A review of adult-type diffuse gliomas in the WHO CNS5 classification with special reference to Astrocytoma, IDH-mutant and Oligodendroglioma, IDH-mutant and 1p/19q codeleted

The fifth edition of the World Health Organization (WHO) Classification of Tumors of the Central Nervous System (WHO CNS5) features several changes in the classification, diagnostic criteria, nomenclature, and grading of diffuse gliomas. Adult-type diffuse gliomas are genetically defined and include astrocytoma, isocitrate dehydrogenase (IDH)-mutant, oligodendroglioma, IDH-mutant and 1p/19q codeleted, and glioblastoma, IDH-wildtype. This review briefly discusses two tumor types: astrocytoma, IDH-mutant, and oligodendroglioma, IDH-mutant and 1p/19q codeleted, with emphasis on relevant changes in their classification and defining molecular genetic alterations. A simplified approach to the diagnosis of these tumors is provided.

Anomalous branching pattern of the axillary artery.

In routine dissection of upper extremity, in a 70yrs old male cadaver, we found the abnormal branching pattern of the third part of axillary artery on the right side. The third part of axillary artery divided into medial and lateral trunks. The medial trunk is referred to as deep brachial artery and lateral trunk is referred to as superficial brachial artery. The superficial brachial artery continues as brachial artery in arm and finally in cubital fossa divides into radial and ulnar arteries. The deep brachial artery at its origin was trapped by two roots of median nerve and later divided in to Subscapular artery, Anterior circumflex humeral artery, Posterior circumflex humeral artery and profunda brachii artery. The branching pattern of the axillary artery was normal on left side. This variation is important for surgeons for lymphectomies, to anesthesiologist and orthopedic surgeons considering the frequency of procedures done in this region.

Holoprosencephaly with cyclopia--report of a pathological study.

A rare case of a lobar holoprosencephaly with cyclopia, associated with non-nervous system anomalies is being reported.

Tuberculous osteitis of clivus.

Tuberculous osteitis of clivus is rare. Its diagnosis is difficult because of the rarity of the site and the non-specific nature of the disease. Management consists of confirmation of diagnosis by biopsy and chemotherapy with anti-tubercular drugs. Meningitis may complicate the clinical course, increase morbidity and mortality.

Glioneuronal migration and development disorders: histological and immunohistochemical study with a comment on evolution.

Glioneuronal migration disorders of the brain evolve primarily due to aberration in neuronal migration, maturation and programming in the development of various topographic zones in the brain, following pathological alterations in glial and neuronal interactions. These are broadly referred as cortical dysplastic conditions. While these dysplastic conditions involving cerebral cortex present as drug resistant seizure disorder, those involving cerebellum present as mass lesions or slowly progressing vertigo. We report 17 cases, representing the histological spectrum of dysplastic, glioneuronal migration disorders which include, hemimegalencephaly (1), tuberous sclerosis (4), Sturge Weber Syndrome with focal dysplasia (1), Dysembryoplastic neuroepithelial tumor (7) and Lhermitte Ductos disease of cerebellum (2). The dysplastic neurons in varied stages of maturation showed neuronal cytoskeletal pathology similar to that in neuro degenerative diseases, especially when associated with cytomegaly. Similarly, cells exhibiting dual expression of glial and neuronal markers were noted in the cerebral dysplastic lesions. The dysplastic glial elements probably form the subependymal giant cell astrocytomas. Dysplastic neuronal elements form the nidus for DNT. When localized, surgical resection ameliorate the symptoms in many of these condition. Study of these conditions provide better insight into glioneuronal interaction and maturation of the brain.

Vasculitic neuropathy in HIV infection: a clinicopathological study.

Vasculitis causing peripheral neuropathy may be the first sign of HIV infection. We report four such cases in whom the onset of peripheral neuropathy led to the detection of HIV infection. Two patients presented with features of mononeuritis multiplex, while the other two had a lumbosacral polyradiculopathy. A prior history of blood transfusion was forthcoming in one of the patients. Sural nerve biopsies in all the four cases and the muscle biopsy in two, histologically showed evidence of vasculitis. Immunohistochemically, the viral antigen was not demonstrable in any of the biopsies, but on electron microscope, virus-like particles were identifiable in the Schwann cell cytoplasm and the perivascular macrophages in one case. To the best of our knowledge, this is the only report that has documented the virus in the Schwann cells as well as the perivascular macrophages lending credence to the fact that these viruses are neurotropic as well as lymphotropic. Immunoglobulin deposits were not demonstrable in any of the cases, suggesting that direct viral invasion may have a role in the pathogenesis of peripheral nerve vasculitis.

Intracranial haemorrhage resulting from unsuspected choriocarcinoma metastasis.

