Overreligious beliefs with neuropsychiatric manifestations in a young lady-Rule out Marchiafava-Bignami disease.

Marchiafava- Bignami disease is a rare condition characterized by demyelination of corpus callosum due to alcohol or malnutrition. Here we report a young lady who, due to her religious beliefs had stopped taking food and presented with neuropsychiatric manifestations. Neuroimaging was suggestive of Marchiafava Bignami disease and with adequate nutritional and thiamine therapy she had dramatic improvement clinically and had near complete resolution of lesions in neuroimaging.

Subconcussive Head Impact Exposure and White Matter Tract Changes over a Single Season of Youth Football.

Purpose To examine the effects of subconcussive impacts resulting from a single season of youth (age range, 8-13 years) football on changes in specific white matter (WM) tracts as detected with diffusion-tensor imaging in the absence of clinically diagnosed concussions. Materials and Methods Head impact data were recorded by using the Head Impact Telemetry system and quantified as the combined-probability risk-weighted cumulative exposure (RWECP). Twenty-five male participants were evaluated for seasonal fractional anisotropy (FA) changes in specific WM tracts: the inferior fronto-occipital fasciculus (IFOF), inferior longitudinal fasciculus, and superior longitudinal fasciculus (SLF). Fiber tracts were segmented into a central core and two fiber terminals. The relationship between seasonal FA change in the whole fiber, central core, and the fiber terminals with RWECP was also investigated. Linear regression analysis was conducted to determine the association between RWECP and change in fiber tract FA during the season. Results There were statistically significant linear relationships between RWEcp and decreased FA in the whole (R2 = 0.433; P = .003), core (R2 = 0.3649; P = .007), and terminals (R2 = 0.5666; P < .001) of left IFOF. A trend toward statistical significance (P = .08) in right SLF was observed. A statistically significant correlation between decrease in FA of the right SLF terminal and RWECP was also observed (R2 = 0.2893; P = .028). Conclusion This study found a statistically significant relationship between head impact exposure and change of FA fractional anisotropy value of whole, core, and terminals of left IFOF and right SLF's terminals where WM and gray matter intersect, in the absence of a clinically diagnosed concussion. © RSNA, 2016.

Multi-contrast echo-planar imaging sequence (Echo-planar imaging mix) in clinical situations demanding faster MRI-brain scans.

Objectives: The excellent resolution offered by magnetic resonance imaging (MRI) has a trade-off in the form of scan duration. The purpose of the present study was to assess the clinical utility of echo-planar imaging mix (EPIMix), an echo-planar imaging-based MRI sequence for the brain with a short acquisition time. Materials and Methods: This was a retrospective observational study of 50 patients, who could benefit from faster MRI brain scans. The T1, T2, fluid attenuated inversion recovery, diffusion-weighted imaging (DWI), and T2*/susceptibility-weighted imaging sequences were acquired, conventionally and with EPIMix. Conventional and EPIMix images were assessed by two radiologists for overall quality, motion, and susceptibility artifacts and scored on a Likert scale. The scores given for conventional and EPIMix images were compared. The diagnostic performance of EPIMix was also assessed by the ability to detect clinically relevant findings. Results: The acquisition time for conventional MRI was 11 min and 45 s and for EPIMix 1 min and 15 s. All EPIMix images were sufficient for diagnostic use. On assessment of the diagnostic performance, it was excellent for ischemic and hemorrhagic strokes. Smaller lesions, lesions adjacent to bone, and post-operative tumors were difficult to identify. Moderate to perfect agreement (Kappa values 0.41-1) was seen between radiologists for all categories except skull base, calvarial, and orbital lesions. Image quality, artifact assessment showed excellent interobserver agreement (>90%) for the scores. All EPIMix images showed reduced motion artifacts. The EPIMix-DWI was comparable to conventional-DWI in terms of quality and artifacts. The remaining sequences showed reduced quality and increased susceptibility. Conclusion: The EPIMix has a significantly reduced acquisition time than conventional MRI and could be used instead of conventional MRI in situations demanding faster scans such as suspected acute ischemic or hemorrhagic stroke. In other clinical scenarios, it could help tailor the MRI examination for each patient.

Primary Angiitis of the CNS: Differences in the Profile Between Subtypes and Outcomes From an Indian Cohort.

