The locked-in syndrome (LIS), also known as motor deafferentation syndrome, is a rare
neurological disorder that was first described by Plum and Posner[1 ] in 1966, and which is characterized by quadriplegia and anarthria with preserved
consciousness. The syndrome was considered to have many etiologies ([Figure 1 ]).
Figure 1 Motor deafferentation syndrome etiologies[9 ], including the central pontine-myelinolysis and the Gayet-Wernicke encephalopathy
caused by thiamine deficiency reported in the Paraguayan War as a hypothesis, and,
in the backgroung, original photo at the church Tuyu Cué (Neembucu, Paraguai) serving
in the Brazilian Infirmary.Source: digital collection of Fundação Biblioteca Nacional.
We emphasize some aspects of a historical vignette that looks like a LIS description.
This is a very peculiar clinical picture described by an academic aristocrat of the
Brazilian Army, the author of Memorias[2 ]: Alfredo Maria Adriano d’Escragnolle Taunay, visconde de Taunay ([Figure 2 ]). Taunay was also a writer, musician, teacher, military engineer, politician, historian
and Brazilian sociologist. He reports the case of a Brazilian soldier who presented
an ascending paralysis, mental confusion, followed by an apparent lucidity associated
with quadriplegia ([Box 1 ]).
Figure 2 Alfredo d’Escragnolle Taunay, Visconde de Taunay (1843-1899).Source: Reproduction
of original picture by Louis Auguste Moreau (public domain).
Box 1
Case reported by Alfredo d’Escragnolle Taunay, published posthumously[2 ], and probably one of the first cases of Locked-in syndrome (LIS) in the literature
•“You cannot imagine what I am suffering. It is a pain of agony, nor is there any
other that is comparable to it. Death is rising! See how cold and immobile feet and
legs are”. And, in fact, as he spoke, their limbs were stiffened. “Now, it's the arms!”
And he stood with them straight, as if they were made of stone. […] If you shut up,
it was for a short time; the moans and cries started again [...]
•At last, he stopped, but when paralysis caught his tongue and lips. And he was stretched
out, stiff and immobile, on the death cot […] like a marble statue of those who sleep
in the tombs of the Middle Ages. Only his eyes swam in his orbits, still indicating
life and horrible anxieties, as tears flowed from them, which wet the pillow. The
unfortunate remained for a day and a half until he exhaled his last breath at one
o’clock on Jul 26, 1866”.
Before discussing this case, we must remember that the Paraguayan War ended 150 years
ago, and it occurred mostly in the extreme southwest of Brazil and in Paraguay. Consequently,
military logistics deficiency led the troops to severe food deprivation and related
scenarios, as the one here presented.
At this wartime, outbreaks of combatants’ limb weakness with particular features such
as tingling occurred. This was supposedly due to “palustrian causes”. However, there
was a shortage of supply, poor environmental conditions, and diarrheal diseases, besides
reports of native flora being used to feed troops and alcoholism. There were also
accounts on the death of horses with symptoms similar to that of combatants[2 ],[3 ],[4 ].
At that time, Science did not have all the critical clinical elements to establish
the diagnosis of thiamine deficiency due to inadequate intake, food with anti-thiamine
factors, or alcoholism, besides rare genetic cases[5 ]. Regardless of the underlying cause, thiamine deficits may have severe detrimental
effects, with most of the symptoms manifesting at the neurological level[5 ]. However, far from the war front, Silva Lima was studying beriberi[6 ]. He had already identified, then, similarities between cases he assisted and the
war cases[7 ].
We scrutinized the Brazilian troop neuropathic outbreaks considering several scenarios
in this study, but mainly this peculiar case. This would be one of the first reported
in the literature that sheds light on an underestimated part of the history of Neurology
in wartime.
This soldier, apparently with delirious and rapidly evolving tetraparesis, but with
the maintenance of eye movements, may have had a LIS. This syndrome is linked to several
etiologies; it can stem from basilar artery occlusion by stroke, Gayet-Wernicke encephalopathy
(GWE) with central pontine myelinolysis (CPM) due to thiamine deficiency, a form of
dry beriberi, and the Guillain-Barré syndrome (GBS).
About 82% of patients with GWE present with delirium, as reported by Osiezagha et
al.[8 ] based on a case series of autopsies. As for the LIS, it is expressed by sustained
eyelid opening, preserved necessary cognitive abilities, severe aphonia or hypophonia,
quadriplegia or quadriparesis, and a primary mode of communication that uses vertical
or lateral eye movement, or upper eyelid blinking[9 ].
Regarding the progressive ascending motor paralysis of the reported case, the GBS
should also be considered. This is an immune-mediated disease of peripheral nerves
and nerve roots that is often activated by infections, which is very common in wartime[10 ]. The progressive phase of GBS usually lasts from two days to four weeks. Consequently,
in patients who reach maximum disability within 24 hours after the onset of the disease,
as supposedly occurred in the reported case, alternative diagnoses should be contemplated.
Likewise, diagnoses related to altered consciousness may be considered - except Bickerstaff's
brain stem encephalitis, a variant of GS with the involvement of cranial nerves.
Besides, a peripheral disconnection syndrome, which can occur with GBS and severe
post-infection polyneuropathy, would include an external ophthalmoplegia, apparently
not present in the reported case[9 ].
In contrast, dry beriberi may mimic the most common form of GBS, and polyneuropathy
secondary to thiamine deficiency may develop gradually over weeks to months, but also
acutely, and consequently may be confused with GBS[10 ]. This polyneuritis can also be associated with GWE by thiamine deficiency, as presented
by Shible et al.[11 ].
Regarding the reported manifestations of irritability, restlessness, and complaint
of intense suffering, with a note about stiffness (muscle spasms?), generalized tetanus
should also be examined[12 ]. Once again, a comment on the report “[...] After all, the doctor reminded him to
give him calomel”. In fact, mercury compounds, like calomel, were used in medicinal
preparations in the past. However, this toxic cause is less likely for this discussed
case: “Toxic effects were soon noticed in individuals given large doses for long periods
[...] They had troubling neurologic symptoms, such as arm and facial tremors, hyporeflexia,
weakness, ataxia, and erethism [...]”[13 ].
In any case, considering the immobility emphasized by the writer, and the significant
probability of diagnosis related to the high prevalence of thiamine deficiency, the
most likely diagnosis for the reported case would be GWE with CPM, less likely GBS.
Coincidently, GBS was first described in the World War I by Georges Guillain, Jean
Alexandre Barré, and André Strohl when they witnessed (1916) two similar cases of
soldiers who had partial paralysis with significant impairment of reflexes with spontaneous
regression[14 ]. However, both GWE with CPM and GBS have some characteristics of motor deafferentation
syndrome or LIS that may encompass many etiologies ([Figure 2 ]).
This case report is similar to that of Alexandre Dumas in “The Count of Monte Cristo”
(1844), who was “a corpse with living eyes”, and one by Emile Zola, in his novel “Thérèse
Raquin” (1868)[9 ]. However, the first case of unmoving physical body, a deafferented patient, in medical
literature was described by Darolles (1875)[9 ]. Consequently, the case witnessed by Taunay in 1866 and only later on published
would be one of the first to recognize the LIS in its initial phase.
This detailed historical research can be useful, as one can learn from the past. The
description by Taunay leads us to believe we are addressing LIS or motor deafferentation
syndrome due to thiamine deficiency. It is more likely to happen in adverse conditions
such as war and in the early phase of LIS, since its diagnosis is usually only noticed
later by attentive caregivers.
