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Thus it is more correct to think of the encephalopathy in terms of both hypoxia and ischemia, which usually occur in utero and are expressed postnatally by recognizable clinical syndromes. Fenichel, following the original work of Sarnat and Sarnat in 1976 and of Levene and colleagues, finds it helpful to divide the cerebral syndromes that follow a difficult birth into three groups, according to their severity, each having a greater prognostic value than the Apgar score: (1) In newborns with mild hypoxic-ischemic encephalopathy, the symptoms are maximal in the first 24 h and take the form of hyperalertness and tremulousness of the limbs and jaw (the "jittery baby") and a low threshold of the Moro reaction. The tone of the limbs is normal except for a mild increase in head lag during traction. After 48 to 72 h, the neonate may improve (having passed through a jittery hyperactive phase) or worsen, becoming less responsive in association with convulsions, cerebral edema, hyponatremia, and hyperammonemia from liver damage. The limbs are hypotonic and motionless even during attempts to elicit the Moro response. Sucking and swallowing are depressed or absent, but pupillary reactions and eye movements may at first be retained, only to be lost as the coma deepens. Included in the category of severe hypoxic-ischemic encephalopathy are also newborns with a variety of developmental anomalies of the brain and other organs. In addition, such infants may have been exposed to certain prenatal risk factors (toxemia of pregnancy, antepartum uterine hemorrhage), or their growth may have been abnormal (small-for-date babies). Some are born at term; others are premature, and the birth process may or may not have been abnormal. One must then consider the possibility, originally pointed out by Sigmund Freud, that the abnormality of the birth process, instead of being causal, was actually the consequence of prenatal pathology. Other evidence of multifactorial etiology in the "causation" of cerebral palsy has been provided by Nelson and Ellenberg, who found that maternal mental retardation, birth weight below 2000 g, and fetal malformation were among the leading predictors. Breech presentation was another factor, and one-third of these cases also had some noncerebral malformation. Twenty-one percent of the 189 children in their series had also suffered some degree of asphyxia. Additional determinants were maternal seizures, a motor deficit in an older sibling, two or more prior fetal deaths, hyperthyroidism in the mother, pre-eclampsia, or eclampsia. In children with cerebral diplegia born at term, likely contributory factors that were operative in nearly half included toxemia of pregnancy, low birth weight for age, placental infarction, and intrauterine asphyxia. The factors enumerated above are involved to different degrees in the outcome of pregnancies but are informative because they bring to light the significant proportion of cases of cerebral birth injury in which hypoxia-ischemia, matrix hemorrhages, and leukomalacia were not operative. In this group, we would also include the symmetrical porencephalies and hydranencephalies. This statement has been amply confirmed by a large and often cited study from Western Australia that detected neonatal encephalopathy in 3.
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Volume of muscle is diminished but is difficult to evaluate in the infant because of the coverings of adipose tissue. Perception of tactile and painful stimuli is undiminished, and emotional and social development measure up to age. As the months pass, the weakness and hypotonia progress gradually and spread to all of the skeletal muscles except the ocular ones. Respiratory movements become paradoxical (abdominal protrusion with chest retraction). Such infants are unable to sit unless propped, and they cannot hold up their heads without support. Their posture is characteristic: arms abducted and flexed at the elbow, legs in the "frog position" with external rotation and abduction at hips and flexion at hips and knees. Until late in the illness, these children appear bright-eyed, alert, and responsive. Infants in whom the disease becomes apparent only after several months of life have a less rapid decline than those affected in utero or at birth. Some of the former become able to sit and creep and even to walk with support; those with later onset may survive for several years and even into adolescence or early adult life, as already mentioned. Motor unit potentials are diminished in number and, in the more slowly evolving cases, some are larger than normal (giant or polyphasic potentials reflecting reinnervation). Motor nerve conduction velocities are normal or fall in the low-normal range (these are normally slower in infants than in adults). Pathologic Findings Muscle biopsy after 1 month of age reveals a typical picture of group atrophy; shortly after birth this change is difficult