BACKGROUND AND OBJECTIVES: Primary angiitis of the CNS (PACNS) is a rare disease that has significant morbidity and mortality. Subtypes of PACNS can have different presentations that could be missed with certain diagnostic modalities, further increasing diagnostic complexity. We sought to distinguish the subtypes of PACNS and describe their outcomes in an Indian cohort. METHODS: Adult patients in this retrospective single-center cohort study were reviewed from the PACNS database between 2000 and 2019. Diagnosis was made as per Calabrese and Malleck criteria. Small and medium vessel vasculitis was defined, and their clinical and radiologic profile, treatment, and outcomes were compared. Functional outcomes were noted at 6-month, 1-year, and at last follow-up, while relapses were noted at last follow-up. A poor outcome was defined as modified Rankin Scale >2. RESULTS: Seventy-two patients fulfilled the inclusion criteria of whom 50 (69.4%) were male. The small vessel vasculitis subtype had a younger age at onset (30.5 vs 40.5 years, p = 0.014), presented less often as a stroke (22% vs 62%, p = 0.001), and had greater delay in diagnosis and treatment initiation (median of 620 days vs 118 days, p = 0.001) compared with medium vessel vasculitis subtype. Although no difference was noted at 6 months, the small vessel vasculitis group had poor outcomes at 1-year and last follow-up (57% vs 20%, p = 0.011 and 72% vs 34%, p = 0.005, respectively) and had more relapses at last follow-up (89% vs 30%, p < 0.001) when compared with the medium vessel vasculitis group. On analyzing the entire cohort, 50 of 72 (69%) and 37 of 53 (69.8%) patients had a good outcome at 6 months and 1 year, respectively. Relapse was noted in 35 of 72 (49%) at final follow-up. The choice of the treatment regimen did not predict outcomes or relapses. DISCUSSION: The small vessel vasculitis subtype of PACNS is a distinct entity that has diagnostic and treatment delays with poor long-term outcomes and more relapses. Recognizing the different subtypes of PACNS may help to expedite diagnosis and plan treatment.

Rituximab for refractory primary angiitis of the central nervous system: Experience in two patients.

Primary Angiitis of the Central Nervous System (PACNS) is an aggressive disease with a high rate of relapse and mortality. Majority of patients attains clinical remission with a combination of glucocorticoids and cyclophosphamide. However, there is limited evidence on further management in patients who relapse or does not achieve clinical improvement while on first line treatment. Here, we present two cases of PACNS in whom clinical course was complicated by recurrent strokes and radiological progression despite receiving optimal immunosuppression with glucocorticoids and cyclophosphamide. Rituximab, a monoclonal antibody, as second line agent was administered with which both patients had clinical improvement and was relapse free at one year followup.

High-Resolution Vessel Wall Imaging in Primary Angiitis of Central Nervous System.

BACKGROUND: High-resolution vessel wall imaging (HRVWI) can aid in differentiating the various intracranial vasculopathies, but has been sparingly used in the diagnosis of primary angiitis of central nervous system (PACNS). This study is aimed to describe the vessel wall imaging characteristics of PACNS. MATERIALS AND METHODS: Patients with confirmed diagnosis of PACNS according to the Calabrese and Mallek criteria who had abnormal HRVWI were included in this retrospective descriptive study. Magnetic resonance image of brain, conventional four-vessel cerebral digital subtraction angiogram, and HRVWI were read by a neuroradiologist. The vessel wall parameters assessed were T1W and T2W appearances, pattern of wall thickening and contrast enhancement, and remodeling index. RESULTS: HRVWI done in 21 patients with PACNS yielded abnormality in 20 (95.2%) who were included in the analysis. The mean age at presentation was 42.55 ± 9.48 years and 14 (70%) were males. The median number of vessels involved were four (range 2-12). The commonest vessels affected were proximal middle cerebral artery (70%) and internal carotid artery (55%). Vessel wall thickening was concentric, eccentric, and absent in 12 (60%), 1 (5%), and 7 (35%) patients, respectively. Vessel wall enhancement was diffuse in 17 (85%), eccentric in 1 (5%), and absent in 2 (10%) patients. One patient had T2W hyperintense stenotic lesion. Remodeling index was negative in 11 (55%) patients. CONCLUSION: Distinctive vessel wall appearances were observed by HRVWI in PACNS, concentric vessel wall thickening and enhancement being more frequent. Hence, HRVWI can be considered as an additional noninvasive imaging modality in the diagnosis of PACNS.

Pleomorphic adenoma of minor salivary gland with therapeutic misadventure: a rare case report.

BACKGROUND: The benign tumors of nasopharynx are least encountered tumors in otolaryngology, as nasopharynx is considered one of notorious anatomical site for the malignant tumors. Pleomorphic adenoma of the minor salivary gland of nasopharynx and parapharyngeal space is rare. We present a pleomorphic adenoma of minor salivary gland which was mismanaged. CASE PRESENTATION: An adult male presented with left nostril obstruction for five months. The examination found big mass extending from nasopharynx to oropharynx. On CT scan, this tumor was quite big and extending to the parapharyngeal space. The FNAB found it a carcinoma but it did not respond to radiotherapy. The excision biopsy of tumor revealed it as pleomorphic adenoma. We found only five published reports on this tumor arising from nasopharynx. DISCUSSION AND CONCLUSION: Although, in this case report exact origin of the tumor could not be ascertained as it also appeared to be a parapharyngeal tumor but we kept the possibility of a nasopharyngeal tumor on the basis of clinical features. The pleomorphic adenoma of nasopharynx is rare. It can be misdiagnosed as malignant epithelial tumor on histopathology. The differentiation from its malignant variant is also difficult. A possibility of benign tumor should always be kept in nasopharyngeal growth with no evidence of metastasis, and histopathological diagnosis of growth should be available before any definitive treatment.

