. RESULTS: Younger patients and those without increased deep tendon reflexes or a Babinski reflex were more likely to have residual neurologic deficits such as paraplegia or tetraplegia, sensory loss and sphincter disturbance. No relationship was seen between prognosis and sex, preceding infections, decreased deep tendon reflexes, time course of peak neurologic impairment, treatment with prednisolone or high-dose methylprednisolone, or timing of treatment initiation. CONCLUSIONS: Age at onset and neurologic features were important for outcome prediction in ATM. Steroid therapy did not associate with better outcome.

Adolescent↗

Conversion disorder presenting as hemiplegia and hemianesthesia with loss of neurologic reflexes: a case report.

Conversion disorders can present with a variety of sensorimotor signs and symptoms. We present the case of a young woman who presented with sudden onset hemiplegia and hemianesthesia including unilateral loss of deep tendon reflexes, Babinski reflex, and loss of rectal tone with bladder incontinence. The loss of rectal tone, loss of deep tendon reflexes, and a flaccid Babinski reflex are unusual signs in conversion disorders. A thorough medical workup including x-rays, CT scans, and magnetic resonance imaging revealed no organic causes for the neurologic deficits. Before the onset of symptoms, the patient was emotionally upset during an argument, which may have provided the psychologic stressor necessary for a conversion disorder. The young woman gradually regained full neurologic function over the next 14 hours. Conversion disorders should be considered when the neurologic findings do not correspond to known anatomic or physiologic pathways, although a thorough medical investigation must be performed to search for organic causes.

Adult↗

A three generations family with blepharo-naso-facial malformations suggestive of Pashayan syndrome.

Blepharo-naso-facial syndrome, described by Pashayan et al. (10), is characterized by telecanthus, lateral displacement and stenosis of lacrimal puncta, bulky nose, mask-like facies, trapezoïdal upper lip, torsion dystonia and mental retardation. We report on a family with this rare malformation syndrome, confirming the existence of this syndrome and its dominant inheritance. The proband had a fleshy nose, a prominant nose bridge, an hypoplastic midface, telecanthus with temporal displacement of puncta, lacrimal excretory obstruction. CNS torsion dystonia, increased deep tendon reflexes, Babinski reflexes, poor coordination and joint laxity. The proband's mother, brother and maternal grandfather also showed manifestations of the syndrome. The proband and his brother had delayed developmental milestones. Hearing impairment was present in the proband, his mother and his grandfather but was absent in the proband's brother. The blepharonasofacial syndrome was described by Pashayan et al. (10) in four members of one family, two male and one female sib and their mother. Two other sibs were unaffected. Many of the features of the blepharo-facio-nasal syndrome also occur in other well known syndromes i.e. Waardenburg syndrome. The pedigrees of the family of Pashayan et al. (10) and of our family are compatible with Mendelian dominant inheritance, either autosomal or X-linked. X-linked dominant inheritance cannot be ruled out except by male-to-male transmission, which does not occur in these families. Pashayan et al. (10) suggested that an autosomal gene with variable expressivity appears more likely. More families are needed for defining the transmission of the condition and for mapping the gene involved in the blepharo-naso-facial syndrome.

Abnormalities, Multiple↗

Effects of gestational alcohol on the development of neonatal reflexes in the rat.

The offspring of rats fed a diet containing 10% (v/v) of ethanol were tested for developmental differences from controls on a variety of maturational events and reflexes. Results indicated slower development of fur onset and ear (pinnae) opening, more rapid and vigorous righting responses and shorter dowel-grasping times in the offspring of ethanol-fed dams. No differences in rooting reflex, Babinski reflex, or in the visual placing response were found. These findings were seen as consistent with the interpretation that gestational alcohol exposure results in reduction in the ability of subjects to inhibit responses. The reduction in inhibition is not inconsistent with damage to the hippocampal area of the brain.

Animals↗

[Babinski's reflex].

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History, 20th Century↗

[Clinical studies on multiple lacunar state].

Sixty-five cases of clinically diagnosed multiple lacunar state have been analysed. The clinical course of multiple lacunar state is usually progressive in nature, simulating degenerative diseases, in contrast with the mode of acute onset seen in the other vascular diseases. Average age at the initial visit was 63.8 year-old, and sex ratio showed marked male preponderance, being 12 for males and 1 for females. Gait disturbance and speech disturbance are the most frequent initial symptoms, followed by slow motion, emotional lability and swallowing difficulty. Neurological manifestations are dysarthria, short-stepped and apraxic gait, hyperreflexia, positive Babinski and Chaddock reflexes, minimal spasticity, dementia, positive palmo-mental reflex, emotional lability, fixed face, rigidity, bradykinesia, foot grasping, dysphagia, positive Myerson's sign and tremor, in the order described. The important point is that the rigidity is paratonic and the tremor is action or postural, not the cogwheel rigidity or resting tremor like Parkinson's disease. The appearance of pathological reflexes (Babinski and Chaddock reflexes) are quite important, especially Chaddock reflex, which can frequently become positive despite negative or equivocal Babinski reflex. Hypertension, especially longstanding in nature, is the major contributing factor in this disorder. CT scan showed the presence of one or more lacunes in 52 out of 65 cases (80.0%). The detection of lacune can be influenced by the quality of CT scan, and the high resolution CT scanner is greatly useful for that purpose. It is stressed that the detailed neurological and computed tomographic evaluations will make it possible to reach the clinical diagnosis and appropriate treatment of multiple lacunar state.

