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J Neurol Neurosurg Psychiatry 74:654-657 doi:10.1136/jnnp.74.5.654
  • Short report

Allgrove or 4 “A” syndrome: an autosomal recessive syndrome causing multisystem neurological disease

Table 1B

Cardiovascular autonomic and adrenal function tests

Case 1 Case 2 Case 3
BP, blood pressure; HR, heart rate; ACTH, adrenocortical trophic hormone. *Denotes an abnormal response.
Adrenal function
    Basal cortisol + synacthen 250 IU 513 nmol/l 215 nmol/l 302 nmol/l
    30 min 926 nmol/l 456 nmol/l 554 nmol/l
    60 min 1260 nmol/l 980 nmol/l 890 nmol/l
    Basal ACTH 26.7ng/l
    Recumbent aldosterone 77.0ng/l 10.9ng/l*
    Recumbent renin 20mU/l 37.0mU/l
Postural challenge
    Lying/standing BP 108/70–89/56* 145/95–104/67* 148/85–162/89
    30:15 ratio 1.0* 1.0*
HR response to respiratory stimulii
    Deep breathing 4* 2* 4*
Pressor tests
    Cutaneous cold
        BP 111/53–112/69 122/85–120/78* 138/84–165/88
        HR 75–81 78–88 70–78
    Isometric exercise
        BP 130/85–122/75* 137/85–136/88*
        HR 77–80 70–68
    Mental arithmetic
        BP 115/80–126/71 127/89–120/74* 137/83–147/93
        HR 76–80 77–84 72–70

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