A retrospective analysis of clinicopathological data of 10 patients with clinically unsuspected cerebral metastatic choriocarcinoma was carried out. All patients were young adult females. History of preceding pregnancy/abortion was forthcoming in 5 cases but none had a prior history of abnormal gestation. Features of raised intracranial tension followed by hemiparesis were the commonest presenting symptoms. A clinicoradiologic diagnosis of intracerebral haemorrhagic mass, either primary or secondary to tumour bleed, cortico-venous thrombosis or arteriovenous malformation, was entertained in 8 out of 10 cases. In other two cases, ring enhancing lesions prompted the diagnosis of granulomatous masses. Eight patients were operated upon, of whom two died after short hospital stay, and were autopsied. All had haemorrhagic masses noted at surgery/autopsy. Accurate diagnosis of metastatic choriocarcinoma was established only by histologic examination of these haemorrhages. This report emphasizes the importance of considering metastatic choriocarcinoma as an important differential diagnosis of haemorrhagic intracerebral lesions in women of child bearing age group. Measurement of serum/CSF level of HCG in suspected cases helps to implement early therapy. The diagnostic value of histopathologic examination of surgically resected blood clots in determining aetiology of intracerebral haemorrhagic masses is highlighted.

Giant orbital and intracranial xanthogranuloma--a short report.

Xanthogranuloma are known to arise in the paranasal sinus or orbit. They may also arise primarily in the brain. Those arising from the sinuses or orbit might involve the intracranial cavity to some extent. But an extensive involvement of the cranial compartment is very rare. This report describes one such case.

Cryptococcosis in the immunocompromised host with special reference to AIDS.

Cryptococcosis is increasing because of an ever rising population of immunocompromised individuals especially those with acquired immune deficiency syndrome (AIDS). Cryptococcal infection of the central nervous system (CNS) were diagnosed in 149 cases over a period of 19.5 years (January 1978-June 1998). Culture was positive in all cases except three who were already on antifungal therapy. India ink mounts of cerebrospinal fluid (CSF) revealed encapsulated cryptococci in 134, and cryptococcal antigen was detected in 111 of 114 patients tested. A comparison of laboratory and certain clinical parameters in patients with and without associated HIV infection showed that a poor CSF cell response and culture of cryptococci from extra-neural sites was more often associated with HIV infection and was statistically significant. Further, presence of concomitant infection especially tuberculosis, and mortality were higher in the HIV positive group.

Primary angiitis of CNS : neuropathological study of three autopsied cases with brief review of literature.

Primary angiitis of CNS(PACNS) or granulomatous angiitis of CNS is a rare inflammatory disease of small blood vessels mostly confined to the CNS. The clinical and pathological features of 3 autopsied cases are described. Clinically all the three PACNS patients were young males, age ranging from 19 to 31 years. All presented with varied neurological manifestations. There was no evidence of systemic disease in any of the cases. The ESR was normal and CSF analysis showed chronic meningitic pattern. The cerebral angiogram in one case was normal and the CT scan done in another case showed multiple intracerebral haematoma due to vasculitis. Brain biopsy was not done. Diagnosis was made at post-mortem examination. Histology showed characteristic but variable degree of granulomatous and non-granulomatous angiitis of small vessels. Venulitis with parenchymal haemorrhages was the predominant feature and in one case phlebitis with thrombosis was noted. Since the disease responds to steroids and immunosuppressive therapy, establishing antemortem diagnosis is important. In view of the association of angiitis of CNS with bacteria and viral infections, their role in the evolution of the disease needs to be investigated.

Centronuclear myopathy--morphological relation to developing human skeletal muscle: a clinicopathological evaluation.

Centronuclear myopathy (CNM), an uncommon condition, is one of the congenital myopathies. It is believed to arise as a result of maturational arrest, with persistence of myotubes postnatally. However, denervation being the basic disease process and its possible influence on central nervous system causing defect in nuclear migration has also been postulated. Keeping in view these existing controversies, we have studied 17 cases of CNM (neonatal - 1, childhood - 13, adulthood - 3) during the last twelve and a half years. Diagnosis was based on histological and enzyme histochemical findings of muscle biopsy along with clinical data. Ultrastructural characterstics of muscle have been studied in 10 cases. The affected muscle fibres showed a central nucleus (40-99%) with perinuclear halo. Type I fibre predominance with hypoplasia was consistently seen. Fibre type disproportion was noticed in 7 cases. The neonatal form revealed dense oxidative enzyme reaction product in the centre. The morphological features of CNM were compared with foetal skeletal muscles obtained at gestational ages ranging from 9 weeks - 36 weeks (n = 18). In the severe neonatal form th myofibres resembled the foetal myotubes. In the less severe childhood and adult form of CNM, aberrant organization of cytoskeletal network might have played a pathogenetic role in causing the disease.

Profile of neurologic disorders associated with HIV/AIDS from Bangalore, south India (1989-96).

