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[100 years of the Babinski sign].

In 1896 Joseph Francois Felix Babinski described for the first time the phenomenon of the toes. In his first paper he simply described extension of all toes with noxious stimulation of the sole of the foot. It was not until 1898 that he specifically described the extension of the hallux with stimulation of the lateral border of the sole. Babinski was probably not aware at the time that E. Remak, a German physician, had previously described the sign. In his third paper of 1903 Babinski concludes that if other authors had described the abnormal reflex before him, they found it fortuitously and did not realize its semiologic value. Babinski probably discovered it by a combination of chance, careful observation and intuition. He also had in mind practical applications of the sign particularly in the differential diagnosis with hysteria and in medico-legal areas. Several of his observations and the physiopathological mechanism proposed by him are still valid today. He realized since 1896 that the Babinski reflex was part of the flexor reflex synergy. He observed that several patients during the first hours of an acute cerebral or spinal insult had absent extensor reflexes. He realized that most patients with the abnormal reflex had weakness of the toes and ankles. He found a lack of correlation between hyperactive myotatic reflexes and the presence of an upgoing hallux. He discovered that not all patients with hemiplegia or paraplegia had the sign. He thought erroneously that some normal subjects could have an upgoing toe. His dream of a practical application of the sign has been fully achieved. The motto of Babinski was Observatio summa lex. Perhaps there is no better dictum in clinical neurology.

Adult↗

The red reflex.

Eliciting the red reflex is a useful clinical test that can be easily performed in the pediatric emergency room. A direct ophthalmoscope is the only tool needed to perform this test. The test is non-invasive, making it a quick screening tool for even the most apprehensive child. A normal red reflex can rule out intraocular pathology; an abnormal reflex can indicate need for urgent ophthalmologic referral. The differential diagnoses for an absent (or black) reflex; an abnormally sized, shaped or positioned reflex; leukocoria; as well as a non-homogenous reflex are listed.

Child↗

Herniated cervical intervertebral discs with radiculopathy: an outcome study of conservatively or surgically treated patients.

To study the functional outcomes of patients with cervical herniated intervertebral discs with radiculopathy but without signs of cervical myelopathy using a retrospective cohort study design. The patients were retrospectively identified by chart reviews. Inclusion criteria were (a) cervical radiculopathy with defined neurological disturbances (sensory disorder, reflex abnormalities, and motor weakness); (b) a spinal computed tomograph scan or magnetic resonance image demonstrating neuroradiologic abnormalities (soft or hard disc); and (c) completion of conservative physical and pharmacological treatment. Of 119 consecutive patients who were treated at the Departments of Neurology and Neurosurgery, University of Erlangen-Nuremberg, between January 1, 1985, and December 31, 1995, 60 patients met the inclusion criteria and were prospectively assessed, with an average follow-up time of 5.5 years. The patients were interviewed with regard to their daily activities, ability to work, and the surgical treatment they had undergone. A complete neurological examination also was performed. In 88.3%, the onset of disease was acute and in 11.7% subacute or chronic. The most common signs and symptoms were brachialgia (98.3%), neck pain (93.3%), sensory disorders (88.3%), reflex abnormalities (61.7%), and motor weakness (51.7%). The neuroimaging procedures demonstrated a disc prolapse (soft disc) in approximately 90% and spondylotic osteophytes (hard disc) in approximately 10%. During an average follow-up time of 5.5 years (range, 4.6 months-10.6 years) 39 (65%) patients had been treated using only conservative methods (COG = conservatively treated group), and 21 (35%) patients had undergone surgery (ventral discectomy) (SUG = surgically treated group). Brachialgia was completely or essentially improved in 100% of the COG and 95.1% of the SUG. Sensory disorders remitted completely or markedly in 97% of the COG and 75% of the SUG. The reflex abnormalities normalized or improved in 59.2% of the COG and in 53.3% of the SUG. Motor weakness improved in 94.1% of the COG and in 50% of the SUG. Neck pain was difficult to treat. It improved in only 36.1% of the COG and in 20% of the SUG. Occupational capacity was lost in 10% of the COG and in 38.9% of the SUG. In a self-rating scale, 89.7% of patients in the COG did not feel disabled in their everyday activities, compared with 66.7% of the patients in the SUG. Patients with a herniated cervical intervertebral disc with radiculopathy can be treated conservatively with good results, although a residual intermittent neck pain syndrome often persists. The patients in the SUG, who initially showed more severe and long-lasting neurological disturbances, were improved at the time of examination, although with more marked residual disorders. Surgery is indicated only when appropriate conservative treatment for a reasonable time has failed.

