328 MEDICAL MYCOLOGY 



ease with 100% fatality is occasional in Europe and America, although it prob- 

 ably is much more common than the number of cases in the literature would 

 indicate. 



Freeman (1931) summarizes its features as follows: The onset is accom- 

 panied by headache that becomes progressively more persistent and severe, 

 associated with stiffness of the neck and pain in it and in the limbs, and with 

 nausea and vomiting. Disturbances in sleep, amblyopia and diplopia are next 

 most common and mental phenomena, suggesting organic disease of the brain, 

 follow. This syndrome occurs in an individual whose bodily functions are not 

 otherwise upset and suggests some primary disturbance in the central nervous 

 system, either chronic meningitis, atypical epidemic encephalitis, or unlocal- 

 ized neoplasm. The frequent association of these symptoms in an individual 

 who has definite indications of some chronic respiratory infection or lymphatic 

 enlargement usuallj^ leads to the tentative diagnosis of tuberculous meningitis. 



No treatment so far attempted seems to have had any fungistatic effect on the 

 organism, although frequent liunbar punctures to reduce intracranial pressure 

 bring temporary relief of the mental state and a highly nutritious diet (up to 

 5,000 calories) , by tube if necessary, has prevented the extreme emaciation which 

 has usually accompanied this disease. Indications of involvement of other por- 

 tions of the body are unusual. 



Necropsy reveals a granulomatous meningitis, usually most marked about 

 the base, bearing some resemblance to tuberculous meningitis. The cerebral 

 subarachnoid fluid and the ventricular fluid are often turbid and even slimy 

 or gelatinous. In over half the cases there is also an invasion of the cerebral 

 cortex that appears as blisters or pits, in the more advanced cases like soapsuds. 

 The contents are clear to turbid, gelatinous and do not flow out when they are 

 cut across. Discrete granulomata are occasionally encountered, spherical with 

 compression of the surrounding tissues. The cerebellar meninges are often 

 invaded, but the parenchyma less so, although the white matter may show 

 mottling or even fissures. 



Microscopically there appear to be three types of lesions, meningeal, peri- 

 vascular, and embolic. The intracerebral lesions may be either cystic or 

 granulomatous and are associated with varying degrees of inflammatory in- 

 filtration. The meninges show diffuse or focal granulomatous lesions with 

 endothelial hyperplasia, fibrosis, moderate round cell infiltration, and a num- 

 ber of foreign body giant cells. Organisms are usually numerous. There is 

 seldom any inflammatory reaction in the cerebral substance, although endo- 

 thelial hyperplasia may be pronounced. The cysts are due to enormous col- 

 lections of organisms with their surrounding mucoid capsules, often associated 

 with very little reaction on the part of the mesodermal elements. The granu- 

 lomata are due to large aggregations of endothelial cells, many of which have 

 phagocytized the invading organisms. The mottling observed in the fresh 

 specimen is due to vast disruption of the parenchymatous elements. The 

 larger granulomata have delicate fibrillar reticulum but consist mostly of the 



