Although younger neurologists may not have
encountered postencephalitic parkinsonism,
encephalitis lethargica merits mention in
contemporary reviews of Parkinson 's disease.
The name of von
Economo is inseparably and justifiably
attached to the disease since his was the major
description of an illness that raged in epidemic
form in Europe and North America between 1916
and 1926. lvy McKenzie provided a scholarly
account of the illness in Glasgow. Longer texts
were written by Jelliffe, and by Wimmer . At
first, others confused encephalitis lethargica
with the pandemic of influenza (Spanish grippe).
Cruchet had first noted encephalitis lethargica
in the winter of 1915-16 in French soldiers in
Verdun, and a few cases were seen in the spring
of 1915 in Rumania. von Economo published 27
papers; including a book, on encephalitis
lethargica.
Excerpts from a translation ofhis classic
description
"We are dealing with a kind of sleeping
sickness, having an unusually prolonged course.
The first symptoms are usually acute, with
headaches and malaise. Then a state of
somnolence appears, often associated with active
delirium from which the patient can be awakened
easily. He is able to give appropriate answers,
and to comprehend the situation. This delirious
somnolence can lead to death, rapidly, or over
the course of a few weeks. On the other hand it
can persist unchanged for weeks or even months
with periods lasting bouts or days or even
longer, of fluctuation of the depth of
unconsciousness extending from simple sleepiness
to deepest stupor or coma ...During the first
days of the illness, isolated signs of meningeal
irritation appear ...The appearance of lever and
its intensity do not seem to have any effect
upon the course and signs of the disease...
As a rule these general symptoms are joined
by paralysis of the cranial nerves as well as in
the extremities ... a paralytic ptosis often
combined with partial or total paralysis of
other branches of the oculomotor nerve . . ..
Paresis of the other cranial nerves and
paralysis of the extremities with reflex
disturbances can occur also"
[seven case reports and an account of the
clinical features are then given]
"The spinal fluid ... showed increased
pressure at the beginning; it later decreased in
spite of persistent somnolence. The total
protein was below the normal upper limit; ... In
case 1, 43 cells/mm3, in case 2, 19 cells/mm3
... strong predominance of polymorphonuclear
leukocytes. Repeated examinations for
microorganisms was negative in every case. The
Wassermann reaction in serum and spinal fluid
was always negative...
As the residual of such a paresis (upper and
lower extremities), spasms, increased
reflexes, or the Babinski phenomenon can persist
for a long time, even after the ocular muscle
paralysis and somnolence have receded ... one
often finds a peculiar rigidity of the
extremities (cases 1, 2 and 4). Like most of the
signs this rigidity disappears later ...
We frequency find (cases 1, 2, 3, 4, 7)
ataxic disturbances that make one think of
involvement of the cerebellum. Case 7
demonstrated severe ataxic tremor. . .. With
reference to pontine encephalitis I describe
briefly several cases seen in the emergency room
during the same period"
[six further case reports and a long
discussion on différential diagnosis and
pathology follow] ...
von Economo concludes: "Therefore we have the
histological picture of a polioencephalitis
cerebri pontis et medullae oblongatae, with a
stight poliomyelitis of a perivascular,
inflammatory and diffusely infiltrative but not
haernorrhagic and only sligtly neuronophagic
character."
Born in Braila, Rumania, of an aristocratic
Greek family he studied engineering belote
reading medicine in Vienna. Early training was
with Nothnagel, Pierre Marie, and Kraepelin
belote he returned to the psychiatric clinic in
Vienna.
His work centred on neuroanatomy, head
injuries in the first world war, and
investigations on sleep, cortical architecture,
and on Wilson's disease.
He married the daughter of Prince Alois von
Schönburg-Hartenstein, and being of
independent means, rejected the chair of
psychiatry on von Jauregg's retirement in 1928.
He died suddenly of heart disease in 1931.
JM S PEARCE 304 Beverley Road, Anlaby, Hull
HUIO 7BG, UK
