Pediatric Macrocorticotropinoma: Do They Differ from Microcorticotropinoma?

INTRODUCTION: Cushing's disease (CD) due to macrocorticotropinoma (MC) in children and adolescents is a rare entity with limited information regarding its characteristics. The objective of the study is to describe the clinical, biochemical, imaging, management, outcome, and genetic characteristics of children and adolescents with CD due to MC and compare them with those of microcorticotropinoma (mc). METHODS: This retrospective study was conducted at a single tertiary care center. Thirty-two patients with CD and MC (maximum tumor dimension ≥10 mm on imaging) and 65 patients with mc (<10 mm on imaging) aged ≤20 years at presentation were enrolled. RESULTS: Nineteen girls and 13 boys with MC presented at a median (IQR) age of 14.5 (12.0-17.9) years. Patients with MC had higher body mass index-standard deviation score (BMI-SDS) (3.70 ± 2.60 vs. 2.59 ± 2.01, p = 0.04), more frequent neuro-ophthalmic symptoms (25% vs. 9% p = 0.04) and short stature (59% vs. 34%, p = 0.049) but less frequent livid striae (53% vs. 77%, p = 0.01), hypokalemia (12% vs. 36%, p = 0.04), and lower cortisol (nmol/L) to corticotropin (pmol/L) ratio (41.20 vs. 55.74, p = 0.04) than those with mc. The remission (59% vs. 64%, p = 1.0) and relapse (53% vs. 37%, p = 0.26) rates after first-line surgery and remission rate after radiotherapy (RT) were comparable between the two cohorts, whereas time to remission after RT (27 vs. 13 months, p = 0.05) was longer in the MC group. A patient with MC had a pathogenic germline variant in CDH23. CONCLUSION: In this large monocentric series of pediatric CD, frequent mass effect symptoms and short stature, higher BMI-SDS, less frequent livid striae, and hypokalemia with lower effective cortisol secretion characterize the MC cohort. The outcomes of surgery and RT were similar between the groups except for a longer time to remission after RT in the MC cohort. Germline variants are rare (4%) in pediatric MC.

Early Pulse Glucocorticoid Therapy and Improved Hormonal Outcomes in Primary Hypophysitis.

INTRODUCTION: The role of glucocorticoids in primary autoimmune hypophysitis (PAH) has been fraught with variability in regimens, leading to inconsistent outcomes in terms of anterior pituitary (AP) hormonal recovery. Hence, we aimed to compare the clinical, hormonal, and radiological outcomes of a standardized high-dose glucocorticoid therapy group (GTG) in PAH with a matched clinical observation group (COG). METHODS: Thirty-nine retrospective patients with PAH evaluated and treated at a single center in western India from 1999 to 2019 with a median follow-up duration of 48 months were subdivided into the GTG (n = 18) and COG (n = 21) and compared for the outcomes. RESULTS: Baseline demographic, hormonal, and radiological features matched between the groups, except pituitary height, which was significantly higher in GTG. Cortisol, thyroid, and gonadal axes were affected in 25 (64%), 22 (56%), and 21 (54%) patients, respectively, and central diabetes insipidus was seen in 7 (18%) patients. Panhypophysitis (PH) was the most common radiological subtype (n = 33, 84.6%). Resolution of mass effects was similar in both groups. Overall and complete AP hormonal recovery was significantly higher in the GTG than in the COG (12/14 [85.7%) vs. 6/14 [42.8%], p = 0.02; 10/14 [71.4%] vs. 1/14 [7.7%], p = 0.0007, respectively). Proportion of cases with empty sella were significantly higher in the COG (9/20 [45%] vs 1/17 [5.9%], p = 0.001). Among PH patients in the GTG (n = 17), we found duration from symptoms onset to treatment as the predictor of recovery. CONCLUSION: In a PH subtype-predominant PAH cohort, a standardized high-dose glucocorticoid regimen resulted in higher overall and complete AP hormonal recovery than that in the COG. Initiation of glucocorticoids in the early disease course may have been contributory.

Giant prolactinoma in children and adolescents: a single-center experience and systematic review.

