Sarala Das

IMPROVING CANCER RADIOTHERAPY WITH 2-DEOXY-D-GLUCOSE: PHASE I/II CLINICAL TRIALS ON HUMAN CEREBRAL GLIOMAS

PURPOSE Evaluation of tolerance, toxicity, and feasibility of combining large fraction (5 Gy) radiotherapy with 2-deoxy-D-glucose (2DG), an inhibitor of glucose transport and glycolysis, which has been shown to differentially inhibit repair of radiation damage in cancer cells. METHODS AND MATERIALS Twenty patients with supratentorial glioma (Grade 3/4), following surgery were treated with four weekly fractions of oral 2DG (200 mg/kg body weight) followed by whole brain irradiation (5 Gy). Two weeks later, supplement focal radiation to the tumor (14 Gy/7 fractions) was given. Routine clinical evaluation, x-ray computerized tomography (CT), and magnetic resonance (MR) imaging were carried out to study the acute and late radiation effects. RESULTS All the 20 patients completed the treatment without any interruption. The vital parameters were within normal limits during the treatment. None reported headache during the treatment. Mild to moderate nausea and vomiting were observed during the days of combined therapy (2DG + RT) in 10 patients. No significant deterioration of the neurological status was observed during the treatment period. Seven patients were alive at 63, 43, 36, 28, 27, 19, and 18 months of follow-up. In these patients, the clinical and MR imaging studies did not reveal any late radiation effects. CONCLUSIONS Feasibility of administering the treatment (2DG + 5 Gy) is demonstrated by the excellent tolerance observed in all 20 patients. Further, the clinical and MR studies also show the absence of any brain parenchymal damage.

Spinal intramedullary cysticercosis

OBJECTIVE Spinal intramedullary cysticercosis is an uncommon manifestation of neurocysticercosis. We review our experience with eight cases of intramedullary cysticercosis. METHODS Eight patients who were surgically treated for spinal intramedullary cysticercosis between 1982 and 1991 were retrospectively reviewed, and the final outcomes were assessed. RESULTS In six patients, the cysticercosis involved the thoracic cord, whereas in the other two, the cysticercosis was cervical in location. Only one patient had multiple soft tissue calcifications, as revealed by plain radiography. Myelography indicated an intramedullary lesion in each of seven patients; two of the seven patients had partial myelographic block, suggesting the segmental nature of the lesion. Cerebrospinal fluid studies were noncontributory. One patient had three cysts, whereas the other seven had one cyst each. Four patients had adjacent soft purulent materials, which were revealed by histopathological examination to be granulation tissue. The neurological statuses of seven patients improved after surgery. Six patients were followed up for a mean of 30.6 months (3 mo-5 yr). Three could resume their previous occupations, two others could manage their daily activities, and one required only minimal assistance for daily activities. CONCLUSION The outcome of intramedullary cysticercosis is not as dismal as reported earlier, and patients with paraplegia also have favorable outcomes. A preoperative diagnosis of cysticercosis can be suspected in an endemic area in the presence of multiple soft tissue calcifications and segmental lesions revealed by myelography or magnetic resonance imaging studies.

Neurocytoma/rhabdomyoma (myoneurocytoma) of the cerebellum

Abstract An unusual case of cerebellar neurocytoma with rhabdomyomatous differentiation in a 6-year-old boy is reported. Immunocytochemical and ultrastructural features of the tumour were studied. Abortive synapse formation, the presence of clear vesicles and synaptophysin immunoreactivity of the tumour cells indicated its intermediate neuronal differentiation, while the presence of myoblasts and myotubes and immunolabelling by desmin confirmed the rhabdomyomatous differentiation. In addition, the mesenchymal cells variably expressed neurofilament protein and glial fibrillary acidic protein, suggesting inductive interaction between the neuroectodermal and ectomesenchymal elements and persistence of the pleuripotential nature of the cells along the rhombic area of the brain stem.

Benign triton tumor of the trigeminal nerve

Abstract Benign triton tumor (neuromuscular hamartoma) is a rare tumor of the peripheral nerves, comprising mature neural and striated muscle elements. We report the third case, according to the literature, of a benign triton tumor affecting the Vth cranial nerve in a 4-year-old male child. The tumor was seen to involve the mandibular division of the trigeminal nerve in the infratemporal fossa and extend intracranially by eroding the base of middle cranial fossa. The mass was totally excised. In the tumor, bundles of axons intermingling with fascicles of relatively mature skeletal muscle were found. This type of hamartomatous lesions of neuroectodermal-mesenchymal origin could have arisen as the result of incorporation of mesenchymal tissue into nerve sheaths during embryogenesis or aberrant differentiation of neuroectodermal component into mesenchymal elements.

