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Program Director, Albert Einstein College of Medicine
Although this benign familial polymyoclonia has not been associated with any biochemical abnormality arrhythmia young quality 2mg terazosin, its association with cellular mitochondrial abnormalities in some cases justifies its inclusion in this chapter rather than with the degenerative diseases blood pressure chart hospital generic terazosin 5 mg mastercard. The mitochondrial diseases as a group are considered in the last part of this chapter pulse pressure 61 cheap 2 mg terazosin amex. When the parkinsonian syndrome or some component thereof has its onset in middle or late adult life blood pressure medication used for nightmares buy terazosin 1 mg amex, it usually indicates idiopathic Parkinson disease or related multisystem forms of striatonigral degeneration. The development of such an extrapyramidal motor disorder in late childhood and adolescence instead suggests Wilson disease, Hallervorden-Spatz disease, and the Segawa type of L-dopa-responsive dystonia as well as other so-called Parkin mutations (see Chap. A similar neurologic disorder had been described previously by Gowers (1906) under the title of "tetanoid chorea" and by Westphal (1883) and Strumpell (1898), as "pseudosclerosis. Interestingly, none of these authors, including Wilson, noticed the goldenbrown (Kayser-Fleischer) corneal ring, the one pathognomonic sign of the disease. The corneal abnormality was first described by Kayser in 1902, and in the following year Fleischer related it to pseudosclerosis. In 1952, Scheinberg and Gitlin discovered that ceruloplasmin, the serum protein that binds copper, is reduced in this disease (see reviews by Scheinberg and Sternlieb for a full historical account and references). The prevalence of the disease cannot be stated exactly but is on the order of 1 per 50,000 to 1 per 100,000 of the general population. Siblings of a patient with Wilson disease have a 1 in 4 risk of developing the disease. The disease is transmitted as an autosomal recessive trait, and the abnormal gene resides on chromosome 13, in the region 13q14. One of the curious aspects of the genetics of the disease is the multitude of mutations within this gene that give rise to the disease, almost akin to an allelic variant at a normal site; and, no one mutation accounts for more than 30 percent of cases. Inadequate functioning of this enzyme in some way reduces excretion of copper in the bile. As noted further on, liver transplantation halts progression of the disease, indicating that the primary biochemical effect of the mutation is in the liver rather than the nervous system. The genetic defect gives rise to two fundamental disturbances of copper metabolism- a reduced rate of incorporation of copper into ceruloplasmin and a reduction in biliary excretion of copper. The deposition of copper in tissues is the cause of virtually all the manifestations of the disease- cirrhosis, hemolytic anemia, renal Epilepsies of Hereditary Metabolic Disease (See Chap. The seizures may occur at all ages but more frequently in the neonate, infant, or young child than in the older child or adolescent. Most often they are generalized grand mal or partial types; typical petit mal probably does not occur. Some diseases may cause focal seizures, simple or complex partial, before becoming generalized. The combination of series of polymyoclonic jerks progressing to a generalized motor seizure is always highly suggestive of one of the hereditary metabolic diseases. The subject of epilepsy and the hereditary metabolic diseases has recently been reviewed by Sansaricq and colleagues. Clinical Features the onset of neurologic symptoms is usually in the second and less often in the third decade, rarely beyond that time. Half of patients are symptomatic by age 15, but exceptional cases, including two under our care, had their first clinical manifestations as late as their mid-fifties. In all instances the initial event is a deposition of copper in the liver, leading to an acute or chronic hepatopathy and eventually to multilobular cirrhosis and splenomegaly (Scheinberg and Sternlieb). In childhood, the liver disorder often takes the form of attacks of jaundice, unexplained hepatosplenomegaly, or hypersplenism with thrombocytopenia and bleeding. The hepatic abnormalities may be asymptomatic (except for elevated serum transaminases), in which case the initial clinical presentation is neurologic. In some instances, a hemolytic anemia or, less often, renal tubular acidosis may first draw attention to the disease. The first neurologic manifestations are most often extrapyramidal with a proclivity to affect the oropharyngeal musculature. The typical presentations are tremor of a limb or of the head and generalized slowness of movement. Exceptionally, an abnormality of behavior (argumentativeness, impulsiveness, excessive emotionality, depression, delusions) or a gradual impairment of intellectual faculties precedes other neurologic signs by a year or more (see Starosta-Rubinstein et al). As the disease progresses, the "classic syndrome" evolves: dysphagia and drooling, rigidity and slowness of movements of the limbs; flexed limb postures; fixity of facial muscles with mouth constantly agape, giving an appearance of grinning or a "vacuous smile"; dysarthria or virtual anarthria (bulbar extrapyramidal syndrome); and a tremor in repose that increases when the limbs are outstretched to a coarse, "wing-beating" movement.
