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دکتر سعید تیموری متخصص طب کار وبیماریهای شغلی مدیریت درمان تامین اجتماعی اصفهان درمانگاه قدس عباس اباد INTRUDUCTION Chemical agents capable of damaging the (CNS) are ubiquitous in the environment. Industrial processes are notorious sources of the most well known of these neurotoxins, which contaminate both the worksite and the surrounding environment. The United States Environmental Protection Agency lists over 65,000 chemicals currently used in the US , adding 2000–3000 new ones each year. INTRUDUCTION Despite the presence of selective permeability barriers metals, gases, solvents, and other chemicals penetrate sufficiently to cause deleterious effects. There are many historical descriptions of neurotoxicity; for example, lead poisoning ; homicidal use of arsenic , and the Minamata Bay epidemic (organic mercury) and glue-sniffer's neuropathy (hexacarbons). INTRUDUCTION Neurotoxicity is apparent when intense high level exposures result in acute illness. There is increasing evidence linking chronic low-level exposures with neurodegenerative diseases. GENERAL PRINCIPLES With few exceptions, pathophysiology of most neurotoxins is not well understood The effects of toxins on the CNS are more complex than on the peripheral nervous system. GENERAL PRINCIPLES The level and duration of exposure , physiologic variables such as the subject's age influence the clinical manifestations. A well-known example is lead toxicity, which may lead to an acute confusional state, chronic mental slowing, or a peripheral neuropathy. Neurotoxins There is evidence that the young are more susceptible than adults to the neurotoxic )lead, mercury, organophosphate insecticides,( Children absorb a larger dose per unit of body weight of toxins through the gastrointestinal and respiratory tracts than adults(lead) Nervous Susceptibility Factors The elderly are more susceptible to some neurotoxic effects than are young individuals. Examples sensitivity to tardive dyskinesia after neuroleptic use and to neuroleptic malignant There is evidence that senescent loss in neurons causes increased vulnerability to neurotoxicants. Neurologic symptoms and signs Symptoms and deficits depend on which groups of brain or spinal cord neurons are affected primarily. The most common syndrome is an encephalopathy from diffuse dysfunction of cortical or subcortical structures. The manifestations are neuropsychiatric A cute encephalopathy associated with alteration in the level of consciousness. Neurologic symptoms and signs Some toxins cause relatively selective injury to the vestibular system or the cerebellum, resulting in dysequilibrium, vertigo, and gait or limb ataxia. Basal ganglia involvement may lead to an extrapyramidal syndrome of bradykinesia, tremors, and rigidity . This may resemble idiopathic Parkinson disease for all practical purposes. Neurologic symptoms and signs Peripheral nervous system disorders lead to sensory disturbances and weakness, often accompanied by impairment of the deep tendon reflexe Neurologic symptoms and signs The hallmark of most polyneuropathies is the distal distribution . The most common syndrome is subacute onset of tingling or numbness experienced in a symmetrical, stockingand-glove distribution. Neuropathic pain is present, and is described variously as burning, deep aching, or lancinating Neurologic symptoms and signs Involvement of the motor nerve fibers manifests as muscle atrophy and weakness. These deficits appear first in the distalmost muscles ( the intrinsic foot and hand muscles). More severe cases may involve muscles of the lower legs and forearms, leading to bilateral foot drop or wrist drop. GENERAL PRINCIPLES 1. A dose-toxicity relationship exists in the majority of neurotoxic exposures. neurologic symptoms appear only after a cumulative exposure reaches a threshold level. GENERAL PRINCIPLES 2.Toxins typically cause a nonfocal; symmetrical neurologic syndrome. Significant asymmetry such as weakness or sensory loss of one limb or one side of the body, should suggest an alternative cause. GENERAL PRINCIPLES 3.There is usually a strong temporal relationship between exposure and the onset of symptoms Immediate symptoms after acute exposure are usually attributable to the physiologic effects of the chemical. These symptoms subside quickly with elimination of the chemical . GENERAL PRINCIPLES Delayed or persistent neurologic deficits That occur after toxic exposures (for example, organo-phosphate-related delayed neuropathy) are generally a result of pathologic changes in the nervous system. Recovery is still possible, but tends to be slow and incomplete. GENERAL PRINCIPLES 4. Nervous system generally has a limited capability to regenerate, some recovery is typically possible after removal of the insulting agent. As a corollary, continuing neurologic deterioration more than a few months after cessation of exposure to a toxin generally argues for a direct causative role of the toxin. GENERAL PRINCIPLES . Multiple neurologic syndromes are possible from a single