Machado C. Jahi McMath: A new state of disorder of consciousness. J Neurosurg Sci 2021;65:211-213.DOI:10.23736/S0390-5616.20.04939-5

Abstract

In this paper, I reviewed the case of Jahi McMath who was diagnosed as being in brain death (BD). Nonetheless, ancillary tests, performed 9 months after initial brain insult, showed conservation of intracranial structures, EEG activity, and autonomic reactivity to “Mother Talks” stimulus. She was clinically in a state of unarousable and unresponsiveness, without evidence of awareness of self or environment, but full absence of brainstem reflexes, and partial responsiveness rejected the possibility of being in coma. Jahi was not a UWS, because she was not in a wakefulness state, and showed partial responsiveness. LIS patients are wakeful and aware, and although these cases are quadriplegic, they fully or partially preserve brainstem reflexes, vertical eye movements, and/or blinking, and respire by their own, rejecting the possibility of classifying her as a LIS patient. She was not a MCS because she did not preserve arousal, and only partially preserved awareness. The CRS-R resulted in a very low score, not corresponding with MCS patients. MCS patients fully or partially preserve brainstem reflexes, and usually breathe by their own. MCS has been always described as a transitional state between coma, UWS, but MCS has never been reported in a patient who has all clinical BD findings. This case doesn’t contradict the concept of BD, but brings again to discussion the needs of using ancillary tests in BD. I concluded that Jahi represented a new state of disorder of consciousness, non-previously described, that I have termed: “responsive unawake syndrome” (RUS).

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Vol. 65 - No. 2 JOURNAL OF NEUROSURGICAL SCIENCES 211
LETTERS TO THE EDITORJournal of Neurosurgical Sciences
April 2021
Vol. 65 - No. 2
© 2020 EDIZIONI MINERVA MEDICA
Online version at http://www.minervamedica.it
Journal of Neurosurgical Sciences 2021 April;65(2):211-3
DOI: 10.23736/S0390-5616.20.04939-5
Jahi McMath: a new state of disorder
of consciousness
Brain death (BD) has been progressively accepted in the last de-
cades, but contentious braindead cases have raised up new dis-
putes on the determination BD, such as the Jahi McMath case.1-3
Jahi McMath suffered a massive bleeding as a surgical com-
plication leading to a cardiorespiratory arrest. She was declared
brain-dead on December 12, 2013. Her parents did not accept
this diagnosis, and after an unusual lawful treaty she was moved
to New Jersey State (USA). The patient nally died on June 22,
2018.2, 3
The author, Dr. Calixto Machado, a Cuban neurologist was
requested as an expert consultant in September 2014. She was
studied at the Rutgers Hospital where an US licensed neurologist,
assessed her clinically and prescribed a group of ancillary tests
suggested by Dr. Machado.
This manuscript was approved by the Havana Institute of Neu-
rology and Neurosurgery Ethics Committee, according to the Hel-
sinki Declaration. The author received informed consent from Jahi
McMath’s mother to publish this material.
Jahi McMath’s ndings (Figure 1, 2, 3) after 9 months of her
initial diagnosis were:1, 3 1) clinical examination revealed absent
brainstem reexes, not even triggering the ventilator; apnea test
was not performed at this time, because Jahi McMath’s relatives
did not give consent; 2) MRI demonstrated conservation of intra-
cranial structures, despite the existence of vast abnormalities; a
huge lesion was found in pons; MRI demonstrated preservation of
tracts connecting the brainstem with thalamus and cerebral cortex,
and tracts were also present in the cerebral hemispheres linking
several cortical areas; 3) EEG activity over 2 μV of amplitude;
the power spectra density showed that EEG activity was mainly
in the delta-theta range; heart rate variability (HRV) bands were
well-preserved; 4) autonomic reactivity, measured by HRV, to the
“mother talks” stimulus demonstrated autonomic function reac-
tivity.
Although the patient fullled clinical BD criteria, the preser-
vation of intracranial structures (including the presence or tracts
connecting the brainstem with upper regions, and linking several
cortical areas), EEG over 2 μV, conservation of HRV components,
and autonomic reactivity response to the “mother talks” stimulus,
demonstrated remaining brain function in both brainstem and ce-
rebral hemispheres, rejecting that she was braindead.1
A complete arrest of cerebral blood ow (CBF) is a condition
sine qua non for the concept of BD. Neurons are permanently in-
jured after a few minutes of complete CBF ending. Hence, con-
servation of intracranial structures is a strong evidence of residual
CBF.1, 3, 4
The use of ancillary tests in BD determination is related to the
denition of death on neurological grounds: whole brain, brain-
stem death and higher brain standards.4
When intracranial pathology is localized to the posterior fossa,
both CBF and EEG may persist, because these lesions do not pro-
duce noticeably raised intracranial pressure. Therefore, several
LETTERS TO THE EDITOR
Figure 1.—A) MRI: T1 sagittal left view is presented. MRI shows preservation of intracranial structures, in spite of the presence of vast abnormali-
ties: remarkable preservation of cortical and brainstem gross anatomy, with non-expected relative slight atrophy, in spite of cortical laminar necrosis,
demyelination, and cystic encephalomalacia in the centrum semiovale, corpus callosum, and posterior pons and medulla. MRI in also shows a huge
lesion in the posterior regions of the pons, lateralized to the left side (arrow). B) MRI tractography placed over an MRI frontal view, shows tracts
connecting the brainstem with thalamic and cortical regions. Fewer tracts are found for left areas of the brainstem. C) MRI tractography placed over
an MRI sagittal view shows tracts connecting several cortical areas. Fewer tracts are found for left brain hemisphere.