PURPOSE: Giant prolactinoma (GP) in childhood and adolescence is a rare entity with scarce literature. We aimed to describe clinical features, biochemistry, radiology, genetics, management, and outcome in pediatric (≤ 20 years) GP. METHODS: Retrospective record review of 18 pediatric GP patients from our center and systematic review including these and 77 from the literature (total cohort: 95). RESULTS: GP constituted 20% of our pediatric prolactinoma cohort. In the total cohort (age: 15.4 ± 3.5 years), the majority (77, 82.8%) were males. Mass effect symptoms (88.6%), and pubertal delay/arrest in males (82.1%) were frequent. Median basal prolactin was 8649 (3246-17,532) ng/ml and the maximum tumor dimension was 5.5 ± 1.5 cm. MEN1 and AIP mutations were noted in 7 (21.9%) and 6 (18.8%) patients, respectively. Males with central hypogonadism had baseline bi-testicular volume of 20.2 ± 8.4 cc, lower LH than FSH (-2.04 ± 0.9 vs. -0.7 ± 1.6 SDS, p = 0.0075), and mostly, normal inhibin B. Majority (49/76, 64.5%) received dopamine agonist (DA) as first-line treatment with additional therapy in 35% (17/49). DA monotherapy arm had less frequent central hypothyroidism (42.9% vs 87.1%, p = 0.002) and central adrenal insufficiency (7.1% vs 66.7%, p = 0.0003) than multimodal therapy. A smaller tumor dimension (4.7 vs. 5.7 cm, p = 0.04) was associated with normoprolactinemia on DA monotherapy and AIP mutations (33.3% vs. nil, p = 0.02) with multimodal therapy. CONCLUSION: GP is characterized by male predominance with frequent delay/arrest of puberty (82%), but relative sparing of the FSH-inhibin B axis in boys. DA monotherapy may be preferred as the first-line therapy in pediatric GP.

Utility of CISS imaging in the management of tethered cord syndrome.

OBJECTIVE: To compare the utility of constructive interference in steady-state (CISS) sequence over T2-weighted (T2W) sequence in the accurate delineation of the tethering element on magnetic resonance (MR) imaging in patients of tethered cord syndrome (TCS) and correlation with intraoperative findings. METHODS: MR findings of fifty-six consecutive patients with operative findings of TCS were studied. The intraoperative findings of all patients were studied and compared with the preoperative imaging findings on T2W imaging and CISS images. RESULTS: CISS images provided more information than T2W imaging. The overall sensitivity of CISS in MR evaluation of patients with TCS was higher (99.17%) as compared with the T2W sequence (71.48%) especially in the detection of precise position/extent of tethered cord, assessment of filum terminale thickening, detection of a fibrous spur in cases of split cord malformation, detection of dorsal dermal sinus, and evaluation of its precise extent and ramifications. The area under the ROC curve was higher with CISS (0.99) than with T2W sequence (0.85) which reflects its good predictive value as a screening test. This information was useful to the operating surgeon. CONCLUSION: CISS sequence for imaging TCS can help enhance the overall surgical outcome of the patients ensuring completeness of the surgery. CISS should be routinely performed in the work-up of patients with tethered cord syndrome.

Cabergoline may act as a radioprotective agent in Cushing's disease.

CONTEXT: Conventional fractionated radiotherapy (CRT) achieves control of pathological hypercortisolism in 75%-80% of patients with persistent or recurrent Cushing's disease (CD), over a mean period of 18-24 months. Medical therapy is recommended as bridge therapy while awaiting RT effect. OBJECTIVE: To determine long-term outcome of CRT and its predictors in CD patients. DESIGN, SETTING AND PATIENTS: This is a retrospective case record analysis of 42 patients with CD who received CRT as a treatment modality and had at least 12 months post-RT follow-up. The dose delivered was 45 Gy in 25 fractions over 5 weeks. Demographic details, hormonal evaluation and radiological data were extracted from case records. Dexamethasone suppressed cortisol at cut-off of 1.8 µg/dL was used to define remission or recurrence. Possible predictors for remission and recurrence were analysed. RESULTS: The mean age at the time of CRT administration was 23.7 ± 10.7 (range: 12-48) years. A total of 29 (69%) patients achieved remission 26.5 ± 28.5 (median: 18, range: 3-120) months after RT, while 13 (31%) patients had persistent disease at last follow-up. There were no significant predictors of disease remission after CRT. Six (20.7%) patients had recurrence after a documented initial remission. Recurrence occurred 66.6 ± 25.9 (median: 74; range: 18 to 90) months after documented remission. Recurrence of the disease was exclusively seen in patients who received peri-RT cabergoline. Peri-CRT use of cabergoline was significantly associated with increased recurrence rates (P = .016). CONCLUSION: Use of cabergoline in the peri-CRT period did not affect initial remission after CRT but was associated with increased recurrence after initial remission in CD.