One hundred patients (95 males, 5 females, mean age at presentation 31.6 +/- 9.4 yr) with various neurological disorders associated with HIV infection during 1989-1996 were evaluated at NIMHANS, Bangalore. Eighty patients belonged to group I associated with opportunistic neuroinfections and 20 to group II--non infectious neurological disorders. Cryptococcal meningitis either alone (n = 31) or associated with tuberculous meningitis (n = 6) was the most common (46.3%) followed by neurotuberculosis either alone (n = 24) or with cerebral toxoplasmosis (n = 4) accounting for 35 per cent. Other opportunistic neuroinfections included cerebral toxoplasmosis, herpes zoster, fulminant pyogenic meningitis and neurosyphilis. Clinical characteristics, diagnostic clues, their laboratory and radiological profiles and problems encountered in diagnosis and management of these opportunistic infections are highlighted. In group II (19 males and one female; mean age of 32.6 +/- 9.4 yr), two patients had cortical dementia, three acute brain stem involvement, two epilepsy and one had features suggestive of progressive multifocal leukoencephalopathy. Two patients of group I during follow up developed cortical dementia. Six had peripheral nervous system involvement similar to Guillain-Barre syndrome. Sixty six patients (63 of group I and 3 of group II) progressed to AIDS, 33 patients from group I and one patient from group II succumbed to the disease. With the rapid increase in the incidence of HIV/AIDS and an increase in the neurological manifestations of HIV/AIDS it is important to recognise the magnitude of the problem for health planning in India.

Intraventricular tuberculous abscess: a case report.

Ventricles can be involved in different ways in neurotuberculosis, however, the occurrence of intraventricular abscess has been rarely reported. We report a young woman who had intraventricular tubercular abscess. Cranial computed tomographic scan showed hypodense ring enhancing lesion in the right lateral ventricle with unilateral hydrocephalus. She underwent parasagittal craniotomy with total excision of the lesion. The pus obtained from the lesion was teeming with acid fast bacilli.

Histopathological & immunohistochemical evaluation of ageing changes in normal human brain.

As age related changes in the brain have not been systematically studied in the Indian population though there is an impression that they are less frequent, we studied 52 brains collected at autopsy from individuals above the age of 60 yr. The incidence of senile plaques (SPs) and neurofibrillary tangles (NFTs) together were found to increase with age from 21 per cent in the seventh decade to 33 per cent in the eighth decade and 54 per cent in the ninth decade, the increasing incidence of NFTs being statistically significant. The SPs were found both in the hippocampus and frontal cortex while NFTs were seen only in the hippocampus in non-demented aged individuals. In contrast, in the three cases of Alzheimer's disease (symbol: see text) AD studied, the NFTs and SPs were found in high density in both hippocampus and frontal cortex. By immunohistochemistry, various morphological forms of SPs were found to have beta amyloid protein consistently, while ubiquitin and phosphorylated neurofilament occurred variably. More number of SPs could be labelled by amyloid immunostaining than by conventional silver stains. The NFTs contained ubiquitin and phosphorylated neurofilament protein as the antigenic components, both in AD and normal ageing. The incidence of age related changes and their antigenic character in the limited sample studied from south India appears to be comparable to findings from the West. Multicentric studies on a large sample derived from different ethnic groups in India are needed to further evaluate these features.

Colloid cyst xanthogranuloma of the third ventricle--a case report.

A colloid xanthogranuloma of the third ventricle is described. Presenting features were symptoms of raised intracranial tensions and two episodes of seizures. The pathogenesis, clinical and radiological features, differential diagnosis and treatment of this rare benign lesion are briefly discussed.

Disseminated Burkitt's lymphoma presenting as multiple cranial nerve palsies.

A case of disseminated Burkitt's lymphoma with nervous system involvement in a HIV negative 35 year old lady is described. She primarily presented with multiple cranial nerve palsies. At autopsy, diffuse involvement of parenchymatous organs and lymphomatous meningitis with conspicuous sparing of gastrointestinal system was observed. In addition, there was an unusual feature of paraneoplastic demyelinating peripheral neuropathy. Incidentally, a large hydatid cyst was also seen in the left lobe in addition to the lymphomatous involvement of the liver.

Pathological lesions in HIV positive patients.

Pathomorphological features of 10 HIV positive individuals studied at autopsy and biopsy are described. Nine patients had evidence of neuro-AIDS and eight of them succumbed to various opportunistic infections. One surviving patient underwent a diagnostic lymph node biopsy which revealed tuberculous lymphadenopathy. Cryptococcal meningitis was the commonest CNS opportunistic infection, seen in five cases, with disseminated systemic cryptococcosis in two. The other opportunistic infections included toxoplasma encephalitis in two, with acanthamoeba infection in one patient. Pulmonary tuberculosis was noted in three patients while other bacterial infections such as meningococcal meningitis, pseudomonas septicaemia were observed in three and pneumocystis carinii pneumonia in one. One seropositive individual was clinically asymptomatic but succumbed to a road traffic accident. The brain in this case showed features of HIV associated early leucoencephalopathy. Bacterial infections caused by organisms other than Mycobacterium tuberculosis associated with AIDS are often underdiagnosed and should be considered, especially in developing countries. In cases of cryptococcal and tuberculous meningitis or multiple parasitic infections, the patients should be screened for associated HIV infection.