Hirayama disease: neutral and flexion magnetic resonance imaging and utility of inter-segmental angle of flexion.

Purpose: Hirayama disease (HD) is a rare disease that was commonly mis-diagnosed in the past. The importance of neutral and flexion magnetic resonance imaging (MRI) in its accurate diagnosis has been emphasized along with utility of the inter-segmental angle of flexion. Aim of the study was to observe MRI findings of HD in neutral and flexion position and measure the inter-segmental angle of flexion. Material and methods: Cervical MR images of 17 patients of suspected HD were evaluated retrospectively for loss of attachment (LOA) of posterior dura, lower cervical cord atrophy, T2 hyperintensity, loss of cervical lordosis, enhancement of posterior epidural venous plexus, and inter-segmental angle of flexion on neutral and flexion MRIs. Results: Flexion MRI showed LOA of posterior dura (most commonly and maximum at C6 vertebral level) and intense enhancement in posterior epidural space in almost all patients. The mean inter-segmental angle of flexion at C5-C6 was 9.2°, and at C6-C7 it was 6°. Neutral MRI revealed LOA in 64.7%, lower cervical cord atrophy in all patients, T2 hyperintensity in the lower cervical cord in 35.2% of patients, and loss of cervical lordosis in 58.8% of patients. Conclusions: Flexion MRI is the gold standard for diagnosis of HD; however, certain imaging attributes, i.e. loss of attachment of posterior dura, asymmetrical lower cervical cord atrophy, T2 hyperintensity, and loss of cervical lordosis, can be seen on neutral MRI as well, which subsequently prompts the radiologist to include flexion MRI for confirmation. The inter-segmental angle of flexion is increased in patients with HD, which plays a role in planning timely surgical intervention.

Novel management of critically induced polyneuropathy in intensive care patients: A case report of two patients.

There has been tremendous growth in patients requiring critical care with severe infections. During a prolonged stay in the intensive care unit (ICU), patients develop critical illness polyneuropathy (CIP). The early identification of neurological involvement requires special attention during ICU care. We describe two cases who developed complete motor weakness after a prolonged stay in ICU. Patients were successfully managed with pyridostigmine and testosterone hormonal therapy initially and later with pyridostigmine only. The present case series highlights the need for early recognition, assessment, and novel management of CIP in ICU patients. However, the role of nutrition, physiotherapy, and supportive care is equally essential for the successful outcome in these patients.

Telestroke in resource-poor developing country model.

CONTEXT: Telemedicine is a major effort to tackle the uneven availability of facilities for thrombolysis in acute ischemic stroke. We present a telestroke model introduced in a small hilly state of Himachal Pradesh in India. AIMS: To provide acute ischemic stroke treatment with tissue plasminogen activator in all district hospitals of Himachal Pradesh with computerized axial tomographic scan facility through Telemedicine. SETTINGS AND DESIGN: Smartphone-based hub and spoke telestroke model was used with two tertiary care hospitals (with neurologists) as hub and 17 district hospitals (without onsite neurologists) as spokes. SUBJECT AND METHODS: The telestroke project was launched in the state of Himachal Pradesh in April 2014. Medical officers in district hospitals (Medicine graduates and Internal Medicine postgraduates) were trained in the treatment of stroke through workshops. Tissue plasminogen activator was made available at all these centers, free of cost through hospital pharmacies. Four neurologists at two tertiary care centers were made available for consultation on phone. RESULTS: Between June 2014 and May 2015, a total of 26 patients received thrombolysis under the telestroke project at nine district hospitals without onsite presence of a neurologist. Eight patients were females and 18 males. The age of patients ranged from 26 to 80 years. Only 2 patients developed an intracranial bleed following thrombolysis, and both were nonfatal. CONCLUSIONS: Smartphone-based telestroke services may be a much cheaper alternative to video-conferencing-based telestroke services and are more portable with less technical glitches. To the best of our knowledge, this is the first telestroke model being reported from India. It seems to be the way forward in providing timely treatment in acute ischemic stroke in underserved and resource poor settings.

Increased electrode impedance as an indicator for early detection of deep brain stimulation (DBS) hardware Infection: Clinical experience and in vitro study.