Genetic spectrum and predictors of mutations in four known genes in Asian Indian patients with growth hormone deficiency and orthotopic posterior pituitary: an emphasis on regional genetic diversity.

CONTEXT: Regional variation in prevalence of genetic mutations in growth hormone deficiency (GHD) is known. AIM: Study phenotype and prevalence of mutations in GH1, GHRHR, POU1F1, PROP1 genes in GHD cohort. METHODS: One hundred and two patients {Isolated GHD (IGHD): 79; combined pituitary hormone deficiency (CPHD): 23} with orthotopic posterior pituitary were included. Auxologic, hormonal and radiological details were studied. All four genes were analysed in IGHD patients. POU1F1 and PROP1 were studied in CPHD patients. RESULTS: Of 102, 19.6% were familial cases. Height SDS, mean (SD) was - 5.14 (1.63). Peak GH, median (range) was 0.47 ng/ml (0-6.59), 72.5% patients had anterior pituitary hypoplasia (APH). Twenty mutations (novel: 11) were found in 43.1% patients (n = 44, IGHD-36, CPHD-8). GHRHR mutations (n = 32, p.Glu72* = 24) were more common than GH1 mutations (n = 4) in IGHD cohort. POU1F1 mutations (n = 6) were more common than PROP1 mutations (n = 2) in CPHD cohort. With few exceptions, this prevalence pattern is contrary to most studies in world-literature. No patients with peak GH > 4 ng/ml had mutations, signifying it as negative predictor. While many parameters were significant on univariate analysis, only positive family history and lower median peak GH levels were significant predictors of mutations on multivariate analysis in IGHD patients. CONCLUSION: At variance with world literature, we found reverse predominance of GHRHR over GH1 mutations, POU1F1 over PROP1 mutations and predominance of GHRHR p.Glu72* mutations thus re-affirming the regional diversity in GHD genetics. We report positive and negative predictors of mutations in GHD.

Spectrum of phenotype and genotype of congenital isolated hypogonadotropic hypogonadism in Asian Indians.

BACKGROUND: Congenital isolated hypogonadotropic hypogonadism (IHH) is caused due to defect in GnRH neuronal development, migration and action. Although genetic aetiology of IHH is increasingly being studied, Asian Indian data on phenotypic spectrum and genetic basis are scarce. OBJECTIVE: To investigate the phenotypic and genotypic spectrum of IHH in Asian Indian subjects. DESIGN, SETTING AND SUBJECTS: A cohort of 135 IHH probands were characterized phenotypically for reproductive and nonreproductive features and screened for rare sequence variations (RSVs) in five genes KAL1, FGFR1, FGF8, GNRHR and KISS1R. RESULT: Of 135 probands [56 normosmic IHH (nIHH) and 79 Kallmann syndrome (KS)], 20 were familial cases. KS group had more male dominance (M:F ratio of 8:1) as compared to nIHH group (M:F ratio of 1·5:1). Complete absence of puberty was more prevalent in KS probands (81% in KS vs 46% in nIHH). The prevalence of MRI abnormalities was more in anosmic group (92·8%) as compared to hyposmic (37·5%) and normosmic groups (15·4%). No particular nonreproductive phenotypic predominance was seen in any group. Genotyping revealed rare sequence variation (RSV) detection rate of 15·5% in five genes studied: (KAL1 - 4·4%, FGFR1 - 4·4%, GNRHR - 6·7%, oligogenicity - 1·5%). Prevalence of RSV was more common in familial cases (35%) as compared to sporadic (12·2%). GNRHR RSV p.C279Y (not reported in patients of ethnicities other than south Asians) was recurring in four unrelated patients. CONCLUSION: In our cohort, 60% were KS with majority of males and a severe reproductive phenotype as against nIHH. Contribution of the genetic burden for the five genes studied was 15·5%. RSV p.C279Y in GNRHR may have a founder effect originating from south Asia. This study provides a model for molecular and phenotypic representation of Asian Indian subjects with IHH.