Adult↗

The spinal component to skin blood flow abnormalities in reflex sympathetic dystrophy.

OBJECTIVE: To determine whether the mechanisms of reflex sympathetic dystrophy, a neuropathic pain syndrome characterized by skin blood flow abnormalities associated with sympathetic vasoconstrictor and antidromic vasodilator mechanisms, are solely of peripheral origin or have an additional spinal component and act exclusively through neural or also involve humoral pathways. PATIENTS: The 54 patients with unilateral reflex sympathetic dystrophy were divided into the following three stages according to their perception of skin temperature in the clinically affected hand: stage I, stationary warmth sensation; stage II, intermittent warmth and cold sensation; and stage III, stationary cold sensation. METHODS: Investigation of basal skin blood flow and vasoconstrictive response to dependency of skin microvessels in the clinically unaffected hand and the clinically affected hand of patients with reflex sympathetic dystrophy and the left hand of 16 control subjects. Microcirculation was investigated at the predominantly neurally controlled thermoregulatory level (Doppler laser flowmetry) and at the predominantly humorally controlled nutritive level (capillary microscopy). RESULTS: In the clinically unaffected hand, at the thermoregulatory level of the microcirculation: (1) basal skin blood flow was increased at stage I compared with the control subjects, whereas no differences could be observed at this stage compared with the clinically affected hand; (2) the vasoconstrictive response to dependency (defined as skin blood flow at heart level divided by skin blood flow in the dependent position) was attenuated at stage I compared with the control subjects, whereas no differences could be observed at this stage compared with the clinically affected hand; and (3) basal skin blood flow and the vasoconstrictive response to dependency did not differ from the control subjects at stages II and III. In the clinically unaffected hand, at the nutritive level, no differences could be observed at any stage of the syndrome compared with the control subjects. CONCLUSIONS: This study indicates that there is a spinal component to microcirculatory abnormalities at stage I of the reflex sympathetic dystrophy syndrome that most likely acts through neural (antidromic vasodilator) mechanisms and that may be initiated by traumatic excitation of a peripheral nerve on the clinically affected side.

Adult↗

Monitoring of head injury by myotatic reflex evaluation.

OBJECTIVES: (1) To establish the feasibility of myotatic reflex measurement in patients with head injury. (2) To test the hypothesis that cerebral dysfunction after head injury causes myotatic reflex abnormalities through disordered descending control. These objectives arise from a proposal to use reflex measurements in monitoring patients with head injury. METHODS: The phasic stretch reflex of biceps brachii was elicited by a servo-positioned tendon hammer. Antagonist inhibition was evoked by vibration to the triceps. Using surface EMG, the amplitude of the unconditioned biceps reflex and percentage antagonist inhibition were measured. After standardisation in 16 normal adult subjects, the technique was applied to 36 patients with head injury across the range of severity. Objective (1) was addressed by attempting a measurement on each patient without therapeutic paralysis; three patients were also measured under partial paralysis. Objective (2) was addressed by preceding each of the 36 unparalysed measurements with an assessment of cerebral function using the Glasgow coma scale (GCS); rank correlation was employed to test a null hypothesis that GCS and reflex indices are unrelated. RESULTS: In normal subjects, unconditioned reflex amplitude exhibited a positive skew requiring logarithmic transformation. Antagonist inhibition had a prolonged time course suggesting presynaptic mechanisms; subsequent measurements were standardised at 80 ms conditioning test interval (index termed "TI(80)"). Measurements were obtained on all patients, even under therapeutic paralysis (objective (1)). The unconditioned reflex was absent in most patients with GCS less than 5; otherwise it varied little across the patient group. TI(80) fell progressively with lower GCS, although patients' individual GCS could not be inferred from single measurements. Both reflex indices correlated with GCS (p<0.01), thereby dismissing the null hypothesis (objective (2)). CONCLUSION: Cerebral dysfunction in head injury is reflected in myotatic reflex abnormalities which can be measured at the bedside. With greater reproducibility, reflex measurements may assist monitoring of patients with head injury.

