A probable case of anti-NMDAR encephalitis from 1830
Josep Dalmau
Neurol Neuroimmunol Neuroinflamm Nov 2020, 7 (6) e901; DOI: 10.1212/NXI.0000000000000901
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,,,For those of you who enjoy a good detective story and
medical mystery, I will end this Editor’s Corner with the
contribution of T?enyi et al.6 While reviewing the historical
medical literature, these authors found reference to the same
case report in medical journals published in Hungarian, German,
and Italian. The case was presented at the 3rd meeting of
the Italian Scientists held in Florence in 1841. The patient
presented in 1830. She was a previously healthy 18-year-old
woman who developed seizures, followed by a 6-day-long
clinical state characterized by catalepsy, unresponsiveness, and
shallow breathing. These symptoms apparently resolved but
returned over the following year and a half during which a
progressively growing abdominal tumor was noted. One day,
she suddenly developed emesis that included pus and blood
mixed with more than 100 bone fragments. At the same time,
she had evacuation from the anus and vagina of a membranous
substance mixed with blood and pus. After this event, the
seizures and the catalepsy-like episodes disappeared. Over the
following years, she gave birth to 3 healthy children and was
still well 11 years later. The authors suggest that this represents
a case of anti-NMDA receptor (NMDAR) encephalitis. They
postulate that the catalepsy-like state was catatonia, which was
not defined as an entity for more than 40 years after this
patient’s presentation. They suggest that the mass was an
ovarian teratoma, which can reach large sizes, contain bone and
perforate into the bowel and vagina, whereas the shallow
breathing was likely because of hypoventilation. The authors
remind us that this description of a likely paraneoplastic syndrome
from 1830 predates by 35 years that of Trousseau
syndrome, which is considered the first paraneoplastic syndrome
reported. The case is fascinating and reminds us of
complications in patients with anti-NMDAR encephalitis
(continuous tumor growth despite partial neurologic improvement
and bowel perforation)7 that were included in the
first description of this disease and are now rarely encountered
due early recognition of this disorder...
Josep Dalmau
Neurol Neuroimmunol Neuroinflamm Nov 2020, 7 (6) e901; DOI: 10.1212/NXI.0000000000000901
- Copyright ? 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.
View Full Text
,,,For those of you who enjoy a good detective story and
medical mystery, I will end this Editor’s Corner with the
contribution of T?enyi et al.6 While reviewing the historical
medical literature, these authors found reference to the same
case report in medical journals published in Hungarian, German,
and Italian. The case was presented at the 3rd meeting of
the Italian Scientists held in Florence in 1841. The patient
presented in 1830. She was a previously healthy 18-year-old
woman who developed seizures, followed by a 6-day-long
clinical state characterized by catalepsy, unresponsiveness, and
shallow breathing. These symptoms apparently resolved but
returned over the following year and a half during which a
progressively growing abdominal tumor was noted. One day,
she suddenly developed emesis that included pus and blood
mixed with more than 100 bone fragments. At the same time,
she had evacuation from the anus and vagina of a membranous
substance mixed with blood and pus. After this event, the
seizures and the catalepsy-like episodes disappeared. Over the
following years, she gave birth to 3 healthy children and was
still well 11 years later. The authors suggest that this represents
a case of anti-NMDA receptor (NMDAR) encephalitis. They
postulate that the catalepsy-like state was catatonia, which was
not defined as an entity for more than 40 years after this
patient’s presentation. They suggest that the mass was an
ovarian teratoma, which can reach large sizes, contain bone and
perforate into the bowel and vagina, whereas the shallow
breathing was likely because of hypoventilation. The authors
remind us that this description of a likely paraneoplastic syndrome
from 1830 predates by 35 years that of Trousseau
syndrome, which is considered the first paraneoplastic syndrome
reported. The case is fascinating and reminds us of
complications in patients with anti-NMDAR encephalitis
(continuous tumor growth despite partial neurologic improvement
and bowel perforation)7 that were included in the
first description of this disease and are now rarely encountered
due early recognition of this disorder...
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