Anthrax meningoencephalitis: Appendices
Douglas J. Lanska, M.D., M.S., M.S.P.H.5-11-02
Veterans Affairs Medical Center
Great Lakes VA Healthcare System
Tomah, WI;
Department of Neurology
University of Wisconsin
Madison, WI
Correspondence:
Douglas J. Lanska, M.D.
Chief of Staff
VA Medical Center
500 E. Veterans Street
Tomah, WI 54660
608-372-1778
608-372-1654 (FAX)
Page 1
Anthrax Meningoencephalitis:
Appendix 1
Translation of
Contribution to the Knowledge of Anthrax Meningitis
By Ernst von Czyhlarz
Translation by Bernd Remler, M.D.
Medical College of Wisconsin, Milwaukee.
CONTRIBUTION TO THE KNOWLEDGE OF ANTHRAX MENINGITIS
By Ernst von Czyhlarz
[Translation by Bernd Remler, M.D., Medical College of Wisconsin, Milwaukee. From: Czyhlarz E. Beitrag zur Lehre von der der Milzbrandmeningitis. Wiener Klinische Wochenscrift 1916;25:768-769.]
The first author who reported a likely case of anthrax meningitis was E. Wagner (1874). He described exclusively patients who had come to autopsy. In the following years, additional cases were reported predominantly by pathologists, in particular forensic pathologists (Eppinger, von Hofmann, Kundrat, R. Paltauf, Risel, Kolisko, Schmorl). In all likelihood, this reflects the circumstance that anthrax meningitis causes unexpected and rapid death due to intrameningeal bleeding in a characteristic pattern.
The limited knowledge and interest afforded anthrax infections of the central nervous system is further reflected in A. Nicolaier’s quote in his standard textbook “The German Clinic” from 1903: “The brain shows mostly smaller hemorrhages, which are also not uncommon in the meninges. In addition, small softening spots (translator: necrotic areas?) are seen.”
There is almost no published information on the clinical course of cerebral anthrax, in particular anthrax meningitis. Its prognosis is generally considered poor, even in the best circumstances. Noteworthy is a contribution by Pollak, who in one case succeeded to demonstrate anthrax bacilli in the cerebrospinal fluid in vivo. Thus, the diagnosis was established before the patient died and came to autopsy.
As already mentioned, most contributions on this topic were made by forensic pathologists. Kolisko stated in his chapter in the Handbook of Expert Medical Evaluation (ed: Dittrich): “There are cases of anthrax progressing rapidly to death without the patient or his peers having any indication of the grave nature of the infection and in whom only autopsy proved the cause of death by anthrax. In such fulminant cases, the cause of sudden death is usually an intrameningeal hemorrhage.” The intrameningeal hemorrhages caused by anthrax are reported to have a highly characteristic feature with regard to their distribution pattern. Either the extravasation is very regularly spread across the entire brain – a feature not seen with any other cause of intrameningeal hemorrhaging - or the blood is pooling on the cerebral surface, particularly over the convexities. In very rare cases, the parenchyma can be involved in such a fashion that numerous capillary hemorrhages are seen in the cortex, the ganglia and the white matter.
As a path of infection, the nasal air passages are the likely route. This has been anatomically and histologically proven in Risel’s and Schmorl’s cases.
CASE DESCRIPTION:
K. R. is a 24-year-old, unmarried, unskilled laborer. She was admitted on 02/19/40.
Past Medical History: She has always been healthy. Three days before admission she developed an acute illness, with vomiting, severe headaches and chills. Several hours after the onset of her illness she became somnolent.
The examination upon admission showed her to be a well-developed female of average build. The skin was normal. She was deeply obtunded. The pulse was 96 and the temperature 39.8 Celsius. She either did not respond to stimulation or mumbled unintelligibly. The neck was retroflexed and stiff. The left upper extremity appeared paralyzed and there was also apparent weakness in the left lower face and the left lower extremity. The fundoscopic examination was unremarkable.
The clinical picture on the following day (02/20) was grossly unchanged.
