Acute intermittent porphyria

來源: Tianyazi 於 2014-12-14 06:27:40 [] [博客] [舊帖] [給我悄悄話] 閱讀數 : (1453 bytes)
回答: 可能病因 由 Tianyazi 於 2014-12-14 06:21:48

The sequence of events in attacks of acute intermittent porphyria (AIP) usually is as follows:

Abdominal pain
Psychiatric symptoms (eg, hysteria)
Peripheral neuropathies, mainly motor neuropathies
Most patients are completely free of symptoms between attacks. How the porphyrin precursors lead to these symptoms is unknown.

During attacks, patients with AIP have no skin manifestations but display neurovisceral signs and symptoms, including autonomic neuropathies that produce constipation, abdominal pain, vomiting, or hypertension and tachycardia. The abdominal pain often is epigastric and colicky in nature; it is severe and lasts for several days. Severe abdominal pain of short (
Central nervous system signs may include the following:

Seizures
Delirium
Cortical blindness
Coma
Patients often experience peripheral neuropathies that are predominantly motor and can mimic Guillain-Barré syndrome. The weakness usually starts in the lower limbs and ascends, but neuropathies can be observed in any nerve distribution.

Patients can have a wide variety of psychiatric symptoms. Depression is very common. Usually, patients have concurrent neurologic or abdominal symptoms.

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