Recurrences in meningioma after surgery

Summary123 cases of meningioma operated upon between 1975 and 1984 were reviewed. There were 13 cases of symptomatic tumour recurrence and 15 cases of tumour progression. Both, recurrence and progression of the tumour were seen twice as often in males. They were not related to any particular age group or site of origin. Recurrecne was more common in the angioblastic type of meningioma. In the majority, progression of the tumour was seen within two years of the initial operation, while recurrence of the tumour was distributed evenly over time.

Balo's concentric sclerosis: a variant of multiple sclerosis associated with oligodendroglioma

We report a case of Balos concentric sclerosis, a rare and acute variant of multiple sclerosis, which coexisted with an oligodendroglioma. The demyelinating lesion was clinically silent and was detected at necropsy. The pathogenesis of concentric sclerosis and the relationship of the demyelinating lesion to the evolution of the glial neoplasm is briefly discussed.

Pathology of Madras type of motor neuron disease (MMND) – a histological and immunohistochemical study

Abstract A neuropathological report of Madras type of motor neuron disease (MMND) is presented and the differences from other forms of MND are discussed. An 18-year-old girl presented with nerve deafness and slowly progressive bulbo-spinal muscular atrophy, characteristic of MMND. Post-mortem examination of the spinal cord showed a severe loss of anterior horn cells, prominent dilatation of vessels, diffuse, but sparse sprinkling of microglial cells and lymphocytes, and demyelination and sclerosis of the ventrolateral columns. Neuronal depletion and marked gliosis was noted in the cochlear nucleus on both sides, while other bulbar motor nuclei were also involved. The cochlear nerve showed demyelination and axonal loss. Trigeminal and vestibular ganglia revealed features of ganglionitis. The possibility of an inflammatory aetiology for MMND needs to be considered.

Neuroanatomy ofCysticercus cellulosae (Cestoda) as revealed by acetylcholinesterase and nonspecific esterase histochemistry

The nervous system ofCysticercus cellulosae, the metacestode stage of the tapewormTaenia solium, was delineated using histochemical methods for the localization of the enzyme markers; nonspecific esterase and acetylcholinesterase. The main features of the nervous system include a pair of cerebral ganglia, a circumcerebral nerve ring, a rostellar nerve ring, and anterior and posterior nerves and their branches. The posterior nerves form a subtegumental network in the strobila and the bladder wall. A nerve network around excretory tubules could also be demonstrated, suggesting neuronal control of excretion in the metacestode. No sheath was observed around the nervous system. The morphological features described suggest “cephalization” of the nervous system in this parasite.

Epidemic of acute inflammatory myopathy in Karnataka, South India : 30 cases

Thirty patients of acute inflammatory myopathy were seen over a short period of 11 months (February to December 1986) at NIMHANS, Bangalore, South India. The characteristic features were: short febrile illness followed a few days later by myalgia, edema of extremities, severe motor weakness and involvement of multiple other systems. Their mean age was 32.3 years and M:F ratio was 4:1. CK levels were increased in all. EMG done in 23 patients showed spontaneous activity in 13 and myopathic pattern in all. Nerve conduction studies revealed abnormalities in 12 cases. Muscle biopsy done in 21 patients showed varying degree of myophagocytosis and inflammatory infiltrates. All patients received steroids for only 6–8 weeks. Twenty‐two patients recovered, one developed residual disability and 7 patients died during the acute phase. None of the survivors has developed relapse so far. Such cases with monophasic illness in clusters have not been reported earlier.

Variable histomorphology of muscle in congenital muscular dystrophy

Congenital muscular dystrophy (CMD) is a relatively uncommon disease with a controversial nosological status. That collagen synthesis could be the primary abnormality has been suggested earlier (Fidzianska et al., 1982). Amongst eighteen cases of CMD diagnosed during the past twelve years, muscle biopsy in three cases revealed prominence of myofibre necrosis and phagocytosis, and serum CPK was markedly elevated suggesting a rapidly progressive form. In twelve cases, marked increase in endomysial collagen, pronounced fallout of myofibres and significant fibre diameter variation was seen. This was associated with myonecrosis and regenerative activity of mild degree resembling the classical form of CMD. In the remaining three cases, polyfocal, polyphasic necrosis was noticed. Fibre splitting was more frequently observed, better delineated in the enzyme histochemical preparations, affecting both fibre types, while endomysial fibrofatty tissue was only moderately increased. The histomorphology in the latter group resembled that of limb girdle dystrophy. Ultrastructural findings in all the eighteen cases correlated well with light microscopic observations. lmmunohistochemical studies done on three of the eighteen cases showed normal localization of dystrophin protein. Such variable histomorphology, revealing a spectrum of myopathic features, suggests that the primary change in CMD is likely to be in the myofibre rather than in collagen synthesis.