Altered Sensorium in a Young Male: Marchiafava Bignami Disease Revisited.

Marchiafava Bignami disease (MBD) is a rare and devastating complication of chronic alcoholism. Degeneration of the corpus callosum is the hallmark feature of MBD. Early diagnosis of MBD by its typical "Sandwich Sign" on magnetic resonance imaging (MRI). Prompt institution of treatment and strict alcohol abstinence can cause regression of changes and hence, clinical improvement. Here we report the case of a young chronic alcoholic male admitted with altered sensorium and his further course in ward.

Primary (autoimmune) hypophysitis: a single centre experience.

BACKGROUND: Autoimmune hypophysitis (AH) is a rare autoimmune inflammatory disorder of pituitary gland. OBJECTIVE: To analyse clinical, hormonal, radiological features and management outcomes of AH. DESIGN: Retrospective analysis of patients with primary hypophysitis (where secondary causes of hypophysitis were ruled out) was carried out from 2006 to 2012. AH emerged as the most plausible aetiology and the diagnosis of exclusion. RESULTS: Twenty-four patients with AH (21 females and 3 males) were evaluated. They presented with symptoms of expanding sellar mass (83.3%), symptoms of anterior pituitary hormone deficiencies (58.3%), and diabetes insipidus (16.7%). The anterior pituitary hormonal axes affected were cortisol (75%), thyroid (58.33%) and gonadotropin (50%). All had sellar mass on magnetic resonance imaging, which was symmetrical (91.7%) and homogenously enhancing (91.7%). Stalk thickening, suprasellar extension, loss of posterior pituitary hyperintensity and parasellar T2 dark sign were seen in 87.5, 87.5, 71.5, and 50% respectively. In addition to hormone replacement, five (20.83%) patients underwent trans-sphenoidal surgery, fifteen (62.5%) were watchfully monitored, while four cases (16.67%) received steroid pulse therapy. On follow up imaging, the sellar mass regressed in all, while, stalk thickening was persistent in 13/19 (68.4%) non-operated patients at median follow up of 1 year. Pituitary hormone axis recovery was seen in 10 (41.67%) and was seen in cortisol 10/18 (55.5%) followed by gonadotropin 5/12 (41.67%) axis. CONCLUSION: Characteristic radiology helps in diagnosis of AH even without tissue diagnosis. Non-operative treatment is the preferred treatment modality. Steroid pulse therapy potentially improves pituitary axis recovery.

Hemangioma of rib: a different perspective.

BACKGROUND: Hemangiomas are one of the common primary benign tumors of the intraosseous and soft tissue compartments in humans. Vertebral hemangiomas being the most common of all are seen in daily radiological practice. Hemangioma of the rib is rarely seen. CASE REPORT: We reported on a case of a rib lesion which had a classic imaging pattern of hemangioma. We highlighted the use of pre-operative embolization of such a vascular rib lesion before surgically removing the lesion by thoracotomy to reduce the risk of bleeding. CONCLUSIONS: We also emphasized overt complications of overzealous needling of such a vascular lesion for histopathological diagnosis as in our case.

COVID-19-Associated Rhino-Orbito-Cerebral Mucormycosis: A Single Tertiary Care Center Experience of Imaging Findings With a Special Focus on Intracranial Manifestations and Pathways of Intracranial Spread.

Background and objective The COVID-19 pandemic and mucormycosis epidemic in India made research on the radiological findings of COVID-19-associated mucormycosis imperative. This study aims to describe the imaging findings in COVID-19-associated mucormycosis, with a special focus on the intracranial manifestations.  Methodology Magnetic resonance imaging (MRI) scans of all patients with laboratory-proven mucormycosis and post-COVID-19 status, for two months, at an Indian Tertiary Care Referral Centre, were retrospectively reviewed, and descriptive statistical analysis was carried out. Results A total of 58 patients (47 men, 81%, and 11 women, 19%) were evaluated. Deranged blood glucose levels were observed in 47 (81%) cases. The intracranial invasion was detected in 31 (53.4%) patients. The most common finding in cases with intracranial invasion was pachymeningeal enhancement (28/31, 90.3%). This was followed by infarcts (17/31, 55%), cavernous sinus thrombosis (11/58, 18.9%), fungal abscesses (11/31, 35.4%), and intracranial hemorrhage (5/31, 16.1% cases). The perineural spread was observed in 21.6% (11/51) cases. Orbital findings included extraconal fat and muscle involvement, intraconal involvement, orbital apicitis, optic neuritis, panophthalmitis, and orbital abscess formation in decreasing order of frequency. Cohen's kappa coefficient of interrater reliability for optic nerve involvement and cavernous sinus thrombosis was 0.7. Cohen's coefficient for all other findings was 0.8-0.9. Conclusions COVID-19-associated rhino-orbito-cerebral mucormycosis has a plethora of orbital and intracranial manifestations. MRI, with its superior soft-tissue resolution and high interrater reliability, as elucidated in this study, is the imaging modality of choice for expediting the initial diagnosis, accurately mapping out disease extent, and promptly identifying and scrupulously managing its complications.

