Acute intermittent porphyria presenting with neurological emergency: review of six cases.
Acute intermittent porphyria presenting with short duration of gastrointestinal symptoms followed by rapidly progressive fulminant neurological syndrome during first attack is relatively uncommon. It is a neurological emergency and mimics many other psychiatric and medical disorders and can be fatal if it remains undiagnosed and untreated. Further, specific treatment in the form of Heme arginate is not universally available and very costly, so high clinical suspicion and early diagnosis and management of acute attack and prevention of further attacks are very important. We report a series of six cases who presented with convulsion and/or polyneuropathy early in the course of disease to highlight this fact.
Kochar
Dhanpat K
DK
Department of Medicine, S.P. Medical College, Bikaner (Raj), India. drdkkochar@yahoo.com
Pal
Mahender
M
Kochar
Sanjay Kumar
SK
Vyas
Arvind
A
Kochar
Abhishek
A
Bindal
Dinesh
D
Agrawal
Rajender Prasad
RP
D000293:Adolescent; D000328:Adult; D000588:Amines / Q000627:therapeutic use; D000927:Anticonvulsants / Q000627:therapeutic use; D003509:Cyclohexanecarboxylic Acids / Q000627:therapeutic use; D004632:Emergency Medical Services; D005260:Female; D000077206:Gabapentin; D006801:Humans; D008140:Lorazepam / Q000627:therapeutic use; D008297:Male; D011115:Polyneuropathies / Q000175:diagnosis / Q000209:etiology*; D017118:Porphyria, Acute Intermittent / Q000150:complications* / Q000175:diagnosis; D011247:Pregnancy; D012640:Seizures / Q000175:diagnosis / Q000209:etiology*; D055815:Young Adult; D005680:gamma-Aminobutyric Acid / Q000627:therapeutic use
D000588:Amines; D000927:Anticonvulsants; D003509:Cyclohexanecarboxylic Acids; D005680:gamma-Aminobutyric Acid; D000077206:Gabapentin; D008140:Lorazepam