PRESenting a Challenge: Posterior Reversible Encephalopathy Syndrome in Pediatric Patients With Guillain-Barré Syndrome: A Case Series and Review of Literature.

BACKGROUND: Guillain-Barré syndrome (GBS) is an autoimmune disorder characterized by demyelination of peripheral nerves. GBS-associated posterior reversible encephalopathy syndrome (PRES) is a rare and potentially life-threatening complication in the pediatric population. We aimed to report and analyze the clinical features, management, and outcomes of three cases of GBS-associated PRES in our setting in the light of the existing literature. METHODS: Medical records of 75 pediatric patients with GBS were reviewed for autonomic changes and GBS-associated PRES. Thirty-one developed dysautonomia while three were identified to have PRES. Clinical, radiological, laboratory, and treatment data were collected and analyzed. RESULTS: All three patients were male and presented with symptoms of acute flaccid paralysis and respiratory distress requiring mechanical ventilation. All three patients experienced various complications, including hypertension, seizures, and hyponatremia, and were subsequently diagnosed with PRES. Multimodal intensive care resulted in patient improvement and discharge in an ambulatory state after an average of 104 days of care. CONCLUSIONS: GBS-associated PRES is a rare and potentially life-threatening complication that can occur in pediatric patients with GBS. Our findings suggest that early recognition, prompt intervention, and multimodal intensive care can improve patient outcomes. Further studies are needed to determine optimal treatment strategies for GBS-associated PRES.

Mimics of Guillain-Barré Syndrome in Pediatric Patients Admitted to the Neuro-Intensive Care Unit of a Tertiary Care Hospital-A Case Series.

Guillain-Barré syndrome is the most common cause of acute flaccid paralysis in children, but several diseases mimic GBS. We aimed to identify and report the clinical pointers and battery of tests required to differentiate Guillain-Barré syndrome from its observed mimics in the pediatric population admitted to our neuro-critical care unit. We conducted a retrospective record analysis of all pediatric patients admitted over ten years from 2008-2018, whose initial presentation was compatible with a clinical diagnosis of GBS. Eighty-three patients were at first treated as GBS, of which seven (8.4%) were found to have an alternate diagnosis-three cases of paralytic rabies, one case each of acute disseminated encephalomyelitis, cervical myeloradiculopathy, neuromyelitis optica, and a case of community-acquired Staphylococcus aureus pneumonia associated sepsis. Neurophysiological and neuro-virological testing, central nervous system imaging, and sepsis screening helped to confirm the alternate diagnosis. Our case series provides knowledge of subtle clinical differences along with the mindful use of diagnostic testing to facilitate the accurate diagnosis of GBS mimics.

Clinicoradiological Features and Long-term Cognitive and Functional Outcome in Patients with Deep Cerebral Venous Thrombosis.

Background: Deep cerebral venous thrombosis (DCVT) can have long-term functional and cognitive sequelae. Although literature exists on cognitive impairment after arterial stroke, cognitive sequelae after cerebral venous thrombosis (CVT) are much less studied. Methods: Clinical records of 29 patients diagnosed with DCVT were reviewed. The Modified Telephonic Interview for Cognitive Status (TICS-M) was adapted and validated in the regional language (Kannada) and applied to 18 patients with DCVT, at a mean follow-up duration of 5.32 years. Screening for depression was done via telephonic Patient Health Questionnaire-9 (PHQ-9)-Kannada version, and functional status was screened by applying the modified Rankin Scale (mRS). Results: DCVT had a mortality rate of 10.34% due to acute complications. mRS scores of 0-1 were achieved at follow-up in all patients who survived. Receiver operating characteristic (ROC) analysis revealed a cutoff of ≤44.5 (maximum score of 49) for the diagnosis of cognitive impairment via TICS-M (Kannada version) in DCVT patients. Evidence of cognitive dysfunction was seen in eight patients (42.10%), and three patients (16.66%) had evidence of depression. Conclusions: Survivors of acute DCVT can potentially have long-term cognitive sequelae. Screening for cognitive dysfunction, depression, and functional status can be effectively done using telephonically applied scales that are adapted to the local language. Neuropsychological evaluation and early cognitive rehabilitation can be initiated for patients in whom deficits are identified on cognitive screening.