Adult↗

[Spinal meningioma as differential diagnosis of diabetic polyneuropathy].

HISTORY AND ADMISSION FINDINGS: A 70 year old woman had suffered from diabetes mellitus type 2 since she was 52. Three years before the surgery she had begun to experience weakness together with altered sensitivity in the right leg, which was regarded as having been caused by diabetic polyneuropathy. During the admission examination the level for algesia on the right-hand side was at about D 11, a distal paraparesis of the leg (3-4 degrees, Janda's classification), more intense on the right, hyperactive deep tendon reflexes, Babinski's reflex on both sides, and depressed abdominal cutaneous reflexes. The sensitivity to vibrations on the Malleolus medialis on both sides was 0/8. The patient could walk only with the help of a Rollator. INVESTIGATIONS: Over the three-year period following onset of symptoms the following tests were carried out: motor nerve conduction speeds of the N. tibialis and N. peronaeus, electromyogram of the N. tibialis anterior and the M. gastrocnemius, somatosensory evoked potentials (SSEP) of the N. tibialis, which indicated a lesion in the peripheral nerves or nerve roots. Cranial computed tomography (CCT), CT scan of the lumbar spine (L3-S1) and angiological investigation elicited no significant pathological findings. An MRI of the thoracal spine showed a vertebra-sized dorsal tumor pressing on the spinal cord from left to right. TREATMENT AND COURSE: By means of microsurgery the spinal tumor was completely removed. Suspected meningeoma was confirmed by histological analysis. During the post-surgical period, the incomplete paraplegia quickly regressed, and 7 weeks after the removal of the spinal meningeoma the patient was able to climb stairs. CONCLUSION: In case of slowly-developing paresis of the legs in diabetic patients, diabetic polyneuropathy should not be diagnosed without careful consideration, and rare spinal tumors should be considered as part of the differential diagnosis, especially if the blood glucose level is normal, and intensive physiotherapy brings no improvement in the patient's condition.

Aged↗

[A patient with alternating hemiplegia in childhood].

Alternating hemiplegia in childhood (Verret, 1971) is a disorder presenting with frequent episodes of alternating hemiplegia from early infancy. We report a patient with this disorder, along with a pathophysiological study and a discussion about the available therapies for this disorder. The patient, an 11-year-old boy, visited our hospital with episodes of alternating hemiplegia from early infancy. His family history showed that many members suffered from migraine. He was born with asphyxia. Mental and motor developmental delays were seen from early infancy. The hemiplegic episodes with ipsilateral facial palsy had occurred frequently from early infancy. The episodes were frequently induced by emotional stress. The duration of hemiplegia varied from 10 minutes to 3 days. From the age of 11 years, he had begun to have migrainous attacks with or without hemiplegic episodes. Neurological examination revealed slight muscle hypotonia, choreoathetosis and dystonic movements induced by locomotion, positive Myerson sign, increased deep tendon reflexes and Babinski reflex. CAG, VAG and CAT revealed normal findings. EEG revealed diffuse generalized slowing during hemiplegic episodes. Measurement of regional cerebral blood flow (CBF) by 133Xe inhalation method revealed a slight decrease of bilateral CBF during a quadriplegic episode. Positron emission tomography using C15O2 revealed a slight decrease of CBF at the insula, putamen and claustrum of the left side during a right sided episode. Increased excretion of urinary 5-HIAA was seen during one episode. From our clinical and laboratory findings, we think this disorder may be a special type of migraine. Therapeutic trials of diazepam and flunarizine were both effective, but the initial effectiveness was decreased after 5 months.

Age Factors↗

["Skip lesions" in neurological deficits caused by tethered cord syndrome].

The neurological deficits caused by tethered cord syndrome do not necessarily follow a dermatomal pattern of the affected spinal cord from caudal to cephalic fashion. The authors defined these neurological deficits as "skip lesions", and reviewed the neurological deficits in tethered cord syndrome. Among 29 cases, inconsecutive segmental motor weakness in 3 cases, inconsecutive segmental sensory impairment in 6 cases, positive Babinski reflex in 8 cases, supranuclear neurogenic bladder in 3 cases and lumbago or leg pain in 4 cases were observed and they were thought to be compatible with the neurological deficits called "skip lesions". The basic mechanism of this sign is that the cephalic portion of the spinal cord is functionally impaired during flexion of the lumbosacral spine, while the caudal spinal cord with decreased visco-elasticity dose not elongate. Inconsecutive motor-sensory disturbance, accelerated deep tendon reflexes in the lower extremities, positive Babinski or other pathological reflexes in the lower extremities, uninhibited neurogenic bladder, lumbago and pain in the legs were considered to belong to the category of "skip lesions" and they can be treated by the untethering procedure. The authors concluded that preoperative careful insight into the mechanism of "skip lesions" revealed surgical efficacy for the reversible neurological deficits by the untethering procedure, or the efficacy of decompression of the lipoma or neurolysis around the involved spinal nerve roots.

Humans↗