Exploring immunoreactivity of TTF-1 and AVP in hypothalamic hamartoma.

INTRODUCTION: Hypothalamic hamartoma (HH) is a rare developmental disorder presenting with gelastic seizures or precocious puberty attributed to gonadotrophin-releasing hormone expression by the hamartoma. The histogenesis of HH is uncertain, and diagnosis of HH is difficult in small biopsies due to its close resemblance to normal hypothalamic nuclei. TTF-1 and arginine vasopressin (AVP) are associated with gonadotropin-releasing hormone release. MATERIALS AND METHODS: In this study, we explored the expression pattern of TTF-1 and AVP in HH and its utility, if any, in diagnosis. We reviewed the clinical, radiologic, and histopathological features of 23 HH diagnosed over the past decade at our Institute. RESULTS: The age at presentation ranged from 11 months to 34 years with gelastic seizures (82.6%), precocious puberty (17.4%), and developmental delay (8.7%) as presenting symptoms. On imaging, all the lesions (n = 9) involved the posterior and tuberal group of hypothalamic nuclei, while 5 cases involved the anterior hypothalamus. Anatomically, the lesions involved mammillary body, arcuate and periventricular nuclei. On histopathology, 52% cases revealed nodular arrangement of small neurocytic cells separated by glial stroma. TTF-1 and AVP immunoreactivity was absent in all the cases, whereas in normal hypothalamus, AVP was expressed in periventricular nuclei. CONCLUSION: Our results suggest that immunoexpression of TTF-1 is absent in HH, particularly in those arising from the posterior hypothalamus, and this can be used in small biopsies to distinguish from a normal hypothalamus as well as from posterior pituitary tumors.

Long-term stability of effects of subthalamic stimulation in Parkinson's disease: Indian Experience.

Reports of long-term effects of subthalamic (STN) stimulation for Parkinson's disease (PD) are few, mostly open-label evaluations and from Western centers. We used single-blind and open-label motor, cognitive and quality of life (QOL) evaluations to study the effects of bilateral STN stimulation in 45 patients over 5 years. Our patients showed a stable and substantial reduction in the cardinal signs of PD, motor fluctuations, and dyskinesias but less so for axial signs. The reduction in medications and the intensity of electrical stimulation needed also remained stable during follow up. Although the total QOL and its parkinsonism and social components showed sustained benefits till 5 years, the gains in emotional and systemic subsets were short lasting. Global scores for mood and cognition did not show significant worsening. Benefits of STN stimulation on the cardinal signs, motor complications, and QOL of advanced PD were substantial and sustained till 5 years. The initial benefits in axial motor signs and emotional and psychological aspects of QOL did not show similar stability. In general, the procedure had insignificant impact on cognition and mood. This is the first report of STN stimulation in Asian patients with PD.

Choroid plexus tumors: an institutional series of 25 patients.

PURPOSE: Choroid plexus tumors (CPT) are rare neoplasms that pose considerable treatment challenges. This study reviews a single institute's experience with 25 patients of CPT and attempts to contribute to the general body of knowledge on CPT. MATERIALS AND METHODS: A retrospective analysis of the case records of 25 patients operated for CPT since January 1998 and having a minimum of 1 year follow-up. RESULTS: The study group included 12 (48%) cases of choroid plexus papilloma (CPP), 09 (36%) cases of choroid plexus carcinoma (CPC) and 4 cases of atypical CPP. The mean age at presentation was 18.6 years (range, 6 months to 54 years; SD, 18.7) and a male preponderance was noted (17:8). Raised intracranial pressure was the commonest presenting symptom (72%). The tumors were distributed as follows: lateral ventricle (16; 64%), fourth ventricle (5; 20%), fourth ventricle with cerebellopontine angle extension (3; 12%), and third ventricle (1; 4%). A complete surgical excision was achieved in 11 cases of CPP and 8 cases of CPC. Operative complications include pneumocephalus (40%), focal deficits (36%), subdural effusion (32%), and persistent hydrocephalus requiring shunt (24%). All patients with CPP had a good outcome at the end of a mean follow-up of 5.4 years, whereas the median survival for patients with CPCs who underwent a subtotal resection with adjuvant therapy was 36 months. CONCLUSION: CPTs include a spectra ranging from CPP to CPC. Radiologic and histologic characterization of these tumors is difficult and newer immunohistochemical and genetic studies should be done to differentiate them from each other. Total excision offers a good prognosis and should be attempted for all forms of CPTs. CPPs carry a good prognosis, and adjuvant therapy is not indicated even after partial excision. CPCs and atypical CPCs carry a poor prognosis, and adjuvant therapy improves survival marginally after total excision. Spinal drop metastases are common for CPC and screening of the spine for possible metastasis should be part of the routine preoperative and postoperative investigation protocol.