to discern. Aside from denervative atrophy, the essential abnormalities are in the anterior horn cells in the spinal cord and the motor nuclei in the lower brainstem. Nerve cells are greatly reduced in number, and many of the remaining ones are in varying stages of degeneration; a few are chromatolytic and contain cytoplasmic inclusions. Other systems of neurons, including the corticospinal and corticobulbar systems, remain intact. Differential Diagnosis the major problem in diagnosis is to distinguish Werdnig-Hoffmann disease from an array of other diseases that cause hypotonia and delayed motor development in the neonate and infant. The list of disorders that imitates spinal muscular atrophy constitutes a large part of the differential diagnosis of the so-called floppy infant. The preservation of tendon reflexes and relative lack of progression of muscle weakness distinguish the latter disorders. Because of the gravity of the diagnosis, muscle biopsy should be performed if there is any suspicion of spinal muscular atrophy. Clinical disorders more or less similar to the spinal muscular atrophies may be identified occasionally in certain hereditary metabolic diseases. A progressive motor neuron or motor nerve disorder has also been observed in glycogen storage disease affecting anterior horn cells. Motor nerve fibers also suffer damage in metachromatic and globoid body leukoencephalopathies, and this may occur in adults, in association with paraproteinemia and multiple myeloma and as a paraneoplastic process.
In our opinion, the block in axonic flow alone could not account for the marked congestion of vessels and hemorrhages that accompany papilledema. The mechanism of papilledema that on rare occasions accompanies spinal tumors, particularly oligodendrogliomas, and the Guillain-Barre syndrome is not entirely clear. Puzzling are cases of papilledema without raised intracranial pressure, as may occur in children with cyanotic congenital heart disease and other forms of polycythemia and possibly with hypocalcemia. Diseases of the Optic Nerves the optic nerves, which constitute the axonic projections of the retinal ganglion cells to the lateral geniculate bodies and superior colliculi (the third visual neurons), can be inspected in the optic nerve head. They may reflect the presence of raised intracranial pressure (papilledema or "choked disc"), as already described; optic neuritis ("papillitis"); infarction of the optic nerve head; congenital defects of the optic nerves (optic pits and colobomas); hypoplasia and atrophy of the optic nerves; and glaucoma. Illustrations of these and other abnormalities of the disc and ocular fundus can be found in the atlas by E. The main causes of visual loss from optic neuropathy are listed in Table 13-2 and discussed in the following portions of this chapter. The most frequent situation is one in which an adolescent or young adult (rarely a child) notes a rapid diminution of vision in one eye (as though a veil had covered the eye), sometimes progressing within hours or days to complete blindness. The optic disc and retina may appear normal (retrobulbar neuritis), but if the inflammation is near the nerve head, there is swelling of the disc, i. As indicated above, papillitis is associated with marked impairment of vision and a scotoma, thus distinguishing it from the papilledema of increased intracranial pressure. Pain on movement and tenderness on pressure of the globe and a difference between the two eyes in the perception of brightness of light are other fairly consistent findings (Table 13-1). The patient may report an increase in blurring of vision with exertion or following a hot bath (Uthoff phenomenon). If there is papillitis, examination may disclose variable haziness of the vitreous that causes difficulty in visualizing the retina. Inflammatory sheathing of the retinal veins, as described by Rucker, is known to occur but has been an uncommon finding in our patients. In extreme cases, edema may suffuse from the disc to cause a rippling in the adjacent retina. However, as just noted, most cases of optic neuritis are retrobulbar and little is to be seen when examing the optic nerve head. In about 10 percent of cases, both eyes are involved, either simultaneously or in rapid succession. In a proportion of such patients no cause of the optic neuropathy can be found, but a first bout of multiple sclerosis is always suspected, as discussed in Chap. After several weeks there is spontaneous recovery; vision returns to normal in more than twothirds of instances. Regression of symptoms may occur spontaneously or may be hastened by the intravenous administration of high doses of corticosteroids, but in one study the oral administration of the same drugs increased the frequency of a relapse of optic neuritis (page 788). The optic disc later becomes slightly pale, especially on the temporal side, and the pallor extends beyond the margins of the disc into the peripapillary retinal nerve fiber layer.