Adult↗

Abnormal H-reflexes in periodic limb movement disorder; impact on understanding the pathophysiology of the disorder.

OBJECTIVE: To get more insight in the pathophysiological basis of periodic limb movement disorder (PLMD) with or without restless legs syndrome (RLS), we investigated whether these patients have spontaneous changes in H-reflexes or show altered reflex patterns after (external) inhibition or excitation of the relevant spinal segment. METHODS: The ratio of the peak-to-peak values of the maximal soleus H-reflex and the maximal direct muscle potential (H/M ratio), H-reflex recruitment curves, vibratory inhibition and recovery curves of the soleus H-reflex in double stimulus experiments were measured in 9 PLMD patients and 11 controls. RESULTS: In comparison to controls the vibratory inhibition, predominantly reflecting pre-synaptic inhibitory action, was depressed in PLMD patients. The soleus H-reflex recovery curves showed increased late facilitation and depressed late inhibition, both reflecting diminished inhibition due to post-synaptic central activity. CONCLUSIONS: Our data indicate diminished inhibition at spinal level in PLMD patients. This is probably due to altered function of the descending spinal tracts, peripheral influence or changes at the inter-neural circuitry at spinal level itself, or combinations of these 3 possibilities. SIGNIFICANCE: The results of this study give further insight in the pathophysiology of PLMD and RLS by stressing the importance of diminished central inhibition.

Adult↗

Isolated posterior column dysfunction: an unusual presentation of spondylotic myelopathy.

Two patients with previously unreported clinically evident myelopathy caused by herniated intervertebral disks are described. These patients had isolated posterior column dysfunction in the lower extremities without motor deficits, abnormal reflexes, spinothalamic sensory abnormalities, or sphincter changes. The level of cord compression was significantly more cranial than suggested by clinical localization, and the results of initial radiographic studies were unremarkable. Study of posterior column dysfunction using somatosensory evoked potentials was helpful in localizing the lesions and guiding appropriate neuroradiologic studies.

Adult↗

Abnormal esophagocardiac inhibitory reflex in patients with diffuse esophageal spasm.

The aim of this study was to establish if patients with an esophageal dysmotility similar to that found in the majority of patients with 'swallow syncope' may also have a pathologic esophagocardiac reflex similar to that observed in these patients. The effects on heart rate induced by dry and solid swallows and by intraesophageal balloon distension were studied in 8 normal subjects and in 10 patients with diffuse esophageal spasm by simultaneous recording of ECG and intraesophageal pressures. Dry swallows induced a brief increase in heart rate, while deglutition of a solid bolus initially induced an increase followed by a decrease in heart rate, significantly more marked in patients with diffuse spasm. The intraesophageal balloon inflation induced a decrease in heart rate which was significantly more intense in patients with diffuse spasm. In conclusion, the esophageal wall distension either due to solid bolus ingestion or to balloon inflation elicits an inhibitory esophagocardiac reflex which is more intense in patients with diffuse spasm and might induce cardiac arrhythmias in predisposed subjects.

Adult↗

[Physical diagnostics--tendon reflexes].

Clinical examination of the tendon reflexes is helpful in localising lesions in the nervous system, which is an important step in the diagnosis of neurological disorders. The large intra- and interindividual variations of reflex amplitudes make interpretation difficult. Published interobserver agreement studies provide conflicting results. The diagnostic specificity of unequivocally abnormal reflexes is moderate to good (70-95%, decreasing with increasing age), but the sensitivity is lower (50-70%), both for peripheral and for central lesions. This implies that the finding of a clearly abnormal reflex pattern is often helpful, but the finding of normal reflexes usually does not exclude a neurological lesion.

Brain Diseases↗

H reflex studies in neurolathyrism.

