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sensory ones; less often both sensory and motor nerves are affected more or less equally and sometimes autonomic nerves as well The symptoms may begin in the feet and legs and ascend, or they may begin in the hands and arms (sometimes asymmetrically) and spread in a few days to the trunk and legs Often, the weakness predominates in the proximal muscles of the limbs and limb girdle muscles, in which case there is loss of knee jerks with preservation of re exes at the ankles Sensory loss, often extending on to the trunk, is present in half the cases Facial paralysis, dysphagia, and ocular palsies are features of only the most severe cases The CSF protein content is normal or slightly elevated The course of the polyneuropathy is variable In mild cases the symptoms regress in a few weeks Severe cases may progress to a fatal respiratory or cardiac paralysis in a few days, or the symptoms may advance in a saltatory fashion over several weeks, resulting in a severe sensorimotor paralysis that improves only after many months A disturbance of cerebral function (confusion, delirium, visual eld defects, and convulsions) is likely to precede the severe but not always the mild forms of polyneuropathy, or there may be none of these central features Cerebral manifestations subside in a few days or weeks, though one of our patients was left with a lasting homonymous hemianopia Tachycardia and hypertension are frequent in the acute phase of the disease, and fever and leukocytosis may also occur in severe cases In general, the prognosis for ultimate recovery is excellent, though relapse of the porphyria may result in cumulative involvement of the peripheral nervous system (see discussion of relapsing polyneuropathy further on) In summary, the most characteristic features are the relapsing nature, acute onset, abdominal pain, psychotic symptoms, predominant motor neuropathy, often, an early bibrachial distribution of weakness, truncal sensory loss, and tachycardia Rarely the neuropathy develops without other symptoms The pathologic ndings in the peripheral nervous system vary according to the stage of the illness at which death occurs In the rst few days, the myelinated bers may appear entirely normal, despite an almost complete paralysis If symptoms had been present for weeks, degeneration of both axons and myelin sheaths are found in most of the peripheral nerves The relation between the abnormality of porphyrin biosynthesis in the liver and nervous dysfunction has never been explained satisfactorily The diagnosis is con rmed by the demonstration of large amounts of porphobilinogen and -aminolevulinic acid in the urine The urine turns dark when standing due to the formation of porphobilin, an oxidation product of porphobilinogen Treatment The use of intravenous glucose and intravenous hematin (4 mg/kg daily for 3 to 14 days) is recommended as the most direct and effective therapy (Windebank and Bonkovsky) Other aspects of treatment include respiratory support, use of beta-blocking agents (labetalol) if tachycardia and hypertension are severe, intravenous glucose to suppress the heme biosynthetic pathway, and pyridoxine (100 mg twice a day) on the supposition that vitamin B6 depletion has occurred Prevention is of the utmost importance, since attacks can be precipitated by the aforementioned drugs, as well as numerous others that are porphyrinogenic. create barcode in excel using vba Using the Barcode Font in Microsoft Excel (Spreadsheet)
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