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They are the most effective agents available when given in an appropriate dose by an appropriate route. The basic principle in dosing these drugs is that they should be titrated in small increments to point of effect or side effect, whichever comes first. Morphine is the standard of this class to which all other derivatives are compared for potency and effect. Meperidine is also commonly used, whereas hydromorphone and fentanyl are occasionally given. Whichever is chosen, the intravenous route is the safest and most effective route for providing adequate analgesia. Hydroxyzine is frequently given along with a narcotic to relieve opiate-induced nausea. Benzodiazepines: Many practitioners prefer a benzodiazepine in addition to or in place of narcotics for the reduction of major dislocations. These drugs possess significant sedative, anxiolytic, and amnestic properties that make them useful for this purpose. They are not true skeletal muscle relaxants, but they do effectively relieve muscle spasm. Midazolam is particularly useful because of its rapid onset of action and short half-life. One must be prepared to provide ventilatory support when this drug is used, especially in combination with a narcotic. Primer for Emergency Medicine Students Local and Regional Anesthesia: Nerve blocks with lidocaine or a related drug can be useful in reducing a number of fractures and dislocations, especially of the wrists, hands, and feet. Recently it has been shown that addition of sodium bicarbonate to lidocaine (1 ml sodium bicarbonate, 1 mEq/ml per 10 ml 1% lidocaine added immediately before injection) can greatly reduce the pain associated with the injection. Nitrous Oxide: Nitrous oxide, inhaled as a 50:50 mixture with oxygen has the advantages of safety, rapid onset and recovery time, and ease of administration. Muscle Relaxants: Muscle spasm may occur as an isolated phenomenon associated with strains or may accompany ligamentous injuries, overuse injuries, radiculopathy, or nervous tension. A number of drugs-including orphenadrine citrate (Norflex), methocarbamol (Robaxin), diazepam (Valium), carisoprodol (Soma), and cyclobenzaprine hydrochloride (Flexeril)-have been promoted as skeletal muscle relaxants. All of these produce drowsiness, and it is likely that most of their benefit is achieved by sedation and forced rest. The incidence of gastrointestinal, hepatic, renal, and hypersensitivity complications is not insignificant and needs to be monitored. Aspirin is the cheapest and oldest member of this group, and its use as an agent of first choice should be strongly considered.
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This takes the form of some combination of spinal arachnoiditis with ataxic paraparesis and sensory disturbance, hydrocephalus, or opticochiasmatic arachnoiditis. The point to be made is that there is always some risk attached to the subarachnoid instillation of any foreign agent. Opticochiasmatic Arachnoiditis this condition was well known to neurologists during the period when neurosyphilis was a common disease. It occurs after years of chronic syphilitic meningitis, sometimes in conjunction with tabes dorsalis or meningomyelitis. However, there were always nonsyphilitic cases, the cause of which was never ascertained. A constriction of visual fields, usually bilateral and asymmetrical (rarely scotomas), developed insidiously and progressed. Pathologically, the optic nerves were found to be enmeshed in thickened, opaque pia-arachnoid. Pachymeningitis the term pachymeningitis refers to a chronic, circumscribed, inflammatory thickening of the dura. The term is somewhat confusing insofar as the pia and arachnoid are usually equally involved in the inflammatory thickening and all three membranes are bound together by dense fibrous adhesions. This type of meningeal reaction, which is now exceedingly uncommon, was first described by Charcot and Joffroy. It occurred mainly in the cervical region (hence the name pachymeningitis cervicalis hypertrophica) and was attributed to syphilis. Involvement of cervical roots and compression of the spinal cord gave rise to variable degrees of paraparesis in association with root pain, paresthesias, sensory loss, and amyotrophy of the upper limbs. In the modern era, rheumatoid arthritis, sarcoidosis, and chronic local infection (fungal, tuberculous) have been the main causes, but some of the cases remain unexplained. Idiopathic instances continue to be reported; a summary of published cases and two personally studied ones is given by Dumont and colleagues. The subdural space and dura can be involved by extension of a pathologic process from the arachnoid, especially in infants and children, in whom subdural hygromas regularly follow meningitis. The fibrous connective tissue of which the dura is composed may also undergo pronounced thickening in the course of a mucopolysaccharidosis, especially in cases where fibroblasts are implicated. Other Abnormalities of the Ependyma and Meninges Although the ependyma may be involved as part of any chronic meningeal reaction, it may also be the site of a relatively isolated process. In experimental animals, Johnson and colleagues found that the mumps virus could localize in and destroy ependymal cells.