A B C
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LETTERS TO THE EDITOR
212 JOURNAL OF NEUROSURGICAL SCIENCES April 2021
the contrary, all HRV bands, BD were preserved in this patient,
demonstrating preservation of autonomic function. Moreover,
autonomic reactivity to “mother talks” stimulation demonstrated
remaining function at different levels of the central autonomic
countries from continental Europe, Central and South America,
and Asia, require the demonstration of an EEG showing electro-
cerebral silence for the certication of BD.3
BD has been characterized by the loss of all HRV power. On
Figure 2.—A) Representation of 65 seconds of an EEG recorded from 19 standard channel leads, contaminated with ECG. A disorganized EEG
background, with a prevalence of diffuse Delta-theta activity is found. Although records were characterized by a low-voltage output, EEG amplitude
was clearly over 2 μV, excluding a pattern of electro-cerebral inactivity. B) Power spectra density obtained for 65 seconds of free of artefacts con-
tinuous EEG. EEG activity is mainly in the delta-theta range. In the Fp2 and Fz leads there is an incipient, but observable, peak in the alpha band. In
the O1 lead, a visible delta peak is also present. Several power spectra density inter-hemispheric asymmetries are found for homologous EEG leads.
Figure 3.—A) The power spectral density for the whole HRV spectrum is present. Discrete spectral frequencies within the very low frequency
(VLF), low frequency (LF), middle frequency (MF), high frequency (HF) and very high frequency bands (VHF) are clearly present, despite low
power spectral density. B) The instantaneous spectral amplitudes of the respective intrinsic mode functions (IMFs) are presented. Tacogram is shown
in the rst diagram. A thick vertical line indicates the beginning of the “mother talks” stimulus, compared with the “basal record.” It is possible to
observe ostensible dynamics in the different HRV frequencies, indicating a manifest autonomic reactivity to the mother’s voice stimulus.
AB
Fp1 Fp2
F7 F3 Fz F4 F8
T3
T5
C3 Cz C4 T4
P3 Pz P4 T6
O1 O2
A
160
140
120
100
80
60
40
20
0
Power spectral density (ms2)w
0 0.1 0.2 0.3 0.4 0.5 0.6
700
680
Input-s
2
1
0
4
2
0
10
5
0
5
0
4
2
0
IMF-1IMF-2IMF-3IMF-4IMF-5
B
Input signal and amplitude Hilbert Spectra of IMFs
50 100 150 200 250
50 100 150 200 250
50 100 150 200 250
0
0
0
50 100 150 200 250
0
50 100 150 200 250
0
50 100 150 200 250
0
VLFB
LFB
MFB
HFB
VHFB
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This document is protected by international copyright laws. No additional reproduction is authorized. It is permitted for personal use to download and save only one le and print only one copy of this Article. It is not permitted to make additional copies (either sporadically
or systematically, either printed or electronic) of the Article for any purpose. It is not permitted to distribute the electronic copy of the article through online internet and/or intranet le sharing systems, electronic mailing or any other means which may allow access
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LETTERS TO THE EDITOR
Vol. 65 - No. 2 JOURNAL OF NEUROSURGICAL SCIENCES 213
the spectrum of consciousness Journal Functional Neurology. Rehabilita-
tion and Ergonomics and Rehabilitation 2017;4:542–56.
4. Bernat JL, Dalle Ave AL. Aligning the Criterion and Tests for Brain
Death. Camb Q Healthc Ethics 2019;28:635–41.
5. Shewmon DA. Truly Reconciling the Case of Jahi McMath. Neurocrit
Care 2018;29:165–70.
Conicts of interest.—The author certies that there is no conict of inter-
est with any nancial organization regarding the material discussed in the
manuscript.
Authors’ contributions.—The author read and approved the nal version of
the manuscript.
History.—Article rst published online: May 13, 2020. - Manuscript ac-
cepted: May 5, 2020. - Manuscript revised: April 28, 2020. - Manuscript
received: March 8, 2020.
(Cite this article as: Machado C. Jahi McMath: a new state of disorder of
consciousness. J Neurosurg Sci 2021;65:211-3. DOI: 10.23736/S0390-
5616.20.04939-5)
© 2020 EDIZIONI MINERVA MEDICA
Online version at http://www.minervamedica.it
Journal of Neurosurgical Sciences 2021 April;65(2):213-5
DOI: 10.23736/S0390-5616.20.04950-4
Von Hippel-Lindau disease
and multispecialist team
We congratulate the Authors of the review article “Von Hippel-
Lindau disease: when neurosurgery meets nephrology, ophthal-
mology and genetics” for focusing their attention on this rare
syndrome.1 Actually, a wider knowledge about VHL disease is
required in order to identify affected patients and refer them to a
multispecialist team. This is important not only to offer them the
best treatment, but, also, to avoid unnecessary and harmful inter-
ventions. However, we believe that some remarks may be added
to the abovementioned study, based on our long-lasting experi-
ence in dealing with VHL patients.