Volume interpolated 3D-spoiled gradient echo sequence is better than dynamic contrast spin echo sequence for MRI detection of corticotropin secreting pituitary microadenomas.

BACKGROUND: Various techniques have been attempted to increase the yield of magnetic resonance imaging (MRI) for localization of pituitary microadenomas in corticotropin (ACTH)-dependent Cushing's syndrome (CS). OBJECTIVE: To compare the performance of dynamic contrast spin echo (DC-SE) and volume interpolated 3D-spoiled gradient echo (VI-SGE) MR sequences in the diagnostic evaluation of ACTH-dependent CS. DESIGN: Data was analysed retrospectively from a series of ACTH-dependent CS patients treated over 2-year period at a tertiary care referral centre (2009-2011). PATIENTS: Thirty-six patients (24 female and 12 male) were diagnosed to have ACTH-dependent CS during the study period. All patients underwent MRI by both sequences during a single examination. Cases with negative and equivocal pituitary MR imaging underwent corticotropin-releasing hormone (CRH) stimulated bilateral inferior petrosal sinus sampling (BIPSS) to confirm pituitary origin of ACTH excess state. Thirty patients were finally diagnosed to have Cushing's disease (CD) [based on histopathology proof of adenoma and/or remission (partial/complete) of hypercortisolism postsurgery]. Six patients were diagnosed to have histopathologically proven ectopic CS. RESULTS: Of 30 patients with CD, 24 patients had microadenomas and 6 patients had macroadenomas. DC-SE MRI sequence was able to identify microadenomas in 16 of 24 patients, whereas postcontrast VI-SGE sequence was able to identify microadenomas in 21 of 24 patients. All six patients of ectopic CS had negative pituitary MR imaging by both techniques (specificity: 100%). CONCLUSION: VI-SGE MR sequence was better for localization of pituitary microadenomas particularly when DC-SE MR sequence is negative or equivocal and should be used in addition to DC-SE MR sequence for the evaluation of ACTH-dependent CS.

Perfusion magnetic resonance imaging characteristics of intracerebral tuberculomas and its role in differentiating tuberculomas from metastases.

BACKGROUND: Intracerebral tuberculomas usually manifest as ring-enhancing of nodular lesions on magnetic resonance imaging (MRI). These imaging findings are also observed in other lesions like metastases and toxoplasmosis. PURPOSE: To study the MRI perfusion characteristics of tuberculomas and its potential role in their definitive diagnosis. MATERIAL AND METHODS: Thirty-four tuberculomas were evaluated by conventional and perfusion MRI. The relative cerebral blood volume (rCBV) values of the center, peripheral wall, and perilesional neuroparenchymal tissue were calculated using rCBV maps. Ten ring-enhancing metastases were similarly evaluated and rCBV values of their peripheral walls were calculated. RESULTS: Thirty-one of the 34 tuberculomas were ring-enhancing or conglomerate lesions and revealed hypoperfused centers with hyperperfused peripheral walls, with the mean rCBV ± SD being 0.42 ± 0.25 and 2.04 ± 0.61, respectively. Three nodular enhancing lesions showed predominantly homogenous hyperperfusion, with the mean rCBV measuring 2.96 ± 0.39 (mean ± SD). The perilesional neuroparenchyma was hypoperfused in both cases. The metastases revealed mean rCBV ratio of the peripheral wall to be 5.43 ± 2.1 (mean ± SD). Analysis of the values by ROC curve method revealed a cut-off value of ≥3.745 for differentiating ring-enhancing metastases from ring-enhancing tuberculomas. CONCLUSION: Perfusion MR is a useful tool for the assessment of tuberculomas and can help differentiate them from neoplasms like metastases. It also has a potential role in monitoring therapy and for early detection of drug resistance.