On 02/21 the patient underwent a lumbar puncture, which yielded hemorrhagic cerebrospinal fluid. The bacteriologic examination (Professor Stoerk) yielded numerous, large, Gram positive anthrax-like rods. The cerebrospinal fluid cultures grew anthrax bacilli. The Wassermann reaction was strongly positive in the CSF, but negative in venous blood.
On 02/22 the patient’s temperature dropped into the normal range of 36.8. During the preceding days the temperature had been elevated between 38.5 and 39.5. During the afternoon of 02/22 the temperature again rose to 38, but dropped into the normal range by the evening and remained normal throughout the further course. Already by 02/22 the patient’s sensorium had improved to nearly normal and she only complained of headaches.
On 02/23 she appears cognitively normal. Her headaches have resolved. The left-sided hemiparesis is now obvious and most pronounced in the left upper extremity.
A repeat lumbar puncture on 02/24 yielded clear spinal fluid. The bacteriologic evaluation and the Wassermann reaction were negative.
In the further course the patient showed persistent left-sided weakness, which only improved in the leg. The arm remained nearly completely paralyzed with early contractures. After many months, the left lower facial weakness also persists nearly unchanged.
The Wassermann reaction in the blood remained normal during the entire observation period and was also negative in the last spinal fluid sample obtained on 03/01.
Our patient had bacteriologically confirmed anthrax meningitis and anthrax encephalitis, with spontaneous resolution, but a residual left hemiparesis. Its causation by an intracerebral hemorrhage appears beyond doubt. It is difficult to imagine that focal destruction of the cortical surface by the intrameningeal hemorrhage was causing the motor deficits. The absence of irritative phenomena would further support an intraparenchymal cause of the weakness.
I was unable to identify another nonfatal case of anthrax meningitis in a detailed review of the literature. However, in discussing this patient with colleagues, I heard about two similar cases. Hofrat Kolisko graciously told me about a case that another colleague had observed. A second patient was seen by Dr. Bylof, who, at the time, practiced in Graz. I gratefully acknowledge his personal communication. His patient worked as a stable hand. Clinically, he presented with acute onset, severe headaches and other symptoms suggestive of a severe meningitis. Following a lumbar puncture, which yielded blood-tinged fluid and anthrax bacilli, the patient improved surprisingly quickly and recovered. Thus, this patient’s course was analogous to ours. Clinical improvement following lumbar puncture would suggest that this procedure was of benefit in both patients. However, the small number of cases does not support firm conclusions in this regard. Nonetheless, our experience indicates that a relatively benign and short lasting form of anthrax meningitis exists that has a cyclical course (translator: it is not clear what the author means by cyclical course). Whether this form of anthrax meningitis is accompanied by anthrax sepsis is uncertain, but probable, considering the findings in epidemic meningitis.
The residual hemiparesis in our patient, as pointed out, suggests a hemorrhage into the internal capsule. We, therefore, propose that anthrax infection of the central nervous system not only causes petechial hemorrhages, which are mostly asymptomatic, but also larger, intraparenchymal hemorrhages that can cause focal neurologic deficits.
A new observation in my case is a strongly positive Wassermann reaction in the cerebrospinal fluid at the height of her neurological illness. This resolved quickly and in parallel with her rapid clinical improvement.
Anthrax Meningoencephalitis:
Appendix 2
CSF results in anthrax meningoencephalitis
by survival status
Douglas J. Lanska, M.D.
Veterans Affairs Medical Center
Great Lakes VA Healthcare System
Tomah, WI;
Department of Neurology
University of Wisconsin
Madison, WI
FIGURE LEGEND: CSF results in anthrax meningoencephalitis by survival status. The figure shows results for glucose, protein, red cells, and white cells from the initial lumbar puncture in cases with and without survival (designated “A” for alive and “D” for deceased, respectively). Given the small number of cases with survival, individual results for this group are plotted (horizontally “jittered” as necessary to allow visualization of each point). For deceased cases, results are summarized with Tukey box plots, with the “box” showing the 25th percentile, median, and 75th percentile values, the “whiskers” showing the 10th and 90th percentiles, and individually plotted (“unjittered”) values showing the extreme values.