Profile and Outcome of Prospective Non-Septic Lateral Sinus Thrombosis Patients from a Stroke Unit of a Developing Country.

Background: Due to effective treatment of middle ear infections there is a change in etiologies causing lateral sinus thrombosis (LST) and outcome. There is a paucity of literature describing homogenous group of patients with nonseptic LST (NS-LST). Objective: To describe the clinical profile, risk factors, outcome of patients of NS-LST seen at a single center from South India. Methods and Materials: Prospective, observational study of 100 patients of NS-LST patients, diagnosed by magnetic resonance imaging (MRI) seen at the stroke unit. Results: During 2 years, 100 patients of NS-LST (isolated: combined: 27:73) (male: female: 44:56), mean age: 31.45 ± 11.13 years, were seen. Subacute presentation (74%) with headache, seizures, focal deficits, and features of raised intracranial pressure were presenting features. Hyperhomocysteinemia (61%), anemia (57%), postpartum state (41%), OCP use (37%), and low VitB12 (32%) were commonly seen risk factors. Imaging with MRI compared withcomputerized tomography (CT) had better diagnostic sensitivity (100% vs. 67%), detection of parenchymal (81% vs. 67%)/hemorrhagic (79% vs. 74%) lesions, and cortical vein thrombosis (31% vs. 15.46%). Treatment with anticoagulation and supportive therapy resulted in good outcome (mRS (0-2)) at 3 months in 81%.There were four deaths, all during admission (one - isolated, three - combined) and 11 patients underwent decompressive surgery. Patients with low GCS level of sensoriumat admission, hemiparesis, combined LST, cerebellar involvement, and decompressive craniectomy had a poor outcome. Conclusion: This single-center large cohort study of NS-LST patients brings out the clinical features, risk factors (peculiar to developing countries), and the superiority of MRI in the diagnosis. Majority of patients have good outcome, with low mortality with 10% requiring decompressive surgery.

Surgical Management of Vision Loss in Cerebral Venous Thrombosis: Case Series from a Tertiary Care Stroke Center.

Background and Purpose: Cerebral venous thrombosis (CVT) presenting as vision loss is uncommon. Raised intracranial tension in CVT is proposed as one of the mechanisms (13.2%). There are still unknown underlying mechanisms to explain vision loss in CVT. The safety and outcome of the surgery (optic nerve sheath fenestration [ONSF] or theco-peritoneal shunt [TPS]) to reduce intracranial hypertension and prevent vision loss has not been studied. Methods: A retrospective case record review of CVT patients with impending vision loss who underwent ONSF/TPS from 2007 to 2019 was performed from the stroke registry. All patients had formal neuro-ophthalmological evaluation and documentation of visual acuity, supplemented by visual field assessments by perimetry in a subset of patients. Safety and outcomes were assessed based on vision improvement and adverse effects after the surgery. Results: Among approximately 1400 patients with CVT admitted in the stroke ward over 12 years, surgery for rescuing vision was done in 18. Among these, the males were 6, and the females were 12. The mean age of presentation was 24 (range 18-52 years). All of them had headaches and progressive blurring of vision with papilledema. The number of patients who underwent TPS was 13, ONSF was 1, and both were 4. In the TPS group (26 eyes), vision improved in 15 eyes (57.7%), remained status-quo in 8 eyes (30.7%), and worsened in 3 eyes (11.5%). Four patients underwent both surgeries; three eyes improved, two remained status quo, and three worsened. One patient underwent ONSF, and his vision remained status quo (no perception of light). Three patients (17.6%) of the TPS group had minor complications (low-pressure headache, subdural hygroma), and five (29.4%) had major complications like subdural hemorrhage, abdominal wound infection, and meningitis. Conclusion and Implications: In patients with CVT, adequate vision monitoring is mandatory. Shunt surgeries (especially TPS) may help in stabilizing/improving vision in CVT patients with impending vision loss, despite adequate anti-edema measures (53.8% improved). Early diagnosis and precise decisions in referring for surgery are crucial.

