Operation Could Be Performed. the Conclu- Lege and Hospital
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J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.48.11.1188 on 1 November 1985. Downloaded from 1188 Letters Chronic mucormycosis manifesting as received, steroids and antituberculous operation could be performed. The conclu- hydrocephalus therapy were started. The patient deterior- sion would seem to be that fungal infection ated and died on 22 January 1984 should be excluded in all patients with Sir; Fungal infections of the brain usually from cardiorespiratory arrest following hydrocephalus of unknown aetiology present as chronic meningitis. Mucor- presumed tonsillar herniation. before shunt operations are undertaken. mycosis of the brain, however, typically At necropsy the brain was oedematous presents as acute rhinocerebral disease and and weighed 1200 g. There was bilateral LATA S BICHILE is fatal.' A search of the English literature tonsillar herniation. The basal meninges SHASHIKALA C ABHYANKAR revealed only three cases of chronic were thickened and pearly white in colour. N K HASE meninigitis due to mucormycosis. All had Whitish gelatinous exudate was seen filling Departments of Medicine and Pathology, rhinocerebral involvement and underlying the basal cisterns and covering the front of Lokmanya Tilak Municipal Medical Col- metabolic disease.23 Fungal meningitis pre- the pons and medulla. The cranial nerves lege and Hospital, senting primarily as hydrocephalus is rare. and the basal vessels were entangled in the Sion, Bombay 400 022. We here report such a patient. There were exudate. Sections revealed dilated lateral India. three uncommon features: (1) The primary ventricles whose walls were smooth. There presentation as hydrocephalus, (2) The were no infarcts or abscesses in the brain presence of chronic basal meningitis substance. Microscopically the brain References without rhinocerebral involvement, (3) showed granulomatous lesions consisting The occurrence in an immunocompetent of giant cells and epitheloid cells with nec- 'Emmons CW, Binford CH, Utz JP, Chung KJ. host. rotic centres containing fungi with broad Medical Mycology, 3rd ed., Philadelphia, A 22-year-old female was admitted non-septate hyphae. Sections from the Lea and Febiger, 1977:254-82. 2 Julian to hospital on 5 January, 1984 with nose and sinuses did not show evidence of EF, Cohen SH, Harold DR, Rytal MW. Chronic rhinocerebral phycomycosis in complaints of convulsions, vomiting and necrosis or mucormycosis. A methanamine association with diabetes. Post Grad Med J headache of three months duration silver stain of brain confirmed that infec- 1977; 53:337-42. together with diplopia and unsteadiness for tion was mucormycosis. Gomori Helderman JH, Cooper HS, Mann J. Chronic 15 days. She denied fever, cough, expecto- methanamine silver stains all forms of fungi phycomycosis in a controlled diabetic. Ann guest. Protected by copyright. ration, pain in the neck or discharge from black. Mucormycosis shows broad non- Int Med 1974;80:419-21. the ears. She was conscious and oriented. septate hyphae. The diameter of these Emmons CW, Binford CH, Utz JP, Kwan The pulse rate was 50/min, the BP 140/80 hyphae is 10-15 As, which differentiates Chung KJ. Medical Mycology, 3rd ed., mm Hg and respiratory rate 30/min. There them from Aspergillus, whose hyphae are Philadelphia, Lea and Febiger, 1977:58-59. was bilateral 'Udani PM, Parekh UC, Dastur DK. Neurolog- papilloedema. The cranial septate and of 3-4 ,u in diameter. The ical and related syndromes in CNS tuber- nerves and the motor and sensory systems uniform dichomatous pattern of branching culosis. J Neurol Sc 1971; 14:431-57. were normal. The tendon reflexes were all of hyphae in tissues also helps to differenti- 6 Deborah M, Gerding DN, Peterson LR, present and the plantar responses were ate Aspergillus from mucormycosis.4 Sarosis GA. Fungal meningitis manifesting extensor. Brudzinski and Kernig signs were Rhinocerebral mucormycosis usually as hydrocephalus. Arch Int Med negative. There was bilateral incoordina- presents as acute meningitis, meningo- 1983; 143: 728-3 1. tion of the limbs, trunkal ataxia and nys- encephalitis or cerebral abscess. Only three tagmus. Romberg's sign was negative. cases of chronic mucormycosis have so far Examination of the abdomen and the car- been reported. These presented with typi- diovascular and respiratory system did not cal sinus and rhinocerebral involvement Impaired neurotransnitter amine metabol- reveal any abnormality. The possibility of and histologically showed chronic ism in arginase deficiency an intracranial space occupying lesion with granulomatous inflammation.23 All had raised intracranial pressure was enter- underlying metabolic disease. Our patient, Sir: Arginase deficiency, an inborn error of tained. The bilateral cerebellar deficit was however, had no such underlying illness. the urea cycle leading to accumulation of considered to be a false localising sign. The history of convulsions, headache and arginine, causes a severe progressive Investigations revealed Hb 12 g%, total vomiting suggested a space occupying neurodegenerative disorder characterised white count 6900/cmm, differential leuco- lesion. The CT scan, however, only by mental retardation and a spastic dip- cyte count: polymorphs 50%, lymphocytes revealed hydrocephalus. In India tubercul- legia.' 2 The mechanisms responsible for http://jnnp.bmj.com/ 42% and eosinophils 8% and ESR 77 mm ous meningitis is the commonest cause of the neurological damage are uncertain. It is in the first hour. Blood sugar was 108 mg/dl acquired hydrocephalus and our patient unlikely to be due to hyperammonaemia fasting and post prandial 120 mg/dl. The was given anti-tuberculous treatment.5 Of alone since only moderate ammonia blood urea, creatinine level and liver four reported patients with fungal mening- accumulation occurs in this disorder which function tests were within normal limits. itis, primarily presenting as hydrocephalus, more closely resembles an amino- The chest radiograph was normal but three had cryptococcal infection and one acidopathy such as phenylketonuria than radiographs of the skull revealed had aspergillosis.6 The diagnosis especially the other urea cycle disorders.2 In a patient osteoporosis of the posterior clinoid pro- of cryptococcosis has become easier, but with arginase deficiency we have recently cesses. A CT scan showed hydrocephalus. the diagnosis of mucormycosis remains observed a disturbance of cerebrospinal on September 30, 2021 by Lumbar puncture was not performed notoriously difficult owing to the altered fluid (CSF) catecholamine and serotonin because of papilloedema and the absence haemodynamics of CSF flow following metabolism similar to that in patients with of signs of meningeal irritation. The patient basal meningitis and the fact that the CSF "classical" phenylketonuria. The findings was put on anticonvulsants and mannitol. is nearly always devoid of the relevant organ- are consistent with the view that inhibition After the report of the CT had been isms. Our patient died before any shunt of aminoacid uptake by the brain is a com-.