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The new generations of Quicklot products are now impregnated with an inert mineral called kaolin which contains no animal or human proteins or botanicals and is unlikely to cause any adverse proteinmediated reactions. To enable good haemorrhage control of deep or cavitating wounds products such as QuikClot combat gauze, or Celox Rapid Unlike a haemorrhage in the arm or leg where a tourniquet can be used to shut off bleeding, there was no way until recently to do the same for wounds in the lower torso, which can kill a person in a matter of minutes. It has also been used to stem major haemorrhage from a gunshot wound to the axilla. Once again, we have only limited anecdotal experience of these devices at present. We have tried these as a Faculty on test subjects and in our limited experience with healthy volunteers with normal blood pressures, the device was ineffective. At present we are unable to recommend this device until further studies are performed and usage data is collected. There are also pellets which will hugely expand to fill any potential space, such as a wound cavity. These have been tried with and without haemostatics in early trials and they appear to work just as well with or without suggesting that the pressure effect of the expanding pellets is the key. Practically the pellets come in an applicator, which is plastic and quite bulky, which theoretically allows delivery deep into a penetrating wound. Although there were initially some concerns about ongoing concealed bleeding within the wound cavity and lack of actual pressure effect, there are some small studies that already suggest that for the right wounds this is an effective device. It has been suggested that the wound could be packed or filled with haemostatic and then held closed with the itclamp for good effect, but once again we are unaware of any case reports or recorded use in this manner. Anecdotal individual case reports suggest that they are useful for many wounds, especially on the scalp, but also even larger vessels in marginal areas. This has been 141 demonstrated in pigs and there is increasing human work, which includes reports of the device not being actually that painful to have applied (quoted at 4/10 for a deep groin wound in one report). Next Steps So what action can be taken once the obvious external bleed has been managed and things are just getting worse? Consider the mechanism and the findings during the primary survey, has anything alerted you to the possibility of massive internal bleeding? If so then at this point the only thing that will save this patients life is surgery and this is not going to happen at the roadside. What is needed now is rapid transportation to definitive care and at this point definitive care is a major trauma centre and with the relevant specialties to deal with this patient. Securing airway patency and protection is an essential skill in caring for the multiply injured or severely ill patient.
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It is worth while to not that if a limb has gotten to the point of pain, pallor, pulselessness, paresthesias, and paralysis major damage has already occurred. Likewise, in the unconscious, multiply traumatized patient, major vascular injuries may not be obvious. Therefore, mechanism of injury and anatomy must dictate the need to assess a possibly injured vessel. If pulses cannot be palpated, a Doppler stethoscope should be used to listen for blood flow. Even palpable pulses may be misleading, however, since it has been demonstrated that in 10% to 15% of significant arterial injuries, distal pulses may initially be normal. When pulses are present but the mechanism of injury suggests the possibility of vascular injury, arteriography should be performed. Late complications of undiagnosed vascular injuries include thrombosis, arteriovenous fistulas, aneurysm, false aneurysm, and tissue ischemia with limb dysfunction. Nerve Injuries: Nerves can be injured by both blunt and penetrating trauma or iatrogenically. Neuropraxia is the contusion of a nerve, with disruption of the ability to transmit impulses. Since the Schwann tubes remain in continuity, spontaneous healing is possible but slow. Certain nerve injuries characteristically accompany particular fractures Orthopedic injury Elbow injury Shoulder dislocation Sacral fracture Acetabulum fracture Hip dislocation Femoral shaft fracture Knee dislocation Lateral tibial plateau fracture Nerve injury Median (esp. Two-point discrimination is a more sensitive examination and should be used routinely in evaluating digital nerves. In the young child, examinations that require a subjective response may be misleading. If doubt exists about a digital nerve injury in a child, soak the digit in a basin of saline for 20 or 30 minutes. If the skin wrinkles, the digital nerve is intact, whereas absence of wrinkling indicates a nerve injury. Compartment Syndrome: this serious complication should be considered whenever pain and paresthesias occur in an extremity following a fracture that occurs in a closed muscular compartment. Pain will be increased with passive stretching of the involved muscles, with active contraction against resistance, or with direct pressure on the compartment. Compartment syndrome is a surgical emergency with the most common site being the anterior compartment of the lower leg. Other sites of Primer for Emergency Medicine Students compartment syndrome include the posterior, deep posterior and lateral compartments of the lower leg, the gluteal compartments, dorsal and volar compartments of the forearm and the interosseous compartments of the hand.