Sensory nerve action potentials should be normal; tests of motor nerve conduction have a normal velocity blood pressure chart stage 2 2 mg terazosin mastercard, but the amplitudes become progressively lower as the disease progresses- in the earliest stages arterial bleeding effective terazosin 1mg, they too may be normal blood pressure low generic terazosin 1 mg otc. When in a typical case the amplitudes of sensory nerve action potentials are reduced heart attack cpr cheap 5mg terazosin mastercard, there is usually an underlying entrapment neuropathy, diabetes, or late-life neuropathy. Sensory evoked potentials are mildly abnormal in a proportion of patients, but the explanation for this finding is obscure. Serum creatine kinase is moderately elevated in patients with rapidly progressive atrophy and weakness, but it is just as often normal. Motor evoked potentials elicited from the cortex are also prolonged in patients with prominent corticospinal signs. In addition to neuronal loss, there is evidence of slight gliosis and proliferation of microglial cells. Many of the surviving nerve cells are small, shrunken, and filled with lipofuscin. It is not uncommon to also detect ubiquitin inclusions in threads, skeins, or dense aggregates within the affected neurons by special stains. According to some reports, swelling of the proximal axon is an early finding, presumably antedating visible changes in the cell body itself. The anterior roots are thin, and there is a disproportionate loss of large myelinated fibers in motor nerves (Bradley et al). Whitehouse and coworkers found a depletion of muscarinic, cholinergic, glycinergic, and benzodiazepine receptors in regions of the spinal cord where motor neurons had disappeared. The corticospinal tract degeneration is most evident in the lower parts of the spinal cord, but it can be traced up through the brainstem to the posterior limb of the internal capsule and corona radiata by means of fat stains, which show the macrophages that accumulate in response to myelin degeneration. Other fibers in the ventral and lateral funiculi are depleted, imparting a characteristic pallor in myelin stains. Motor system disease beginning in the proximal limb muscles may be misdiagnosed as a limb-girdle type of muscular dystrophy. The main considerations in relation to progressive bulbar palsy are myasthenia gravis and, less often, polymyositis, muscular dystrophy, and especially the inherited (Kennedy) type of bulbospinal atrophy, which is discussed further on. The spastic form of bulbar palsy may suggest the pseudobulbar palsy of lacunar disease. There is also a rare form of subacute poliomyelitis (possibly viral) in patients with lymphoma or carcinoma; it leads to an amyotrophy that progresses to death over a period of several months. For further discussion of the paraneoplastic variety of motor system disease, see page 585. Infrequently, we have seen myelopathic motor findings and motor radiculopathy with vitamin B12 deficiency, and there are exceptional reports of myeloradiculopathy with lead poisoning; we sometimes include tests for these conditions. However, we regard this condition of more diffuse pallor as due to a loss of collaterals of motor neurons that contribute to the lamina propria. Over the years, the authors have occasionally encountered young men with localized and asymmetrical amyotrophy of the leg or arm that became arrested and did not advance over a decade or two. Several reports of such a partial spinal amyotrophy have appeared in recent years (Hirayama et al; Moreno Martinez et al). In the type described by Hirayama, young men are affected with progressive and asymmetrical amyotrophy of the forearm and hand that has been traced to ligamentous hypertrophy in the ventral spinal canal. This causes a compression of the cervical spinal cord, presumably a chronic ischemic effect (page 1077). In a familial variety of pure restricted amyotrophy, only the vocal cords became paralyzed over a period of years in adult life; only later were the hands affected. Some patients who have recovered from paralytic poliomyelitis may develop progressive muscular weakness 30 or 40 years later; the nature of this relationship is obscure. We favor the explanation that atrophy of anterior horn cells with aging brings to light a critically depleted motor neuron population (see further on). A number of cases of this type have been discovered in Ashkenazi Jews by the use of lysosomal enzyme analysis. An even rarer and recessively inherited childhood form of motor neuron disease (affecting corticospinal more than spinal motor neurons) has been attributed to mutations in a gene whose protein (alsin) is a component of the neuronal cell-signaling pathways.