Persistent Falcine Sinus-The Variant Venous Structure With Anterior Interhemispheric Type And The Classic Posterior Location: Report of Two Cases.

A 14-year-old girl presented with suspected dermoid cyst of scalp underwent Neuroimaging to look for intracranial communication. Her neurological examination was essentially normal. On Magnetic Resonance Imaging(MRI), an anterior persistent falcine sinus(PFS) was noted draining into the middle portion of the superior sagittal sinus. There was developmental venous anomaly (DVA) involving the right frontal lobe. To our knowledge, it is a rare instance of the combination of an anterior persistent falcine sinus and associated DVA.

Shunt Site Localization of Direct Carotid-Cavernous Fistula Using 3D Rotational Angiography-Utility of Broken-Rim Sign.

BACKGROUND: Direct carotid-cavernous fistulas (DCCF) develop due to breach in the integrity of the wall of the internal carotid artery, and its localization can sometimes be difficult due to rapid high flow shunts. We hypothesized that 3D rotational angiography could locate the fistula site accurately, where an interrupted rim of the carotid wall would be silhouetted against opacified vascular structures. This finding was described as a broken-rim sign, and in this study, we assessed the utility of this sign in the localization of the shunt point of DCCF. METHODS: Retrospective analysis of 15 cases of DCCF was performed, and the rent was characterized based on the broken-rim sign. Two observers independently evaluated the results and compared them against the intraoperative observations. RESULTS: The broken-rim sign was identified and correlated with the actual fistula site in 86.6% and 100% of patients by observers 1 and 2, respectively. The inter-rater agreement was 0.87 (P < 0.001). Misinterpretation was made in 2 patients by 1 observer, due to poor contrast opacification of the vascular structures secondary to very rapid shunting and mild movement artifacts. CONCLUSIONS: Our study highlights the role of 3D rotational angiogram and analysis of the carotid wall in the localization of the shunt site in DCCF. The broken-rim sign accurately points to the location of the fistula.

Diagnostic accuracy of fine-needle aspiration cytology of thyroid gland lesions: A study of 200 cases in Himalayan belt.

AIMS: The study is undertaken to correlate the fine-needle aspiration cytology (FNAC) findings with histopathology in a spectrum of thyroid lesions and to find the diagnostic accuracy of fine-needle aspiration (FNA) so that unnecessary thyroidectomies can be avoided in benign lesions. MATERIALS AND METHODS: This study was carried out over the period of 1-year (May 1, 2012-April, 30 2013). FNA specimens obtained from 200 patients were analyzed. Of these, only 40 patients underwent surgery and their thyroid specimens were subjected to histopathological examination. RESULTS: The age of the patients ranged from 9 to 82 years with mean age being 43 years. There was female preponderance, with male to female ratio being 1:7. On cytology out of 200 cases, 148 (74%) were benign, 25 (12.5%) were malignant, 16 (8%) were indeterminate, and 11 (5.5%) were nondiagnostic. Only 40 patients underwent surgery. On histopathology, 21 (52.5%) cases were benign and 19 (47.5%) were malignant. The statistical analysis of cytohistological correlation for both benign and malignant lesions revealed sensitivity, specificity, and diagnostic accuracy of 84%, 100% and 90%, respectively. CONCLUSION: FNAC is a minimally invasive, highly accurate and cost-effective procedure for the assessment of patients with thyroid lesions and has high -sensitivity and specificity. It acts as a good screening test and avoids unnecessary thyroidectomies.

Fibrolipoma of the eustachian ostium.

Nasopharyngeal fibrolipoma is extremely rare, with only four case reports in the literature. However, lipoma of the eustachian tube is the rarest with a single case report in the literature. A 50-year-old woman presented with obstruction of both nostrils for the past 1 year. CT scan showed a mass in the postnasal space extending from the right side of the nasopharynx to the oropharynx. The nasal endoscopy found a polypoidal mass arising from the posterior-inferior margin of the right eustachian tube ostium and extending to the oropharynx. Histopathological examination of the excised polyp found features suggestive of fibrolipoma. Fibrolipoma is characterised by prominent bundles of mature collagenous or myxocollagenous stroma intermixed with mature adipocytes. CT is useful in the diagnosis of lipoma but MRI is preferable. This is the second case of eustachian tube fibrolipoma to be reported in the literature that emphasises the role of nasal endoscopy and rarity of this tumour.