Spontaneous Downbeat Nystagmus in Anti-GAD-Antibody-Associated Paraneoplastic Syndrome.

Spontaneous downbeat nystagmus and ocular flutter are rare clinical signs. Such findings are commonly related to cerebellar pathology, predominantly ischemia. In a significant percentage of patients, the cause may not be found. If these signs are associated with ataxia, cognitive decline, and seizure, anti-glutamic acid decarboxylase-associated neurological syndrome must be suspected. Background history of tumor has to be enquired. Treatment with immune modulation helps in partial recovery of such cases.

Inflammatory Myositis in a Child due to Anti-NXP2 Antibody, First Case Report from India.

We present a case of a 10-year-old boy with 1-month history of proximal more than the distal weakness of all four limbs with myalgias, contractures, and bulbar symptoms on a background history of exertional myalgias for 2 years. His power was grade two-three. Investigations showed elevated creatine phosphokinase (CPK). His auto-antibody profile showed anti- nuclear matrix protein 2 (NXP2) antibody positivity. Muscle magnetic resonance imaging (MRI) showed extensive T2 fat-saturated hyperintense signal changes in the glutei, thigh, and leg muscles suggestive of active myositis. He improved significantly with immunomodulation with steroids, intravenous immunoglobulins (Iv Ig), and mycophenolate mofetil (MMF). He was continued on monthly pulse steroids and MMF. He is on regular follow-up. This is a rare case of anti-NXP2 antibody-mediated inflammatory myositis and the first report from India.

A Rare Genetic Cause of Young Onset Rapidly Progressive Dementia- First Report from India.

We present a case of a 40-year-old man with rapid decline in cognition followed by Parkinsonism, apraxia and stimulus sensitive myoclonus within 9 months of onset. Magnetic Resonance imaging (MRI) showed periventricular confluent white matter changes with persistent diffusion restriction even after 9 months. Clinical exome sequencing showed colony stimulating factor 1 receptor (CSF1R) gene mutation. The phenotype, MRI and genotype are suggestive of adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP). This is the first case being reported from India. Differential diagnosis of young onset dementia is broad. Therefore, finding the exact etiology is challenging. Neuroimaging and genetic analysis greatly aid in the final diagnosis.

A Rare Case of Neurosyphilis Presenting As Normal Pressure Hydrocephalus Syndrome.

Neurosyphilis, a chronic infection of the nervous system by Treponema pallidum can present in all stages of syphilis. Recently, it is found that neurosyphilis presents with novel manifestations. Here, we report a young patient who had neurosyphilis presenting as Normal Pressure Hydrocephalus (NPH) with bilateral optic atrophy. The patient showed improvement with treatment for neurosyphilis. Hence, it is evident that in young patients presenting with NPH and associated features, secondary etiologies should be investigated.

A Bio-Psychosocial Framework for Chronic Daily Headaches: A Mixed Methods Study.

Chronic daily headaches (CDH) are primarily understood from a psychophysiological formulation. A broad biopsychosocial understanding, where there is equal importance given to biological, psychological and socio-cultural factors, is underexplored in headache. Socio-cultural factors, such as gender, socio-economic factors can perpetuate and worsen the condition. For an effective and sustainable intervention, these factors need to be considered. The current study aims to explore and develop a biopsychosocial framework for headache disorders. A convergent parallel mixed methods design was used and participants were recruited from a tertiary referral care hospital, Bengaluru, India. Headache Assessment Sheet, GAD 7, HDRS, PSS and B COPE were used in the quantitative phase. The data was analysed using r software. Qualitative phase of the study, in depth interview guide was used and data analysed thematically. Quantitative phase, 38 participants were recruited. The average age, was 38.02 (±10.17), majority of the participants being females 31 (81.58%). The mean duration of illness was 8.63 (±4.73) years. The anxiety scores positively correlated with pain intensity (r = 0.50 at P ≤ .001) and the median anxiety scores varied with photophobia (P ≤ .03). The anxiety scores correlated with PSS (r = 0.428 at P ≤ .007) and HDRS (r = 0.428 at P ≤ .007) scores. Gender variations in coping were seen, avoidant coping having higher median scores in women. (P ≤ .08). In qualitative phase of the study, six participants were recruited. Three main themes emerged from the qualitative phase of the study: headache - an illness, headache factors and the impact. Chronic daily headaches are influenced by biological, psychological, environmental and socio-cultural factors. A bio-psychosocial framework will help to understand and develop targeted interventions.