Sixteen patients with lathyrism, age ranging between 18 and 55 years and duration of illness between 2 and 25 years, underwent H reflex studies with the aim of studying motor neurone excitability. The patients had marked spasticity (Ashworth score ranging between 2 and 5) and mild to moderate leg weakness. Knee and ankle reflexes were exaggerated in all and the plantar response was extensor in 14 patients. The H reflex abnormalities included increased HM ratio indicating increased motoneurone excitability, significant lack of vibratory inhibition indicating altered transmission in the premotoneuronal portion of the H reflex pathway, and lack of reciprocal inhibition (P < 0.01). These H reflex abnormalities were not related to spasticity, weakness, clonus or plantar response. The H reflex recovery curve in 6 patients revealed increased excitability throughout the recovery curve. The secondary facilitation started and peaked slightly earlier than normal, and the late depression was not marked indicating change in excitability of motoneurones or of interneurones.

Adolescent↗

Midbrain 6-hydroxydopamine lesions modulate blink reflex excitability.

The blink reflex abnormalities present in the 6 hydroxydopamine (6-OHDA) lesioned rat model of parkinsonism mimicked those of the human with Parkinson's disease. In alert rats, we monitored the long and short latency components of the orbicularis oculi electromyographic (OOemg) response evoked by electrical stimulation of the supraorbital branch of the trigeminal nerve (SO). Two paradigms, habituation and double pulse, provided a measure of blink reflex excitability. In normal rats, repeated stimulation of the SO produced habituation of the R2 component of the blink. In the double pulse paradigm, presentation of two identical SO stimuli resulted in a reduced or suppressed OOemg response to the second stimulus relative to the first. In rats with complete, unilateral lesions of midbrain dopamine neurons, repeated SO stimulation produced facilitation rather than habituation of the R2 component of the blink reflex. This facilitation occurred only with the eyelid contralateral to the lesion. In the double pulse paradigm, the lesioned rats showed increased excitability rather than suppression. This effect occurred bilaterally, although the increased excitability was strongest contralateral to the lesion. Rats with partial lesions of midbrain dopamine neurons exhibited qualitatively similar, but less pronounced blink reflex abnormalities. The R1 component of the blink reflex was unaffected by either the complete or partial lesions. Thus, modification of the blink reflex by 6-OHDA lesions provides a reproducible parkinsonian-like symptom which is amenable to investigations of increases in reflex excitability.

Amphetamine↗

Abnormal cardiovascular reflex tests are predictors of mortality in Type 2 diabetes mellitus.

AIMS: To determine whether diabetic autonomic neuropathy is an important factor contributing to mortality in Type 2 diabetes mellitus. METHODS: Between 1989 and 1993, 431 men and 181 women with Type 2 diabetes were given diabetic autonomic neuropathy cardiovascular reflex (CVR) tests. These subjects were followed for the subsequent 5--9 years to assess mortality rates. RESULTS: The prevalence rate of abnormal CVR tests was 46.1% in patients with the history of diabetes less than 5 years and up to 69.4% when the history of diabetes exceeded 20 years. During the follow-up period from 1 January 1989 to 31 December 1997 (mean 7.7 years), a total of 135 participants died. The 8-year survival rate for patients with abnormal CVR tests was 63.6% in males and 76.4% in females, compared with 80.9 and 93.3% for patients with normal CVR tests. The results were grouped as: group 1, normal CVR tests without postural hypotension (PHT); group 2, normal CVR tests with PHT; group 3, abnormal CVR tests without PHT; and group 4, abnormal CVR tests with PHT. The 8-year survival rate was 85.4% in group 1, 80.9% in group 2, 74.5% in group 3 and 61.1% in group 4. CONCLUSION: Type 2 diabetic patients with abnormal CVR tests may have increased mortality, and those combined with postural hypotension have higher mortality than those without. Abnormal CVR tests may be important predictors of mortality in Type 2 diabetes mellitus.

Blood Pressure↗

[Localization of level of lesions in internuclear ophthalmoplegia through assessment of masseter and blink reflex].