BACKGROUND: When deep brain stimulation (DBS) infections are identified, they are often too advanced to treat without complete hardware removal. New objective markers to promptly identify DBS infections are needed. We present a patient with GPi (globus pallidus interna) DBS for dystonia, where the electrode impedance unexpectedly increased 3-months post-operatively, followed by serologic and hematologic markers of inflammation at 6-months, prompting explantation surgery. We recreated these conditions in a laboratory environment to analyze the pattern of changing of electrical impedance across the contacts of a DBS lead following Staphylococcus biofilm formation. METHODS: A stainless-steel culture chamber containing 1 % brain heart infusion agar was used. A DBS electrode was dipped in peptone water containing a strain of S. aureus and subsequently introduced into the chamber. The apparatus was incubated at 37 °C for 6 days. Impedance was measured at 24hr intervals. A control experiment without S. Aureus inoculation was used to determine changes in impedance over a period of 6-days. RESULTS: The mean monopolar impedance on day-1 was 751.8 ± 23.8 Ω and on day-3 was 1004.8 ± 68.7 Ω, a 33.7 % rise (p = 0.007). A faint biofilm formation could be seen around the DBS lead by day-2 and florid growth by day-3. After addition of the linezolid solution, a 15.9 % decrease in monopolar impedance was observed from day 3-6 (p = 0.003). CONCLUSION: This study gives insight into impedance trends following a hardware infection in DBS. Increased impedance outside expected norms may be valuable for early prediction of infection. Furthermore, timely management using antibiotics might reduce the frequency of infection-related explant surgeries.

Idiopathic intracranial hypertension and visual function in North Indian population.

BACKGROUND: Idiopathic Intracranial Hypertension (IIH) is a neurological disorder with varied presentation, visual morbidity being the most important one. Literature is sparse as regards the effects of IIH on the visual system in Asians. OBJECTIVE: Assessment of visual morbidity and change with treatment in IIH patients in North India through a prospective interventional study. MATERIALS AND METHODS: Sixty eyes of newly diagnosed IIH patients were evaluated for functional and structural tests of visual system. Lumbar puncture was performed to establish IIH diagnosis and received oral Carbonic anhydrase inhibitor thereafter. Change in visual function was studied from baseline to 6-month follow-up visit. RESULTS: Mean age of participants at presentation was 33.27 ± 10.68 years and majority were females (73.3%). Seventy percent eyes (n = 42) had visual acuity 20/20 at presentation. Average Pelli Robson contrast sensitivity (CS) improved from 1.86 ± 0.34 to 2.11 ± 0.24 (Friedman Test: X2 = 59.2, p ⩽ 0.001) while number of eyes with detectable visual field deficits reduced from 70% to 43.7%. Retinal nerve fibre layer (RNFL) thickness reduced from 176.27 to 114.97 µ (Friedman Test: X2 = 69.3, p ⩽ 0.001). CONCLUSIONS: The socio-demographic profile in our study showed higher male incidence than noted in previous studies. Visual function deficits were present in patients, even in those with 20/20 visual acuity. Significant improvement was seen after lumbar puncture and initiation of medical treatment. CS and RNFL thickness showed significant improvement even after 1 month of treatment and can be used for monitoring IIH patients.

Dihydrotestosterone in Amyotrophic lateral sclerosis-The missing link?

OBJECTIVE: Testosterone has been postulated to be involved in ALS causation. MATERIALS AND METHODS: CSF levels of free testosterone and dihydrotestosterone were measured in 13 ALS patients [7 males, 6 females] and 22 controls [12 males, 10 females]. RESULTS: CSF free testosterone levels did not show any significant differences but CSF dihydrotestosterone levels were significantly decreased in all male and female ALS patients. CONCLUSIONS: DHT is probably integral to survival of motor neurons. In patients predisposed to develop ALS, there is possibly a sort of "testosterone resistance" at level of blood-brain barrier [BBB] existing right from birth and is likely the result of dysfunctional transport protein involved in testosterone transfer across the BBB. In these patients, lesser amount of testosterone is able to breach the BBB and enter the central neural axis. Lesser amount of testosterone is available to 5 α reductase in the anterior pituitary to be converted to DHT and lesser amount of DHT is generated. There is inadequate negative feedback suppression of LH at the level of anterior pituitary by DHT. As a result of higher LH levels, testosterone levels rise in the peripheral testosterone fraction [the fraction outside the BBB] and this explains the various physical attributes of ALS patients like lower Ratio of the index and ring finger lengths (2D:4D ratio), increased incidence of early onset alopecia etc. This deficiency of DHT leads to motor neuron death causing ALS.

Syringomyelia as a delayed complication of lumbar-sacral adhesive arachnoiditis in Pott's disease.

Syringomyelia is the development of a fluid-filled cyst (syrinx) within the spinal cord and is an extremely rare chronic manifestation of tuberculosis. The syrinx so formed may expand over time, causing compression or destruction of spinal tracts and surrounding nerve roots. Development of syringomyelia in a patient of Pott's disease is particularly infrequent. We report this rare case of a 31-year-old man with syringomyelia as a prolonged complication of Pott's disease.

The role of pyridostigmine in recovery from motor paralysis in a snakebite patient with an allergic reaction to anti-snake venom.