Imaging of Uncommon Bacterial, Rickettsia, Spirochete, and Fungal Infections.

This article reviews uncommon bacterial (brucellosis, actinomycosis, neuromelioidosis, nocardiosis, whipple disease, and listeriosis), Rickettsia, spirochete (neurosyphilis and Lyme disease), and fungal (mucormycosis, aspergillosis, candidiasis, cryptococcosis, and Cladophialophora bantiana) diseases affecting central nervous system (CNS), focusing primarily on their cranial manifestations. These infections often show a variety of neuroimaging features that may be similar or differ from typical pyogenic bacterial meningitis and abscess. Familiarity with these patterns is essential for timely recognition and initiation of appropriate management. Neuroimaging is also useful for identifying complications of CNS infections and follow-up evaluation after initiation of treatment.

Giant prolactinoma in Asian-Indians: A single-center experience from Western India.

PURPOSE: Giant prolactinomas (GP) are rare tumors accounting for 4.3% of prolactinomas, with paucity of literature from India. We aim to describe clinical, biochemical, radiological, and treatment outcomes in a large series of Asian-Indian patients with GP. METHODS: A single-center retrospective analysis of GPs (n=84), age-based (adults: 66 versus pediatric: 18) and gender-based (males: 64 versus females: 20) comparison was done. RESULTS: The mean age at presentation was 34.1±13years, and 64 (76.2%) were males. Males were younger at presentation (32.1±12.2 versus 40.1±13.8years, P: 0.01). The majority presented with mass-effect-related manifestations (visual disturbances: 91.6%, headache: 84.5%) and/or hypogonadism (98.7%). At baseline, largest tumor dimension was 5.3±1.0cm, and serum prolactin was 8343 (3865.5-12,306) ng/mL; most (94.6%) had gonadal axis involvement. Dopamine-agonist (DA) as first-line therapy (45/67, 67.2%) achieved normoprolactinemia (maximum cabergoline dose: 2.0±1.2mg/week) in 36/45 (80%) and tumor response (≥50% reduction) in 36/37 (97.3%) patients at the last follow-up (median duration: 33 [14.5-53.5]months). Notably, gonadal axis recovery was poor (6/30, 20%) despite normoprolactinemia post-DA monotherapy. At latest follow-up, secondary hypothyroidism (32.5% versus 82.6%, P: 0.001) and central hypocortisolism (5.6% versus 42.9%, P: 0.007) were less frequent in DA monotherapy (n=43) than in multimodal therapy group (n=23). The proportion of males (94.4% versus 71.2%, P: 0.04) was higher in the pediatric age group, with DA-induced (first-line) normoprolactinemia observed in 66.7% of them. CONCLUSION: GP has male predominance, DA as first-line therapy normalized prolactin in four-fifths of patients with better preservation of HPT and HPA axes in patients with DA monotherapy.

Ectopic posterior pituitary and stalk abnormality predicts severity and coexisting hormone deficiencies in patients with congenital growth hormone deficiency.

Certain pituitary imaging abnormalities are a specific indicator of hypopituitarism. The objective of this study is to compare phenotypical features with radiological findings in patients with congenital growth hormone deficiency (GHD). Magnetic Resonance imaging (MRI) was performed in 103 patients [72 with Isolated GHD (IGHD) and 31 with Combined Pituitary Hormone Deficiency (CPHD)]. Images were assessed for the following abnormalities: (1) small/absent anterior pituitary, (2) thin or interrupted pituitary stalk (PSA), and (3) Ectopic posterior pituitary (EPP), and (4) others. Radiological findings were correlated with the clinical and biochemical parameters. MRI abnormalities were observed in 48.6% patients with IGHD, 93.5% with CPHD. Jaundice, hypoxia, hypoglycemia and breech deliveries were more common in EPP/PSA group. EPP/PSA was observed in 87.1% patients with severe GHD (peak GH < 3 µg/L) as compared to 12.9% with mild to moderate GHD (peak GH: 3-10 µg/L). Amongst CPHD, EPP/PSA was present in 80% of subjects with associated hypocortisolism ± hypothyroidism as compared to 18.2% of subjects with hypogonadism. Over a mean follow up period of 4.5 years, 5.4% of subjects with IGHD and abnormal MRI progressed to CPHD while none of those with normal MRI progressed. This study emphasizes a significant clinico-radiological correlation in Asian Indian GHD patients. MRI abnormalities in the hypothalamic pituitary area, especially EPP/PSA are more common in patients with CPHD and severe GHD. Among CPHD, EPP/PSA predicts association with hypothyroidism or hypocortisolism. IGHD with MRI abnormality may evolve into CPHD.

