Neurological complications of porphyria.
نویسندگان
چکیده
Accepted 3 October 1995 A 20-year-old girl presented with a three-year history of recurrent colicky abdominal pain associated with the passage of reddish urine. During the past three months she had also noticed progressive weakness and wasting of her right hand and forearm. There were no sensory symptoms. There was no history of photosensitivity or any skin changes. On examination she had wasting of the small muscles of the hand, predominantly on the right side (figure 1). Deep tendon reflexes were diminished in the upper limbs but normal in the lower limbs. Plantar reflexes were bilaterally flexor and there was no sensory, cranial nerve or cerebellar involvement. All routine investigations including biochemical tests of thyroid function were normal. Ehrlich's test for porphobilinogen in urine was repeatedly positive. A clinical diagnosis of acute intermittent porphyria was confirmed by further laboratory tests. Electrophysiological studies revealed bilateral median nerve axonal neuropathy. She was appropriately treated and discharged with advice to take certain precautions. Seven months later she was re-admitted with additional features of increase in the weakness and wasting of the left hand, brisk deep tendon reflexes in the lower limbs and bilateral consistently extensor plantar reflexes. X-Rays of the cervical spine and CV junction as well as magnetic resonance imaging (MRI) of the cervical spine (figure 2) were normal. Her third admission a month later was with the presenting features of hoarseness, nasal regurgitation and dysphagia along with abdominal pain and reddish urine. Her blood pressure was 150/100 mmHg with a drop to 130/80 mmHg on standing. On neurological examination she had a bilateral 9th and 10th cranial nerve palsy which resolved completely over the next 10 days.
منابع مشابه
[Acute intermittent porphyria: a diagnostic dilemma].
Acute intermittent porphyria (AIP) is a rare condition characterized by abdominal pain and a wide range of nonspecific symptoms. We report the case of a 24-year-old woman with syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and motor and sensory neurological deficits due to an attack of acute porphyria. The patient presented to the emergency department with abdominal pain. T...
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ورودعنوان ژورنال:
- Postgraduate medical journal
دوره 72 852 شماره
صفحات -
تاریخ انتشار 1996