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Motor paralysis is variable; with partial recovery, there may be a clumsiness that resembles cerebellar ataxia. Since the sensory disorder simulates that due to a thalamic lesion, it was called pseudothalamic by Foix and coworkers. The pseudothalamic syndrome was related by Foix and colleagues to a sylvian infarct; Bogousslavsky and associates have traced it to a parietal infarct due to occlusion of the ascending parietal branch of the middle cerebral artery. In each of the aforementioned parietal lobe syndromes, if the dominant hemisphere is involved, there may be an aphasia, a bimanual tactile agnosia, or a Gerstmann syndrome; with nondominant lesions, there may be anosognosia (page 401). A lesion confined to only a part of the parietal cortex (the best examples have been due to glancing bullet or shrapnel wounds of the skull) may result in a circumscribed loss of Diagnosis of Somatosensory Syndromes Affirmation of the clinical sensory syndromes is often possible by the application of electrophysiologic testing. Slowing and reduced amplitude of sensory nerve conduction is found with lesions of nerve, but only if the lesion lies distal to (or within) the sensory ganglion. Severe sensory loss in a neuropathic pattern with preserved sensory nerve action potentials therefore indicates a radiculopathy. Loss or slowing of H and F responses corroborates the presence of lesions in proximal parts of nerves, plexuses, and roots. By the use of somatosensory evoked potentials, it is possible to demonstrate slowing of conduction in the peripheral nerves or roots, in the pathways from spinal cord to a point in the lower medulla, in the medial lemniscus to the thalamus, and in the pathway from the thalamus to the cerebral cortex. In the context of regional sensory loss, evoked potentials find their greatest utility in demonstrating root disease when sensory nerve conduction studies are normal; otherwise, they are used most frequently to support the diagnosis of multiple sclerosis, in which case there may or may not be corresponding sensory features. With single peripheral nerve lesions, touch and pinprick testing are the most informative. With spinal cord disease, pinprick and thermal stimuli are most revealing of lateral column lesions; testing the senses of vibration, position, and movement, and particularly the sense of direction of a dermal stimulus, reliably indicates posterior column lesions. In brainstem lesions, all modes of sensation including touch may be affected, and this applies in general to thalamic lesions. Thus, one is guided in the selection of tests by the suspected locale of the disease. In fact, there are so many cases of headache that special headache clinics have been established in many medical centers. Insofar as many headaches are due to medical rather than neurologic diseases, the subject is the legitimate concern of the general physician. Yet there is always the question of intracranial disease, so that it is difficult to approach the subject without a knowledge of neurologic medicine. For one thing, the face and scalp are more richly supplied with pain receptors than many other parts of the body, perhaps in order to protect the precious contents of the skull. Also, the nasal and oral passages, the eye, and the ear- all delicate and highly sensitive structures- reside here and must be protected; when afflicted by disease, each is capable of inducing pain in its own way. Finally, for the intelligent person, there is greater concern about what happens to the head than to other parts of the body, since the former houses the brain, and headache frequently raises the specter of brain tumor or other cerebral disease. Semantically, the term headache should encompass all aches and pains located in the head, but in practice its application is restricted to discomfort in the region of the cranial vault.