In search of the 'self': Holistic rehabilitation in restoring cognition and recovering the 'self' following traumatic brain injury: A case report.

BACKGROUND: Following mild-moderate traumatic brain injury (TBI), an individual experiences a range of emotional changes. It is often difficult for the patient to reconcile with their post-injury persona, and the memory of pre-injury personhood is particularly painful. Insight into one's cognitive deficits subsequent to injury can lead to an existential crisis and a sense of loss, including loss of self. OBJECTIVE: Restoration of cognitive functions and reconciliation with loss of pre-traumatic personhood employing a holistic method of neuropsychological rehabilitation in a patient suffering from TBI. METHODS: Ms. K.S, a 25-year-old female, presented with emotional disturbances following TBI. She reported both retrograde and anterograde amnesia. A multidimensional holistic rehabilitation was planned. Treatment addressed cognitive deficits through the basic functions approach. Cognitive behavioural methods for emotional regulation like diary writing helped reduce irritability and anger outbursts. Use of social media created new modes of memory activation and interactions. Compensatory strategies were used to recover lost skills, music-based attention training helped foster an individualised approach to the sense of one's body and self. RESULTS: As a result of these differing strategies, changes were reflected in neuro-psychological tests, depression score and the patient's self-evaluation. This helped generate a coherent self-narrative. CONCLUSION: Treatment challenges in such cases are increased due to patient's actual deficits caused by neuronal/biochemical changes. Innovative and multi-pronged rehabilitation strategies which involve everyday activities provided an answer to some of these problems. This method of rehabilitation may provide an optimistic context for future research.

Is Perls Prussian Blue Stain for Hemosiderin a Useful Adjunct in the Diagnosis of Vasculitic Neuropathies?

BACKGROUND: Perls Prussian blue stain (PPB) for hemosiderin, a marker of vascular injury is often employed as an adjunct in the diagnosis of vasculitic neuropathies. However, inflammation/vascular injury is also seen in leprosy, immune mediated, paraproteinemic, diabetic neuropathies, etc. The frequency of detection of hemosiderin in these neuropathies and its utility in diagnosis of vasculitis has not been explored. OBJECTIVE: We evaluated 208 peripheral nerve biopsies for hemosiderin deposits by PPB stain in vasculitis (78) and compared with inflammatory/immune neuropathies [leprous neuritis-32, chronic inflammatory demyelinating polyneuropathy (CIDP)-15, paraproteinemic neuropathies (POEMS)-12, diabetic neuropathy-37] and nonimmune neuropathies [Charcot-Marie-Tooth (CMT) disease-15, vitamin B12 deficiency-7, and ischemic neuropathy in aged-12)]. RESULTS: Hemosiderin deposits were most frequent in vasculitis (48.72%) [59.2% in systemic; 43.1% in nonsystemic vasculitides] and enhanced the sensitivity of diagnosis in "probable" vasculitis (34.48%) that lacked transmural inflammation. Hemosiderin was also detected in infectious/immune-mediated neuropathies (leprous neuritis-56%, POEMS-33.3%, diabetes-18.9%) but absent in CMT, B12 deficiency, and ischemic neuropathy. Hemosiderin deposits involved epineurium in vasculitis, compared to endoneurial/perineurial location in leprosy and perineurial in POEMS and diabetic neuropathy. The sensitivity of detection was high in vasculitic neuropathy (49.35%) compared to other inflammatory neuropathies (22.3%) (P < 0.05) with high specificity (77.69% [positive predictive value (PPV)-56.71%; negative predictive value (NPV)-71.6%]. The specificity increased to 89% if leprous neuropathy was excluded, with PPV-77.5% while NPV dropped to 68.5%. CONCLUSION: These findings suggest that PPB stain for detection of hemosiderin is a useful adjunct in diagnosis of vasculitic neuropathy with high specificity but low sensitivity.