toxin. Different neuron and different areas of the nervous system react differently to the neurotoxin. GENERAL PRINCIPLES The level and duration of exposure , physiologic variables such as the subject's age influence the clinical manifestations. A well-known example is lead toxicity, which may lead to an acute confusional state, chronic mental slowing, or a peripheral neuropathy. GENERAL PRINCIPLES 6. Few toxins present with a pathognomonic neurologic syndrome Symptoms and signs may be mimicked by many psychiatric, metabolic, inflammatory, neoplastic, and degenerative diseases of the nervous system. It is important to exclude other neurologic diseases with appropriate clinical examina tion and laboratory investigations. APPROACH TO PATIENTS A confident diagnosis of a neurotoxic disorder can only be made after documentation of all (1) a sufficiently intense or prolonged exposure to the toxin; (2) an appropriate neurologic syndrome based on knowledge about the putative toxin; (3) evolution of symptoms and signs over a compatible temporal course (4) exclusion of other neurologic disorders that may account for a similar syndrome. IMAGING The extent and severity of neurologic deficits are difficult to assess with precision. Present tools, as (CT) and (MRI), provide only visualization of macroscopic structural changes. While they are invaluable in detecting neoplastic, inflammatory and infectious disorders of the nervous system, they are far less helpful in documenting neurotoxic injuries. APPROACH TO PATIENTS A detailed history of nature, duration, and intensity of the exposure is essential in every evaluation. APPROACH TO PATIENTS What are the potential toxins? What is the mode of exposure? How long and how intense are the exposures? Are there other confounding factors such as alcoholism, psychosocial issues, and possibility of secondary gains. APPROACH TO PATIENTS Chronic exposures are especially difficult to assess. History should be followed by a of the neurologic complaint Documentation of the temporal course of the disease is very important. APPROACH TO PATIENTS Symptoms may appear acutely (minutes or days), subacutely (weeks or months), or chronically (years). Fluctuating symptoms may suggest recurrent exposures or unrelated superimposed factors. Recovery after discontinuation of exposure helps to implicate the exposure APPROACH TO PATIENTS Physical examination of patients with PNS should include testing of muscle strength, sensation, and tendon reflexes of all four extremities. The longest axons are the most vulnerable, neurologic deficits are frequently more severe in the feet than in the hands. DIAGNOSIS Prominent sensory impairment in the hands without signs of neuropathy in the feet is more likely to be caused by carpal tunnel syndrome than by a systemic polyneuropathy. Most polyneuropathies are accompanied by absent reflexes of the Achilles tendons and demonstrable sensory impairment in the toes. DIAGNOSIS . Approximately one-half to two-thirds of all polyneuropathies remain undiagnosed . Thus, the absence of an alternate etiology does not necessarily implicate a toxin. DIAGNOSIS Aside from the presence of sufficient exposure and compatible syndrome, the diagnosis quite frequently depends on the documentation of progressive sensory or motor deficits during exposure, and recovery of function months or years after cessation of exposure. DIAGNOSIS The most nonspecific syndrome is a distal, symmetrical, sensorimotor polyneuropathy. This is indistinguishable from the neuropathies caused by common systemic diseases such as alcoholism, uremia, diabetes mellitus, and vitamin B12 deficiency. Some toxins, such as lead, cause a neuropathy with prominent weakness. Symmetric generalized neuropathies The most common peripheral neuropathy that results from PNS insults, toxin-induced injuries, is a symmetric . These neuropathies probably reflect failure of axonal transport, with resultant degeneration distal nerve segments, predominantly affecting large-diameter axons. Symmetric generalized neuropathies Degeneration subsequently proceeds proximally toward the nerve cell body, both in the PNS and in central projections within the spinal cord distal axonopathy Most distal symmetric axonopathies have a subacuteonset with gradual progression. Symmetric generalized neuropathies The longest, largest diameter fibers are usually the most clinically affected, motor and sensory findings initially appear in the feet, later moving proximally (length-dependent relationship). With extreme progression, the vertex of the head is affected. Symmetric generalized neuropathies There is usually an early and symmetric loss of ankle reflexes; the more proximal reflexes may be spared until late in the disease. In most toxic neuropathies, sensory symptoms and signs initially predominate over motor deficits Symmetric generalized neuropathies . Muscle wasting may occur in chronic cases, including loss of hair over the distal leg,skin ulceration, and loss of sweating in the feet. Because recovery depends on axonal regrowth, complete recovery is prolonged