A 30-year-old woman presented as an emergency with a history of snakebite 5 h previously with signs of bulbar palsy, ptosis, respiratory distress and weakness of all four limbs. Mechanical ventilation, anti-snake venom (ASV) and supportive management were immediately instituted. With the third dose of ASV, an early anaphylactic reaction ensued. Subsequent management with corticosteroids and antihistamines over the next few days allowed consciousness to return but muscle power did not improve beyond 2/5. A trial of intravenous neostigmine with glycopyrrolate, however, improved motor power in all four limbs to 3/5. Oral pyridostigmine at 60 mg every 8 h allowed subsequent full motor recovery in all four extremities. We suggest consideration of pyridostigmine to promote motor recovery after an allergic reaction to ASV.

Immune reconstitution inflammatory syndrome in non-HIV patients with tuberculosis. A case series.

Tuberculosis associated Immune reconstitution inflammatory syndrome (IRIS) in a HIV negative patient can present with a multitude of clinic-radiological presentations that are often confused with drug resistance/treatment failure. Being a diagnosis of exclusion, this clinical entity is often prone to under-diagnosis. We present a series of 5 patients who presented with varied but uncommon IRIS manifestations. High index of suspicion coupled with clinical reasoning and judicious use of phenotypic and genotypic culture methods helped in their timely detection and successful treatment.

Changing Clinicoradiologic Spectrum of Intracranial Neurotuberculosis in Children: A Cross-sectional Study.

BACKGROUND: Neurotuberculosis is among the most severe type of tuberculosis with high mortality and morbidity in all age groups. Various sociodemographic and disease-/treatment-related factors have emerged over the years that can affect clinical and radiologic features of neurotuberculosis. OBJECTIVE: To investigate various clinical and neuroradiologic presentations of neurotuberculosis. METHODOLOGY: This cross-sectional study was done in a tertiary care center of northern India. The patients between the ages of 3 months and 18 years with newly diagnosed neurotuberculosis were enrolled after taking informed consent. RESULTS: A total of 78 patients (37% males) were enrolled. Fifty-six patients (72%) had tubercular meningitis (TBM) and 22 (28%) isolated tuberculomas. Very high percentage of patients in both the groups was BCG vaccinated. In the tubercular meningitis group, fever (68%), headache (59%), and vomiting (54%) were the most common complaints whereas in the tuberculoma group, seizures (95.5%) were the main complaint and systemic symptoms were rare. In tubercular meningitis patients, cerebrospinal fluid-based studies showed cartridge-based nucleic acid amplification test (Xpert MTB/RIF) positivity for Mycobacterium tuberculosis in 17.6% cases, whereas on gastric aspirate and sputum examination, acid-fast bacilli were seen in 30.7% and cartridge-based nucleic acid amplification test was positive in 19% patients. On neuroimaging, hydrocephalus was seen in 44.6% of tubercular meningitis patients, infarcts in 32%, and basal exudates in 12% patients only; coexistent tuberculomas were seen in 53%. CONCLUSION: Compared with the available literature, the present study showed a smaller percentage of children <5 years of age, stage III tubercular meningitis cases, and typical neuroradiologic findings like hydrocephalus and basal exudates and more tuberculomas associated with tubercular meningitis.

Neuropsychiatric manifestations of methyl iodide toxicity.

Methyl iodide is an industrial chemical used in methylation for pharmaceutical intermediates. Its toxicity is a rare industrial hazard. Its toxicity is reported with both acute sudden exposure and gradual exposure. The authors report a case of methyl iodide toxicity occurring in a 47 year-old male with sudden exposure to this chemical. He presented with neuropsychiatric symptoms primarily slurring of speech, ataxia followed by delusion and hallucinations which resolved within 2 weeks with parenteral thiamine and an antipsychotic. Symptoms reappeared after a re-exposure, this time with more prominent psychiatric symptoms which were late to resolve (4 weeks) with similar treatment. Neuroimaging revealed hyperintensities in posterior regions of the brain which resolved after 4 weeks. The patient had prominent neurocognitive deficits which were persistent. The case highlights the rare chemical toxicity with neuropsychiatric manifestations with persistent neurocognitive symptoms.

Familial Creutzfeldt-Jakob Disease: The First Reported Kindred from South-East Asia.

Creutzfeldt-Jakob disease (CJD) belongs to a group of prion disease that is caused by abnormally folded proteins and is clinically characterized by rapidly progressive cognitive decline, gait abnormalities, and myoclonus. Familial CJD is very rare and is described only in few families around the world. We report a case with rapidly progressive cognitive decline, ataxia, and myoclonus, with a history of several members of his family developing similar symptoms and succumbing to it. Clinical presentation and neuroimaging were suggestive of CJD. On genetic analysis, our index case and two of his family members (younger brother and younger son) were found to have D178N mutation in PRNP gene. The polymorphism of the 129th amino acid was V/V. We report the first kindred familial CJD from South-East Asia with genetically proven D178N-129V haplotype.