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The authors have not observed such a relationship between coma and delirium except possibly in patients suffering from bacterial meningitis arrhythmia yawning terazosin 2 mg line, certain drug intoxications or withdrawal states blood pressure for teens purchase 2 mg terazosin overnight delivery, or hepatic stupor and coma pulse pressure low values order 2mg terazosin amex, which in some few instances may be preceded by a brief period of delirium blood pressure causes order 2mg terazosin with mastercard. Drowsiness and Stupor In these states, mental, speech, and physical activity are reduced. Drowsiness denotes an inability to sustain a wakeful state without the application of external stimuli. The lids droop without closing completely; there may be snoring, the jaw and limb muscles are slack, and the limbs are relaxed. This state is indistinguishable from light sleep, with slow arousal elicited by speaking to the patient or applying a tactile stimulus. Stupor describes a state in which the patient can be roused only by vigorous and repeated stimuli, at which time he opens his eyes, looks at the examiner, and does not appear to be unconscious; response to spoken commands is either absent or slow and inadequate. Restless or stereotyped motor activity is common in stuporous patients and there is a reduction in the natural shifting of positions. When left unstimulated, these patients quickly drift back into a sleep-like state. The eyes move outward and upward, a feature that is shared with sleep (see further on). Tendon and plantar reflexes and breathing pattern may or may not be altered, depending on how the underlying disease has affected the nervous system. In psychiatry, the term stupor has been used in a second sense- to denote an uncommon condition in which the perception of sensory stimuli is presumably normal but activity is suspended and motor activity is profoundly diminished (catatonic stupor). Coma the patient who appears to be asleep and is at the same time incapable of being aroused by external stimuli or inner need is in a state of coma. There are variations in the degree of coma; in its deepest stages, no reaction of any kind is obtainable: corneal, pupillary, pharyngeal, tendon, and plantar reflexes are in abeyance, and tone in the limb muscles is diminished. With lesser degrees of coma, pupillary reactions, reflex ocular movements, and corneal and other brainstem reflexes are preserved in varying degree, and muscle tone in the limbs may be increased. Respiration may be slow or rapid, periodic, or deranged in other ways (see further on). In still lighter stages, sometimes referred to by the ambiguous term semicoma, most of the above reflexes can be elicited, and the plantar reflexes may be either flexor or extensor (Babinski sign). Moreover, vigorous stimulation of the patient or distention of the bladder may cause a stirring or moaning and a quickening of respiration. For example, patients with alcoholic coma may be areflexic and unresponsive to noxious stimuli, even when respiration and other vital functions are not threatened. The depth of coma and stupor, when compared in serial examinations, is most useful in assessing the direction in which the disease is evolving. Relationship of Sleep to Coma Persons in sleep give little evidence of being aware of themselves or their environment; in this respect they are unconscious. Sleep shares a number of other features with the pathologic states of drowsiness, stupor, and coma. These include yawning, closure of the eyelids, cessation of blinking and swallowing, upward deviation or divergence or roving move- ments of the eyes, loss of muscular tone, decrease or loss of tendon reflexes, and even the presence of Babinski signs and irregular respirations, sometimes Cheyne-Stokes in type. Upon being awakened from deep sleep, a normal person may be confused for a few moments, as every physician knows. Nevertheless, sleeping persons may still respond to unaccustomed stimuli and at times are capable of some mental activity in the form of dreams that leave traces of memory, thus differing from persons in stupor or coma. The most important difference, of course, is that persons in sleep, when stimulated, can be roused to normal consciousness. Cerebral oxygen uptake does not decrease during sleep, as it usually does in coma. The Persistent Vegetative and Minimally Conscious States, Locked-in Syndrome, and Akinetic Mutism With increasing refinements in the treatment of severe systemic diseases and cerebral injury, more and more patients who formerly would have died have survived for indefinite periods without regaining any meaningful mental function. For the first week or two after the cerebral injury, these patients are in a state of deep coma.
The term was used further during World War I by French doctors treating the battlefield wounded at the aid stations behind the front blood pressure herbs generic terazosin 1mg on-line. This rations patient treatment efficiently when resources are insufficient for all to be treated immediately blood pressure levels vary discount terazosin 5mg overnight delivery. Triage may result in determining the order and priority of emergency treatment blood pressure 4060 discount terazosin 1mg mastercard, the order and priority of emergency transport prehypertension jnc 7 generic 5 mg terazosin mastercard, or the transport destination for the patient. The categorisations of the victims are frequently the result of triage scores based on specific physiological assessment findings. As triage concepts become more sophisticated, triage guidance is also evolving into both software and hardware decision support products for use by caregivers in both hospitals and the field. If a casualty has massive haemorrhage, a tourniquet is applied where applicable and then the assessor simply moves on to the next casualty. These people are not breathing and an effort to reposition their airway has been unsuccessful. These people are in critical condition and would die without immediate assistance. Vehicles are now designed to absorb energy and redirect forces around the passenger call. There are usually a minimum of six airbags in new vehicles and the use of boronated steel makes the structure much stiffer and stronger yet much lighter. Despite these advances, high speed collisions still lead to occupants being trapped and suffering major polytrauma. The extrication process is a complex mixture of medical and technical expertise and requires a high degree of on scene communication and dynamic planning. In the most severe collisions of course; the entrapment will be both physical and medical meaning that space will have to be created by the technical rescue team (usually the fire and rescue service). This is a six phase process: the vehicle will be stabilised in order to prevent unnecessary movement and assist medical interventions. This is called the "B-Plan" Hydraulic rescue equipment will be used Glass will be systematically removed to reduce the hazard to gain access, move vehicle construction and often remove the roof for full access. The glass will be systematically removed and controlled to reduce the hazard on scene as well as allowing access into the vehicle for personnel and equipment 99 5) Full Access Medics Role In most collision situations the technical rescue team (fire and rescue service) will be responsible for formulating the Extrication Plan. This can only be contrived by gathering critical information from the casualty via the medic. In addition to the medical information, the technical rescuers will assess the vehicle and decide (with medical advice) on the best extrication route. This will, where possible, be the most sympathetic way of extricating the casualty from the vehicle in the least amount of time. It is possible that a medic arrives on scene An example of full access Full access such as a roof removal gives rescuers more access, making rescue safer, easier and quicker. However it may be that medical personnel arrive on scene after the technical rescue has begun. Communication the importance of on scene communication cannot be over emphasised, this is principally due to safety, but moreover it ensures a timely, effective and casualty centred rescue.
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