and slow Symmetric generalized neuropathies Axonal regenerationoccurs at a rate of, on average, 2–3 mm/day. Function is restored in the reverse order to that lost; proximal muscles recover before distal muscles, and sensory loss recedes from proximal to distal levels. Toxic polyneuropathies . Mostly sensory or sensorimotor polyneuropathy (little or no weakness) Acrylamide Carbon disulfide Ethylene oxide Metals: arsenic, lead, mercury, thallium Methyl bromide Polychlorinated biphenyls (PCBs) Thallium Toxic polyneuropathies Predominantly motor or sensorimotor poly neuropathy with significant weakness Hexacarbons: n-hexane, methyl n-butyl ketone Metals: lead, arsenic, mercury Organophosphates Toxic polyneuropathies "Purely" sensory neuropathy (disabling sensory loss with no weakness) Cisplatinum Pyridoxine abuse Cranial neuropathy Thallium Trichloroethylene (trigeminal neuropathy) Prominent autonomic dysfunction Acrylamide n-Hexane (glue-sniffer) Thallium Vacor(PNU) Possible association with neuropathies (mostly anecdotal) Benzene Carbon monoxide Dioxin Methyl methacrylate Pyrethrins Carpal tunnel syndrome usual presentation acroparesthesias, numbness, tingling, and burning sensations in the lateral three fingers Nocturnal exacerbation of pain Shaking the hand frequently relieves pain palm is usually spared Raynaud’s phenomenon may occasionally be present Median nerve CTS most commonly involves the dominant hand bilateral involvement is extremely common causes include tuberculous tenosynovitis, rheumatoid arthritis, osteoarthritis, pregnancy, hemodialysis, myxedema, acromegaly patients with generalized peripheral neuropathies, such as uremia and diabetes Repetitive tasks CTS should be considered for any unexplained pain or sensory disturbance Diagnosis is obvious from the clinical signs and symptoms. Confirmatory electrodiagnostic studies should be obtained in all patients undergoing surgery. The most sensitive physiologic parameter is sensory and mixed nerve conduction Treatment Lead Acute high-level exposure typically comes from accidental ingestion, inhalation, or industrial exposure. neurologic symptoms such as headache, tremor apathy lethargy. Massive intoxication can lead to convulsions, cerebral edema, stupor, or coma, and eventually to transtentorial herniation. Lead Lead encephalopathy typically appears in adults at blood levels of 50-70 ftg/d Children are more vulnerable than adults probably because of the immaturity of the blood-brain barrier Chronic low-level exposure to lead is responsible for impaired intellectual development in children. Lead The classic description bilateral wristdrop and foot-drop. Toxicity also may manifest as a generalized proximal and distal weakness and loss of the tendon reflexes. Lead The best known clinical syndrome is a predominantly motor neuropathy with little if any sensory symptoms. It mimics in many ways motor neuron diseases such as amyotrophic lateral sclerosis Lead In patients with acute encephalopathy, the radiologic abnormalities reflect focal areas of edema, and are most commonly seen in bilateral thalami and basal ganglia. Imaging studies, and autopsy, may detect intracranial calcification in patients with chronic lead toxicity. Arsenic Peripheral neuropathy is the most common neurologic manifestation of toxicity, and may occur after either acute or chronic exposure After a single massive dose, an acute poly neuropathy develops within 1-3 . weeks Arsenic This neuropathy mimics Guillain-Barre syndrome in many ways, and respiratory failure may rarely occur. Occasionally, systemic symptoms may be accompanied by seizures and encephalopathy. Arsenic Diagnosis of arsenic neuropathy EMG and nerve conduction studies provide evidence of a nonspecific axonal neuropathy. Blood arsenic level returns to normal in about 12 hours, and urine arsenic clears within 48 to 72 hours after exposure.but detectable in hair and nail for months after exposure. Manganese The potential risk of organic manganese in the form of methylcyclopentadienyl manganese tricarbonyl (MMT), an additive used in gasolin. Compared to idiopathic Parkinson disease, the extrapyramidal symptoms of manganism are less responsive to dopaminergic therapy. Manganese Neurologic deficits often continue to progress for many years after cessation of exposure. Tremor, rigidity, masked facies, bradykinesias slowly develop. Heavy metal toxicity. Arsenic ;Insecticide, Paris green Sensory > motor neuropathy, red hands, burning feet, hyperhidrosis Lead Paint, gas, batteries Adults: neuropathy, painful joints; children: cerebral edema, encephalopathy, low IQ Mercury Industrial, polluted fish Severe arm and leg pain, dementia with primarily motor neuropathy Thallium Insecticide, rat poison Stocking-glove sensorimotorneuropathy, with alopecia THANKS YOUR ATTENTION “Coasting phenomen’’? The phenomenon of "coasting," the continuing deterioration sometimes seen for up to a few weeks after discontinuation of toxic exposure. The delay reflects the time necessary for the pathophysiologic steps to evolve to neuronal injury and death.