ANNALS
of Neurology
TABLE. Association of rs3796529 with Hippocampal CA1 Volume for Each Diagnostic Group
CN Hippocampal CA1 Volume
MCI
AD
b
p
b
p
b
p
Adjusting for APOE e4 status
24.948
0.1889
6.304
0.0247
10.63
0.0366
Without adjusting for APOE e4 status
24.992
0.1843
5.834
0.0380
10.42
0.0406
AD 5 Alzheimer disease; CN 5 cognitively normal; MCI 5 mild cognitive impairment.
Congenital Insensitivity to Pain with Anhidrosis Thomas D. Bird, MD1,2 and Phillip D. Swanson, MD, PhD2 The article by Norcliff-Kaufmann et al in the May issue of the Annals of Neurology regarding congenital insensitivity to pain with anhidrosis brought back vivid memories for us.1 We remember very well the two brothers with this disease when we saw them as patients at the University of Washington Hospital shortly after they had been reported by Dr August Swanson in 1963 (reference 2 in Norcliff-Kaufmann et al).2 Their lack of pain was truly remarkable. One of the brothers died during a febrile illness with body temperature to 1108F. This is the case whose neuropathological study showed complete absence of Lissauer’s tract in the spinal cord. This rare finding was described in 1965 by Swanson, Buchan, and Alvord and inadvertently misidentified by Norcliff-Kaufmann et al in their reference 44.3 The living brother subsequently had extensive studies of his insensitivity to pain by Chatrian et al in 1975.4
4.
Chatrian GE, Farrell DF, Canfield RC, Lettich E. Congenital Insensitivity to noxious stimuli. Arch Neurol 1975;32:141–145.
DOI: 10.1002/ana.24450
Reply Horacio Kaufmann, MD and Lucy Norcliffe-Kaufmann, PhD We thank Drs Bird and Swanson for their comments on our article.1 Their report of the cause of death in 1 of the brothers with hereditary sensory and autonomic neuropathy type IV2 is a reminder of the extreme hyperthermia that can occur in these patients due to their lack of sweating. We also thank the authors for pointing out our error in reference 44. We had meant to cite the article by Swanson et al, which described the neuropathology findings in the sibling who died of a febrile illness.3
Potential Conflicts of Interest Nothing to report.
Potential Conflicts of Interest Nothing to report.
Department of Neurology, New York University School of Medicine, New York, NY
1
References
2
University of Washington, Seattle, WA GRECC, VA Puget Sound Health Care, Seattle, WA
1.
Norcliffe-Kaufmann L, Katz SD, Axelrod F, Kaufmann H. Norepinephrine deficiency with normal blood pressure control in congenital insensitivity to pain with anhidrosis. Ann Neurol 2015;77:743– 752.
2.
Swanson AG. Congenital insensitivity to pain with anhydrosis. A unique syndrome in two male siblings. Arch Neurol 1963;8:299– 306.
3.
Swanson AG, Buchan GC, Alvord EC Jr. Anatomic changes in congenital insensitivity to pain. Absence of small primary sensory neurons in ganglia, roots, and Lissauer’s tract. Arch Neurol 1965;12: 12–18.
References 1.
Norcliff-Kaufmann L, Katz SD, Axelrod F, Kaufmann H. Norepinephrine deficiency with normal blood pressure control in congenital insensitivity to pain with anhidrosis. Ann Neurol 2015;77:743–752.
2.
Swanson AG. Congenital insensitivity to pain with anhydrosis. A unique syndrome in two male siblings. Arch Neurol 1963;8:299– 306.
3.
Swanson AG, Buchan GC, Alvord EC. Anatomic changes in congenital insensitivity to pain. Absence of small primary sensory neurons in ganglia, roots, and Lissauer’s tract. Arch Neurol 1965;12:12–18.
500
DOI: 10.1002/ana.24451
Volume 78, No. 3
of Neurology
TABLE. Association of rs3796529 with Hippocampal CA1 Volume for Each Diagnostic Group
CN Hippocampal CA1 Volume
MCI
AD
b
p
b
p
b
p
Adjusting for APOE e4 status
24.948
0.1889
6.304
0.0247
10.63
0.0366
Without adjusting for APOE e4 status
24.992
0.1843
5.834
0.0380
10.42
0.0406
AD 5 Alzheimer disease; CN 5 cognitively normal; MCI 5 mild cognitive impairment.
Congenital Insensitivity to Pain with Anhidrosis Thomas D. Bird, MD1,2 and Phillip D. Swanson, MD, PhD2 The article by Norcliff-Kaufmann et al in the May issue of the Annals of Neurology regarding congenital insensitivity to pain with anhidrosis brought back vivid memories for us.1 We remember very well the two brothers with this disease when we saw them as patients at the University of Washington Hospital shortly after they had been reported by Dr August Swanson in 1963 (reference 2 in Norcliff-Kaufmann et al).2 Their lack of pain was truly remarkable. One of the brothers died during a febrile illness with body temperature to 1108F. This is the case whose neuropathological study showed complete absence of Lissauer’s tract in the spinal cord. This rare finding was described in 1965 by Swanson, Buchan, and Alvord and inadvertently misidentified by Norcliff-Kaufmann et al in their reference 44.3 The living brother subsequently had extensive studies of his insensitivity to pain by Chatrian et al in 1975.4
4.
Chatrian GE, Farrell DF, Canfield RC, Lettich E. Congenital Insensitivity to noxious stimuli. Arch Neurol 1975;32:141–145.
DOI: 10.1002/ana.24450
Reply Horacio Kaufmann, MD and Lucy Norcliffe-Kaufmann, PhD We thank Drs Bird and Swanson for their comments on our article.1 Their report of the cause of death in 1 of the brothers with hereditary sensory and autonomic neuropathy type IV2 is a reminder of the extreme hyperthermia that can occur in these patients due to their lack of sweating. We also thank the authors for pointing out our error in reference 44. We had meant to cite the article by Swanson et al, which described the neuropathology findings in the sibling who died of a febrile illness.3
Potential Conflicts of Interest Nothing to report.
Potential Conflicts of Interest Nothing to report.
Department of Neurology, New York University School of Medicine, New York, NY
1
References
2
University of Washington, Seattle, WA GRECC, VA Puget Sound Health Care, Seattle, WA
1.
Norcliffe-Kaufmann L, Katz SD, Axelrod F, Kaufmann H. Norepinephrine deficiency with normal blood pressure control in congenital insensitivity to pain with anhidrosis. Ann Neurol 2015;77:743– 752.
2.
Swanson AG. Congenital insensitivity to pain with anhydrosis. A unique syndrome in two male siblings. Arch Neurol 1963;8:299– 306.
3.
Swanson AG, Buchan GC, Alvord EC Jr. Anatomic changes in congenital insensitivity to pain. Absence of small primary sensory neurons in ganglia, roots, and Lissauer’s tract. Arch Neurol 1965;12: 12–18.
References 1.
Norcliff-Kaufmann L, Katz SD, Axelrod F, Kaufmann H. Norepinephrine deficiency with normal blood pressure control in congenital insensitivity to pain with anhidrosis. Ann Neurol 2015;77:743–752.
2.
Swanson AG. Congenital insensitivity to pain with anhydrosis. A unique syndrome in two male siblings. Arch Neurol 1963;8:299– 306.
3.
Swanson AG, Buchan GC, Alvord EC. Anatomic changes in congenital insensitivity to pain. Absence of small primary sensory neurons in ganglia, roots, and Lissauer’s tract. Arch Neurol 1965;12:12–18.
500
DOI: 10.1002/ana.24451
Volume 78, No. 3