Numb chin syndrome secondary to infiltration of inferior alveolar nerve as a presentation of relapse in treated testicular lymphoma-diagnosis on PET/CT and MR neurography.

Numb chin syndrome (NCS) is a rare presentation of primary or recurrent malignant neoplasms among other non-neoplastic causes. The syndrome is characterized by altered sensations in the distribution of the mental nerve and presents with pain and paresthesias along the distribution of the inferior alveolar nerve and its branches. The primary diagnosis is indicated while following up patients through positron emission tomography/computed tomography (PET/CT) when a hypermetabolic focus is seen in the vicinity of the angle of the mandible. Further anatomical localization is performed using magnetic resonance (MR) neurography and postcontrast MR imaging, which demonstrates neural involvement. We hereby describe a case of a 56-year-old man, a treated case of DLBCL (diffuse large B-cell lymphoma), presenting with NCS and diagnosed with perineural invasion through PET/CT and further MR evaluation. To our knowledge, there are no other reports in the literature describing the MR neurography appearance of the inferior alveolar nerve in NCS. We hereby stress on the use of MR neurography followed by postcontrast 3D sequences with multiplanar reformatting for adequate lesion detection.

A Pictorial Review on Reversible Splenial Lesions.

Splenium of corpus callosum can be involved in a variety of pathologies causing reversible or irreversible damage. Magnetic resonance imaging (MRI) is a useful investigation to evaluate the same. In spite of the differing etiologies implicated, MRI findings can be quite common. We review the reversible causes of diffusion restriction involving the splenium of corpus callosum and highlight the etiopathologic mechanisms implicated in these pathologies. We further discuss these pathologies in entirety with relevant clinical and laboratory findings helping make definitive diagnosis and guiding appropriate management.

Does Tc-99m ECD ictal brain SPECT have incremental value in localization of epileptogenic zone and predicting postoperative seizure freedom in cases with discordant video electroencephalogram and MRI findings?

OBJECTIVE: Localization of epileptogenic focus in drug-refractory epilepsy using Tc-99m ethylene cystine dimer (ECD) brain single photon emission computed tomography (SPECT) is less studied in patients with discordant findings on video electroencephalogram (VEEG) and MRI. The study was done to evaluate brain SPECT for epileptogenic focus localization and postoperative seizure freedom. METHODS: Epilepsy patients with discordant VEEG and MRI findings underwent brain SPECT at ictal and interictal phases. Various groups unilateral/bilateral mesial temporal sclerosis (MTS), solitary and multifocal lesional, nonlesional epilepsy were studied for localization of epileptogenic focus and postoperative seizure freedom (>2 years) using Engels classification. Reasons for nonoperability was evaluated in nonoperated group. RESULTS: SPECT could localize epileptogenic focus in 49/67 (73.13%) and guided surgery in 19/33 (57.57%) patients in operated group. SPECT was useful in 12 (46.12%) of unilateral (2)/bilateral (10) MTS. Postoperative seizure freedom of Engels Class I and II in 22 (66.67%), III in six (18.2%) and IV in one patient based on SPECT findings (P = 0.0086). Overall sensitivity and specificity were 79.3% and 85.7%, respectively. SPECT could localize epileptogenic focus in 23/34 (67.64%) patients in nonoperated group; 10 (29.41%) patients refused for surgery and no epileptogenic focus was localized in the rest of 14 (41.2%). CONCLUSION: Ictal SPECT showed incremental value and was found necessary for epileptogenic focus localization and subsequent surgery in unilateral/bilateral MTS in this study. Seizure freedom in patients undergoing epilepsy surgery based on ictal SPECT assistance was comparable to the surgical group not requiring ictal SPECT.