The masseter and blink reflexes were investigated in 100 patients with internuclear ophthalmoplegia due to multiple sclerosis (58 patients) or lacunar brainstem infarction (42 patients). In unilateral internuclear ophthalmoplegia, 38 of 60 patients (63.3%) had masseter reflex abnormalities, two patients (3.3%) showed changes of the blink reflex R1 component, and 13 patients (21.7%) combined alterations of the masseter reflex and the blink reflex R1 component. 46 (86.8%) of these 53 patients with electrophysiological abnormalities had unilateral changes, which were ipsilateral to the medial longitudinal fasciculus lesion in 42 patients (91.3%). In bilateral internuclear ophthalmoplegia, 24 of 40 patients (60.0%) had abnormalities of the masseter reflex, two (5.0%) showed changes of the blink reflex R1, and nine (22.5%) combined alterations of the masseter reflex and the blink reflex R1 component. 20 (57.1%) of these 35 patients with electrophysiological abnormalities had bilateral changes. Thus, masseter reflex abnormalities indicating midbrain lesions were seen in 63.3% and 60.0%, respectively, of unilateral and bilateral internuclear ophthalmoplegia. Blink reflex R1 component changes with or without impairment of the masseter reflex indicating rostral pontine to midpontine lesions occurred in 25.0% and 27.5%, respectively. These figures correspond to the results of postmortem examinations and to theoretical considerations based on the length of the medial longitudinal fasciculus.

Adult↗

Abnormal vascular reflexes in Charcot-Marie-Tooth disease.

Vascular reflexes were assessed in 17 adult patients with Charcot-Marie-Tooth disease using the Valsalva manoeuvre, and the pulse rate and systolic blood pressure responses to standing. Six patients showed abnormalities consistent with an autonomic neuropathy. One patient had giant nerve fibre bundles in the myenteric plexus of bowel resected for co-existent Crohn's disease, indicating histological involvement of these autonomic neurones. Other evidence of an autonomic component to the peripheral neuropathy of Charcot-Marie-Tooth disease is reviewed.

Adult↗

Vascular abnormalities in reflex sympathetic dystrophy (CRPS I): mechanisms and diagnostic value.

Complex regional pain syndrome type I (CRPS I, formerly known as reflex sympathetic dystrophy) is a painful neuropathic disorder that develops after trauma affecting the limbs without overt nerve injury. Clinical features are spontaneous pain, hyperalgesia, impairment of motor function, swelling, changes in sweating, and vascular abnormalities. In this study, the pathophysiological mechanisms of vascular abnormalities were investigated. Furthermore, the incidence, sensitivity and specificity of side differences in skin temperature were defined in order to distinguish patients with definite CRPS I from patients with extremity pain of other origin. In 25 CRPS I patients and two control groups (20 healthy subjects and 15 patients with other types of extremity pain), cutaneous sympathetic vasoconstrictor activity was altered tonically by the use of controlled thermoregulation. Whole-body temperature changes were induced with a thermal suit in which cold or hot water circulated. The vascular reflex response (skin blood flow, laser Doppler flowmetry, skin temperature, infrared thermometry) was analysed to quantify sympathetic outflow. Measurements were performed during a complete thermoregulatory cycle, i.e. during the entire spectrum of sympathetic vasoconstrictor activity from high (whole-body cooling) to low sympathetic activity (whole-body warming). Venous noradrenalin levels were determined bilaterally in five CRPS patients. (i) Three distinct vascular regulation patterns were identified related to the duration of the disorder. In the "warm" (acute) type of regulation, the affected limb was warmer and perfusion values were higher than in the contralateral limb during the entire spectrum of sympathetic activity. In the "intermediate" type of regulation the limb was either warmer or colder. In the "cold" (chronic) type of regulation, skin temperature and perfusion values were lower on the affected side during the entire spectrum of sympathetic vasoconstrictor activity. (ii) Noradrenalin levels were lower on the affected side, even in chronic patients with considerable cutaneous vasoconstriction. (iii) Temperature and blood flow differences between the two sides were dynamic and most prominent at a high to medium level of vasoconstrictor activity. (iv) In both control groups, there were only minor side differences in flow and temperature. In conclusion, it is suggested that, in CRPS I, unilateral inhibition of sympathetic vasoconstrictor neurones leads to a warmer affected limb in the acute stage. Secondary changes in neurovascular transmission may lead to vasoconstriction and cold skin in chronic CRPS I, whereas sympathetic activity is still depressed. Vascular abnormalities are dynamic. The maximal skin temperature difference that occurs during the thermoregulatory cycle distinguishes CRPS I from other extremity pain syndromes with high sensitivity and specificity.

Adult↗