Neurosyphilis as a cause of dementia. Does it still exist?

Neurosyphilis as a cause of dementia. Does it still exist?

186 Letters to the Editor chloramphenicol may be controversial, since it combines a bactericidal with a bacteriostatic agent. Due to the rare occurr...

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186

Letters to the Editor

chloramphenicol may be controversial, since it combines a bactericidal with a bacteriostatic agent. Due to the rare occurrence of listerial brain abscess, a final evaluation of an optimal treatment regimen will depend on the outcome described in sporadic case reports. The present case illustrates the critical importance of receiving a specimen from a brain abscess for culture, especially when the abscess develops during antibiotic treatment. A. Sj6str/Sm, 1 I'. Olsson, 2 L. A. B u r m a n 1 a n d A. Tfirnvik 1

Departments of 1Infectious Diseases, 2Internal Medicine, Ume~ University, University Hospital, S-90185 Ume~, Sweden

References 1 Lamont RJ, Postlethwaite R, MacGowan AP. Listeria monocyto~enes and its role in h u m a n infection. J lnfect 1988: 17: 7-28. 2 Nieman RE, Lorber B. Listeriosis in adults: a changing pattern. Report of eight cases and review of the literature, 1 9 6 8 - 1 9 7 8 . Rev Infect Dis 1980; 2: 2 0 7 - 2 2 7 . 3 Cherubin CE, Appleman MD, Heseltine PNR, Khayr W, Stratton CW. Epidemiological spectrum and current treatment of listeriosis. Rev Infect Dis 1991; 13: 1 1 0 8 - 1 1 1 4 . 4 Dee RR, Lorber B. Brain abscess due to Listeria monocytogenes: Case report and literature review. Rev Infect Dis 1986; 8: 9 6 8 - 9 7 7 . 5 Larsson S, Linell F. Correlations between clinical and postmortem findings in listeriosis. Scand J Infect Dis 1979; 11: 55-58.

6 ReadEJ, OrensteinJM, Chorba TL et al. Listeria monocytogenes sepsis and small cell carcinoma of the rectum: An unusual presentation of the acquired immunodeficiency syndrome. Am ] Clin Pathol 1985; 83: 385-389. 7 LorberB. Listeriosis following shigellosis. Rev Infect Dis 1991; 13: 865-866. 8 Brown PH, Ingram CW, van der Horst C. Brain abscess caused by Listeria monocytogenes. Rev Infect Dis 1991; 13: 768-769. 9 Patey O, NedelecC, EmondJP, MayorgaR, N'GoN, LafaixC. Listeria monocytogenes septicemia in an AIDSpatient with a brain abscess. Eur J Clin Microbiol Infect Dis 1989; 8: 746-748. 10 Spitzer PG, Hammer SM, Karchmer AW. Treatment of Listeria monocytogenes infection with trimethoprim-sulfamethoxazole: case report and review ofthe literature. Rev Infect Dis 1986; 8:427-430.

Neurosyphilis as a Cause of D e m e n t i a . Does It Still Exist? Accepted for publication 3 August 1 9 9 4

Sir, Merrit defined parenchymatous neurosyphilis as a chronic and progressive meningoencephalitis caused by invasion of the brain by Treponema pallidum. 1'2 Between 5 % and 15 % patients admitted to psychiatric units during

Address correspondence to Dr Miguel Montejo, Uutdad de Enfermedades Infecciosas (Plantas 12), Hospital de Cruces, Pza. de Cruces s/n, 4 8 9 0 3 Barakaldo, Bizkaia, Spain.

