"Neuroimaging in ethambutol induced optic neuropathy: MRI in time can save the vision".

Ethambutol is an integral part of Antitubercular therapy (ATT) and is often associated with optic neuropathy, However, neuroimaging of ethambutol induced optic neuropathy has been sparsely reported in the literature. We describe the case of a 45-year male patient, diagnosed as Tuberculous spondylodiscitis and was on ATT. Four months after ATT initiation, he presented with visual blurring in both the eyes with bitemporal hemianopia and central scotomas. Visual evoked potential (VEP) revealed prolonged latencies in N75 and P100 waveforms bilaterally. Magnetic Resonance Imaging (MRI) showed optic chiasma and bilateral optic tract hyperintensities on 3D Fluid Attenuated Inversion Recovery (FLAIR) and 3D Double Inversion Recovery (DIR) sequences. Ethambutol was discontinued immediately. On follow-up after 8 weeks, visual acuity reversed back to normal in both eyes.

Sleep profile and Polysomnography in patients with drug-resistant temporal lobe epilepsy (TLE) due to hippocampal sclerosis (HS) and the effect of epilepsy surgery on sleep-a prospective cohort study.

OBJECTIVES: We analyzed changes in sleep profile and architecture of patients with drug-resistant TLE-HS using three validated sleep questionnaires- Epworth Sleepiness Scale (ESS), Pittsburgh Sleep Quality Index (PSQI), NIMHANS Comprehensive Sleep Disorders, and polysomnography (PSG). We studied the effect of epilepsy surgery in a subset of patients. METHODS: In this prospective observational cohort study, sleep profile of 40 patients with drug-resistant TLE-HS was compared to 40 healthy matched controls. Sleep architecture of 22 patients was studied by overnight PSG and compared to 22 matched controls. Sleep profile was reassessed in 20 patients after a minimum period of three months after epilepsy surgery. RESULTS: The mean PSQI was higher among patients compared to controls(P=0.0004) while mean ESS showed no difference. NCSDQ showed fewer patients feeling refreshed after a night's sleep compared to controls (p=0.006). PSG revealed a higher time in bed (p=0.0001), longer total sleep time (p=0.006) and more time spent in NREM stage 1 (p=0.001) and stage 2 (p=0.005) while spending less time in stage 3 (p=0.039) among TLE patients. Sleep efficiency was worse in patients on ≥3 ASMs compared to those on 2 ASMs (p-0.044). There was no change in mean ESS (p=0.48) or PSQI (p=0.105) after surgery. CONCLUSIONS: Patients with drug-resistant TLE-HS have an altered sleep profile and architecture. Patients on ≥3 ASMs have a lower sleep efficiency. Reassessment at short intervals after epilepsy surgery did not reveal significant changes in sleep profile.

Non-Wilsonian hepatolenticular degeneration: Clinical and MRI observations in four families from south India.

Non-Wilsonian hepatolenticular degeneration (NWHD) is a heterogeneous neurological disorder occurring secondary to chronic acquired liver disease. Genetically determined familial NWHD is rare, poorly understood, and often mistaken for Wilson's disease (WD). We analysed clinical and MRI profiles of NWHD patients who did not have obvious cause for acquired liver disease, such as alcohol intake or hepatitis. Six patients from four families (four males, two females, mean age: 17.0±standard deviation 7.9years), presenting with chronic extrapyramidal disorder resembling WD and imaging (abdominal ultrasound/MRI) evidence of cirrhosis were studied. They lacked Kayser-Fleischer rings or biochemical and/or genetic evidence of WD. Clinical features included dystonia (n=6), parkinsonism (n=3), tremor (n=1), cerebellar ataxia (n=3), orofacial dyskinesia (n=1), behavioural abnormalities (n=3), and cognitive decline (n=1). Brain MRI revealed T1-weighted hyperintensity in the pallidum (n=6), crus cerebri (n=4), putamen (n=1), caudate (n=1), thalamus (n=1), and red nucleus (n=1) with T2-weighted shortening in some of these regions. Additional findings included giant cisterna magna (n=1), face of giant panda sign (n=1) and thin corpus callosum (n=1). Areas of "blooming" on susceptibility weighted images were noted in two patients in the caudate (n=2) and putamen (n=1). The finding of T1 shortening is distinct from that of WD where the majority of lesions are T1-hypointense and T2-hyperintense. Extrapallidal T1-hyperintensity is also an exceptional observation in NWHD. The MRI appearance of intense T1 shortening coupled with the lack of increased susceptibility changes suggests that the most likely mineral deposited is manganese. The association of this neurological disorder and cirrhosis of the liver in the absence of an acquired liver disease is a distinct disease entity. This syndrome may represent a disorder of manganese metabolism resulting in its toxic deposition.