Valproate-induced hyperammonemic encephalopathy in neurosurgical patients: Our experience and systematic literature review.

Sodium valproate is used for the management of seizures, status epilepticus, chronic pain syndrome, bipolar, and other affective disorders. Even with an acceptable safety profile, severe idiosyncratic reactions can occur with valproate use. A rare, serious, and life-threatening side effect of valproate is valproate-induced hyperammonemic encephalopathy (VHE). We intend to analyze the clinical presentation, diagnosis, treatment options, and outcome of VHE in neurosurgical patients and review the pertinent literature on this rare sequelae. We retrospectively reviewed patients who developed VHE following valproate use, either for the treatment of epilepsy or for seizure prophylaxis in our centre. We analyzed the demographic details, clinical presentation, diagnosis, management, and outcomes. A total of four patients with a mean age of 26.3 ± 5.1 (range 19 - 32 years). Valproate was prescribed for primary seizure prophylaxis in 2 patients (50%). The commonest etiology for valproate prescription was for brain tumors (3, 75%) followed by drug-refractory epilepsy (DRE) (1, 25%). None of the patients were documented to have urea cycle disorder. The mean daily prescribed dosage of valproate was 1250 ± 559 mg and the mean duration of administration was 13 ± 13.3 months (range 4 months - 36 months). The mean serum NH3 level was 136,5 ± 44.2 µmol/L (range 107 - 212.8) and all patients (4, 100%) had hyperammonemia with a mortality rate of 50% (2 patients). The hyperammonemia was treated by stopping the valproate use (4, 100%) and dialysis (2, 50%). Normalization of ammonia levels led to clinical improvement in 50% (2 patients). Neurological deterioration in the postoperative period is a diagnostic challenge. VHE is a rare and life-threatening sequelae of Valproate-associated Hyperammonemia (VAH) in neurosurgical patients. A high index of suspicion is required due to its ambiguous presentation. Early diagnosis and prompt treatment can change the course of this life-threatening sequelae.

Trans-sellar-trans-sphenoidal encephalocele presenting as nasal obstruction in an infant: a case report and systematic review of existing reports.

Trans-sellar trans-sphenoidal encephalocele is a rare congenital anomaly, with only around 20 cases having been documented in literature around the world. Surgical repair of these defects in the pediatric population commonly uses either the transcranial or the transpalatal approach, with the choice of approach being individualized based on the clinical features, age, and associated defects present in the patient. Here, we document a case of a 4-month-old child who presented to us with nasal obstruction, who was diagnosed with this rare entity and successfully underwent a transcranial repair for the same. We also provide a systematic review of all existing case reports that have described this rare condition in the pediatric population, as weel as the different surgical approaches used in each case.

Comparison of Neurological Manifestations in the Two Waves of COVID-19 Infection: A Cross-Sectional Study.

Introduction: Coronavirus Disease-19 (COVID-19) is an ongoing pandemic caused by highly contagious virus severe acute respiratory syndrome coronavirus-2 (SARS-COV-2) that has infected millions of people across the world. Most of the countries have seen two wave patterns of the pandemic. The second wave is potentially more challenging due to high influx of cases, differing properties of the emerging mutants, and other dynamics of the evolving pandemic. Neurological manifestations are common among COVID-19 positive patients. In this context, the present study attempts to compare the neurological manifestation in the first and second waves of COVID-19. Methodology: A single-center retrospective observational study was undertaken to compare neurological manifestations in the first and second waves of COVID-19. A sample of 1500 patients in the second wave admitted with COVID-19 were included in this study and the findings were compared with 1700 patients in the first wave (data derived from a former study in the same center). A detailed questionnaire addressing co-morbidities, admission details, and clinical features was employed to collect data from the hospital records. Results: Out of 1500 COVID-19 patients in the second wave of COVID-19, 355 (23.7%) of them had one or more neurological manifestations during their in-patient stay. The most common neurological symptom in the 2nd wave of COVID-19 was headache reported in 216 (14.4%) of patients followed by fatigue in 130 (8.7%), myalgia in 120 (8.0%), smell and taste disorders (STD) in 90 (6.0%), altered sensorium in 40 (2.7%), dizziness in 24 (1.6%), seizures in 34 (2.3%), encephalopathy in 26 (1.7%), strokes in 13 (0.9%), etc., Compared to the first wave of COVID-19, dizziness (P < 0.001), myalgia (P = 0.001), headache (P < 0.001) and meningoencephalitis (P = 0.01) were more common while cerebrovascular syndromes (P = 0.001) were less common in the second wave. The mortality in the 2nd wave neurological subgroup was higher [66 (18.6%)] than 1st wave neurological subgroup [23 (10%)]. Conclusion: Meningoencephalitis, headache, and seizures were found to be more common in second wave as compared to first wave. The severity and mortality rate were higher in the second wave.

Delayed bilateral thalamic bleeding post-ventriculoperitoneal shunt.

Ventriculoperitoneal shunt is one of the most common procedure done by neurosurgeon worldwide. We present a rare case of delayed intracerebral bleed post VP shunt and discuss the possible causes of intracerebral hemorrhage post ventriculoperitoneal shunt and bilateral thalamic bleed. The presumed cause in our case was shunt induced disseminated coagulation profile.

Multiple intracranial fungal abscesses in an immunocompetent infant treated surgically.

Intracerebral abscess are rare in neonates and infants. We present a rare case of 4-month-old immunocompetent infant with multiple intracranial fungal abscess. Right frontoparietal craniotomy was done with excision of multicystic mass containing 200cc of dirty colored fluid. Biopsy and culture of abscess came as Aspergillus fumigates. Patient received parental amphotericin B. An immunocompetent infant with intracranial aspergillosis is rare. We advocate combination of surgical excision with parental amphotericin B as best way to achieve good result.

Lateral extradural, supratentorial neurenteric cyst.

Neurenteric cysts are rare congenital, benign, endodermal lesions of the central nervous system that occur mainly in the spinal canal. Intracranial neurenteric cysts are rare; the posterior fossa being the most common site. A laterally based supratentorial neurenteric cyst is exceptionally uncommon. The rarity of supratentorial neurenteric cysts and their variable imaging features preclude preoperative diagnosis. A search of the literature revealed no other extradural supratentorial neurenteric cyst. We present a patient with an extradural giant neurenteric cyst that occupied both the supratentorial and infratentorial compartments, further highlighting the heterogeneity of this rare intracranial lesion and emphasizing the need to consider this in the differential diagnosis of cystic intracranial brain lesions.