the pre-antibiotic era suffered from this infection. Nowadays, thanks to penicillin, neurosyphilis has become an u n c o m m o n clinical condition. 3 Males are affected more frequently than females. The incidence of neurosyphilis peaks at between 10 and 20 years after the primary infection, although it may rarely develop within the first year. We report the case of a young w o m a n with a syphilitic dementia for 2 years, in order to emphasise that this entity still exists. Our patient was a 33-year-old woman, who had no relevant clinical history. She was admitted to the Neurosurgery service because of an active hydrocephalus. Her family reported that she had suffered from a progressive loss of memory, attention and concentration impairment, behavioural changes, sphincter incontinence and restlessness, during the previous 2 years. No motor deficit was observed. On physical examination, the patient was afebrile and disoriented as to time and place. She could not perform simple commands, and she was able to say only monosyllabic words. Neither nuchal rigidity nor papilloedema were observed. She had mydriatic pupils, slightly reactive to light. Eye movements were preserved. Motor and sensory modalities were normal. The peripheral reflexes were hyperactive, especially on the left side, and the left plantar response was extensor. The patient tended to fall to the right while walking. General examination revealed no other abnormality and there was no lymphadenopathy. Laboratory studies showed a positive V D R L test in serum, at a titre of 128. FTA-Abs and TPHA tests in serum were also positive. Other serological investigations excluded rickettsial, borrellial and neurotropic viral infection. Antibodies against HIV were not present. The CSF was clear, with glucose 70 mg/dl and protein 3 0 m g / dl. There were 370 x 106/I RBCs and 3 x 106/1 WBCs. Adenosine-deamynase (ADA) value was normal. VDRL in CSF was positive at a titre of 8. FTA-Abs and TPHA were also positive. The cultures for bacteria, mycobacteria, fungi and viruses were negative. Antibodies to Borrellia burgdorferi were not found. The chest X-ray was normal. The brain CT scan showed signs of active hydrocephalus. The NMR could not be performed due to the patient's lack of co-operation. A brain biopsy disclosed signs of encephalitis with no inclusion bodies. The silver stain did not reveal spirochaetes. Treatment was started with IV penicillin G, 24 million units daily. After 2 weeks, it was changed to IM procaine penicillin, 600,000 units daily, for a further week. While in hospital she improved slightly, being able to take part in a simple conversation, and to have intermittent control of sphincters. This however was the limit of her recovery. Dementia is the commonest clinical manifestation of

Letters to the Editor neurosyphilis. It consists m a i n l y of loss of r e c e n t m e m o r y , intellectual i m p a i r m e n t , m o o d a n d b e h a v i o u r a l c h a n g e s a n d loss of c o n c e n t r a t i o n . If the infection is n o t d i a g n o s e d a n d treated, the p a t i e n t ' s c o n d i t i o n u s u a l l y w o r s e n s a n d at the end of its course n e u r o s y p h i l i s is i n d i s t i n g u i s h a b l e from a t r u e p s y c h i a t r i c illness. P a t i e n t s m a y also p r e s e n t w i t h d y s a r t h r i a , u n s t e a d y gait, a b n o r m a l h a n d a n d t o n g u e m o v e m e n t s a n d apraxia. In t h e later stages, inc o n t i n e n c e , paresis a n d c o n v u l s i o n s m a y develop, a n d t h e y m a y lead to the p a t i e n t ' s death. The illness u s u a l l y r u n s a progressive course d u r i n g a 3 to 4 y e a r period, 45'6 t h o u g h o c c a s i o n a l l y the course m a y be acute. Frequently, t h e diagnosis is s u g g e s t e d b y the finding of A r g y l l - R o b e r t s o n pupils (small pupils w h i c h fail to r e a c t to light b u t do so to a c c o m m o d a t i o n ) . T h e y c a n be found in m o s t p a t i e n t s d u r i n g the course of t h e illness. In the CSF a h i g h p r o t e i n c o n c e n t r a t i o n , a rise in cell u l a r i t y a n d a positive ( n o n - t r e p o n e m a l test) is u s u a l l y a p p a r e n t . VDRL positivity in CSF is h i g h l y specific, false positive results b e i n g e x t r e m e l y rare. On the o t h e r h a n d , CSF FTA-Abs positivity alone is n o t diagnostic. 7 Spirochaetes are seen in 2 5 % - 4 0 % silver-stained b r a i n tissues o b t a i n e d by biopsy. In patients w i t h g e n e r a l progressive paresis, the b r a i n CT m a y r e v e a l h y p o d e n s e a r e a s in the w h i t e m a t t e r (especially in t h e frontal lobes), cortical a t r o p h y a n d v e n t r i c u l a r e n l a r g e m e n t , all reflecting the progressive n e u r o n a l loss. ~ R e c o m m e n d e d t h e r a p y consists of IV penicillin G, 12 to 2 4 million u n i t s / d a y for 10 to 14 days. 8 M u c h imp r o v e m e n t in the p a t i e n t s ' c o n d i t i o n s h o u l d n o t be expected, since n e u r o n a l d a m a g e is u s u a l l y irreversible. Only 10% of all t r e a t e d patients h a v e some r e c o v e r y of n e u r o l o g i c a l function, w h i c h u s u a l l y occurs w i t h i n weeks or m o n t h s . Unfortunately, some p a t i e n t s deteriorate even while being correctly treated. 9'1° Spain a n d o t h e r developed c o u n t r i e s h a v e r e p o r t e d n e w cases of syphilis d u r i n g t h e last few years. Most of these patients w e r e co-infected w i t h t h e HIV, a l t h o u g h some