Necrotizing cervical fasciitis: Prognosis based on a new grading system.

We conducted a 10-year retrospective study to determine the prognosis of necrotizing cervical fasciitis (NCF). Our study population included 38 patients-32 males and 6 females, aged 10 months to 70 years (mean: 55 yr)-who had presented for management of NCF at our tertiary care hospital between Jan. 1, 2000, and Dec. 31, 2009. We classified each case into four categories based on the duration of disease prior to presentation, the severity of disease, and other factors that influence outcomes. We found that the most important factor in determining prognosis was the time interval between the onset of NCF and subsequent presentation for specialist or surgical intervention. Patients with a higher grade of NCF had longer hospital stays. Although aggressive surgical and medical intervention is the gold standard for the management of NCF, many of our patients presented with a relatively healthy appearing wound, which could mislead the evaluating clinician and delay prompt management. We believe that our new grading system will help obviate this problem and make clinicians more vigilant when faced with a new case of necrotizing fasciitis.

Hearing loss with phosphodiesterase-5 inhibitors: a prospective and objective analysis with tadalafil.

OBJECTIVES/HYPOTHESIS: To assess the effect of tadalafil on auditory functions. STUDY DESIGN: Prospective observational study in a tertiary care hospital. METHODS: Twenty-five tadalafil naïve patients with erectile dysfunction or pulmonary artery hypertension were subjected to pure-tone and brainstem-evoked response audiometry before drug therapy, and 3 and 30 days following drug therapy. Results were compared using analysis of variance for repeated measures with Bonferonni correction. RESULTS: Fifteen patients were taking tadalafil 10 mg for erectile dysfunction, and another 10 patients were on tadalafil 20 mg once daily for pulmonary artery hypertension. No statistically significant difference was found in hearing threshold at baseline and at follow-up (P > .05). However, three patients on tadalafil 20 mg showed a significant increase in hearing threshold at higher frequencies. There was no incidence of sudden sensorineural hearing loss in the study group. CONCLUSIONS: This is the first prospective and observational study that evaluated the effect of tadalafil on auditory functions with objective tests. Although there was no statistically significant result to confirm or refute the association between tadalafil and hearing impairment, increased threshold at higher frequencies after taking tadalafil supports the results from previous studies and hints at a possible relationship between the two. Similar large sample studies are warranted to know the exact association of phosphodiesterase-5 inhibitors on auditory functions.

Ectopic canine associated with a dentigerous cyst in the maxilla.

Ectopic eruption of a tooth is common in the dental arch, palate, and nose, but it is rare in the maxillary antrum. We present the case of a 35-year-old man with an ectopic canine and an associated dentigerous cyst in the maxillary sinus that masqueraded as an antrochoanal polyp.

Primary non-Hodgkin's lymphoma of the infratemporal fossa: a rare case report.

BACKGROUND: The head and neck are two of the most common sites of extranodal non-Hodgkin's lymphoma (NHL). However, primary tumors of the infratemporal fossa are infrequent, and NHL in this region is extremely rare. CASE PRESENTATION: We present a case of a 41-year-old female that presented with swelling in the right preauricular region that had persisted for the past two years. The patient was diagnosed as having a small lymphocytic NHL. She initially underwent chemo-radiation but reported relapse. The tumor was excised and again the patient underwent chemotherapy. The patient remained symptomatic and developed a second primary squamous cell carcinoma in the right retromolar trigone. DISCUSSION AND CONCLUSION: We discussed NHL with an emphasis on extranodal manifestations. Extranodal NHL that is limited to a single site can be managed by surgery and regular follow up. To the best of our knowledge, this is only the second case of primary NHL of the infratemporal fossa to be reported in the literature.

Giant tonsillolith causing odynophagia in a child: a rare case report.

Giant tonsillolith is a rare clinical entity. Commonly, it occurs between 20-77 years of age. We had a twelve years old female patient, who had odynophagia due to a giant tonsillolith. The stone was removed and tonsillectomy was performed. We reviewed the literature on this rare clinical entity and found that this is the fourth case of giant tonsillolith in a child and largest ever tonsillolith to be reported in English literature.

Himalayan black bear mauling: offense or defense?

The Asiatic Black Bear (Ursus thibetanus or Selenarctos thibetanus), also known as the Tibetan black bear, the Himalayan black bear, or the moon bear is a omnivorous mammal. This animal is declared threatened animal and rarely comes in human contact. Recent decrease in forest area has, however, increased the chances of bear-human interaction, hence causing injuries to humans. There is only one published report in English literature on Himalayan black bear mauling. We present 5 cases referred to our department over a period of 1 year.