The VHL-Padova Network, founded in 1996, is currently the
most experienced Italian national reference group for patients
with VHL disease. Since its foundation, this group, which in-
cludes specialists in endocrinology, neurosurgery, urology, oph-
thalmology, otosurgery, general surgery, and molecular genetics,
has taken care of 288 VHL patients (170 on active follow-up, 41
deceased, 77 lost to follow-up).
In 2016 we published a prospective analysis of 128 VHL pa-
tients who were followed up for about 12 years, with an average
period of 45 months.2 In our attempt to dene the natural history
and evolution of the whole VHL disease, we also assessed any
genotype-phenotype correlation and surgical timing for each or-
gan involved, as they both might affect the course of the disease.
Moreover, we focused for the rst time on the participants’ dis-
system.3 These results support Dr. Shewmon’s analysis of Jahi
McMath’s videos, who emphasized that her movements reected
responses to her mother.1, 3, 5
Jahi McMath was not comatose because although she was
clinically in a sleep-like state of unarousable and unresponsive-
ness, without evidence of awareness of self or environment, a
full absence of brainstem reexes, rejected the possibility to be in
coma. In this case the possibility of drug intoxication was exclud-
ed, which could explain the nonappearance of brainstem reexes.
Some comatose patients show favorable outcome, but persist un-
aware of self or environment, rstly named vegetative state (VS),
and more recently, “unresponsive wakefulness syndrome” (UWS).
Therefore, Jahi McMth was not a UWS, because she was not in a
wakefulness state, and showed partial responsiveness. Moreover,
UWS patients fully or partially retain brainstem reexes, and usu-
ally breathe by their own. It is not possible to classify Jahi McMath
as a locked-in syndrome (LIS), because she was not in a wakeful-
ness state, and showed partial responsiveness. Furthermore, LIS
patients, although quadriplegic, usually preserve fully or partially
brainstem reexes, vertical eye movements, and/or blinking, and
do not require mechanical ventilation. The “minimally conscious
state” (MCS) is a higher state in the continuum of consciousness,
showing inconsistent but reproducible evidence of awareness of
self or environment. (MCS). Jahi McMath was not an MCS, be-
cause she was in a sleep-like condition without preservation of
arousal. Another critical difference is that MCS has been always
dened as an intermediate state between coma, UWS, and higher
level of consciousness, but a MCS has never been described in a
patient fullling all clinical BD criteria.3
The relative intactness of the upper brainstem, thalamus and
cortex as well as the partial sparing of the mesopontine tegmen-
tal reticular formation, might explain the intermittent conscious
responses in this patient. Her connections to the thalamo-cortical
and/or its ventral pathway to the cortico-cortical projection sys-
tems, and parts of the associative cerebral cortices, were surely
preserved.1, 3
Jahi McMath was a rare and argumentative case. The concept
of BD is not denied with the discussion of this case but brings back
the debate of using or not ancillary tests in BD conrmation.1
In conclusion, Jahi was in a new state of disorder of conscious-
ness, non-previously described, that I have termed as a “respon-
sive unawake syndrome” (RUS).
Calixto MACHADO *
Department of Clinical Neurophysiology, Institute of Neurology
and Neurosurgery, Havana, Cuba
*Corresponding author: Calixto Machado, Department of Clinical Neuro-
physiology, Institute of Neurology and Neurosurgery, Havana, Cuba.
E-mail: braind@infomed.sld.cu
References
1. Machado C, Estévez M, DeFina PA, Leisman G. Response to Lewis
A: Reconciling the Case of Jahi Mcmath. Neurocrit Care 2018;29:521–2.
2. Truog RD. Lessons from the Case of Jahi McMath. Hastings Cent Rep
2018;48:S70–3.
3. Machado CD, Estevez M, Leisman G, Rodriguez R, Presitigiacomo C,
Fellus J, et al. A Reason for care in the clinical evaluation of function on
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©
2021 EDIZIONI MINERVA MEDICA
This document is protected by international copyright laws. No additional reproduction is authorized. It is permitted for personal use to download and save only one le and print only one copy of this Article. It is not permitted to make additional copies (either sporadically
or systematically, either printed or electronic) of the Article for any purpose. It is not permitted to distribute the electronic copy of the article through online internet and/or intranet le sharing systems, electronic mailing or any other means which may allow access
to the Article. The use of all or any part of the Article for any Commercial Use is not permitted. The creation of derivative works from the Article is not permitted. The production of reprints for personal or commercial use is not permitted. It is not permitted to remove,
cover, overlay, obscure, block, or change any copyright notices or terms of use which the Publisher may post on the Article. It is not permitted to frame or use framing techniques to enclose any trademark, logo, or other proprietary information of the Publisher.