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were n o t J 1'12 The n u m b e r of n e w cases of b o t h p r i m a r y a n d late syphilis is expected to increase in the n e x t few years. This case r e p o r t m a y help to keep in m i n d t h a t syphilis is still w i t h us. As the illness presents w i t h p r o t e a n clinical findings, a h i g h index of suspicion is n e c e s s a r y in order to m a k e a correct diagnosis. Miguel Montejo, Guillermo Ruiz-Irastorza, Koldo Aguirrebengoa, Javier Oflate and lon Aurrekoetxea* The Infectious Diseases Unit, Service of Internal Medicine and Neurosurgery Service,* Hospital de Cruces, Bizkaia, The Basque Country, Spain

References 1 Merrit HH, Adams liD, Solomon HC. Neurosyphilis New York, Oxford University Press, 1946. 2 Gjesfland T. The Oslo study of untreated syphilis: an epidemiologic investigation of the natural course of syphilitic infection based on a restudy of the Boeck-Bruusgaard material. Acta Derm Venereol 1955; 35(suppl stockh 34): 1-15. 3 Dewhurst K. The neurosyphilitic psychoses today: a survey of 91 cases. BrJ Psychiatry 1969; 115: 31-36. 4 Simon RP. Neurosyphilis. Arch Neurol 1985; 42: 606-613. 5 Tramont EC. Treponemapallidum (syphilis). In: Mandell GL, Douglas fiG, Bennet JE eds. Principles and Practice of Infectious Diseases, 3rd ed. New York, Churchill Livingstone, 1990: 1794-1808. 6 Hook m EW. Central nervous system systemic syphilis. In: Scheld WM, Whitley liJ, Durak DT. Infections of the Central Nervous System. New York, Raven Press, 1991: 639-656. 7 Swartz MN. Neurosyphilis. In: Holmes KK, Mardh PA, Sparling PF, Wiesnsr PJ eds. Sexually Transmitted Diseases. McGraw Hill, 1984: 318-334. 8 Centers for Disease Control 1989 sexually transmitted diseases treatment guidelines. MMWR 1989; 38(suppl 8): 9-19. 9 Dattner B, Thomas EW, Melco LD. Criteria for the management of neurosyphilis. Am ] Med 1951; 10: 463-467. 10 Wilner E, Brody JA. Prognosis of general paresis after treatment. Lancet 1968; 2: 1370-1371. 11 Molin A, Alvarez Sabin J, Malagelada A, Codina A. Hemiparesiaataxia en la s~filismeningovascular. Neurologia 1992; 7: 190-193. 12 Pizarro A, Fonseca E, Anciones B, Lara M, Contreras F. Long-term undiagnosed syphilis with clinical presentation of meningitis. Clin Exp Dermatol 1992; 17: 125-126.