Scrub typhus with opsoclonus-myoclonus-ataxia-seizure as primary presentations.

Scrub typhus is a simple acute febrile illness with rash or an eschar, with up to one-fifth of the patients complicated with the nervous system. Hence, certain cases present to physicians with rather a different systemic manifestation and incidentally have been diagnosed with scrub typhus. We present two such cases of scrub typhus with neurological manifestations. The first case was of a 14-year-old boy with no previous history of any comorbidities who presented with bilateral opsoclonus with multifocal spontaneous myoclonus with cerebellar ataxia with a preceding history of fever and acute gastroenteritis. The second case of a 30-year-old gentleman with no previous history of any comorbidities presented to us with generalized tonic-clonic seizures and spontaneous multifocal myoclonus with a preceding history of fever. Both cases had no motor, sensory, cerebellar, or autonomic involvement. The pathophysiology of central nervous system (CNS) infections in scrub typhus is attributed to three major mechanisms of vasculitis, direct invasion, and immune-mediated. CNS involvement in scrub typhus is a significant marker for risk of mortality or morbidity. The most common CNS manifestations in scrub include meningitis, encephalitis, and seizures. Opsoclonus, myoclonus, and parkinsonism are comparatively rare manifestations.Scrub typhus infection must be considered in the differential diagnosis of clinical neurological features with even a remote history of acute febrile illnesses in endemic regions like ours, despite the absence of any eschar, rashes, and unremarkable neuroimaging.

Pulsatile giant sacrococcygeal teratoma appearing like 2nd head.

Sacrococcygeal teratoma is a congenital tumor of early infancy. Eighty percent are present in the first month of life. It is considered to be an extragonadal germ cell tumor that can be diagnosed in utero. It is considered to be a rare tumor in infancy and childhood. Incidences in girls are more common as compared to boys. Association with other congenital abnormalities of the gastrointestinal system, cardiovascular system, and urological manifestations may be present. Association with spinal dysraphism may be present. A complete systemic evaluation needs to be done before any surgical intervention. We hereby present you a case of a 5-day-old male child, born from a non-consanguineous marriage, who presented to us with a large swelling at the coccygeal region. The mass had a variegated consistency with an ear-like appendage over it with associated hair, bones, and necrotic tissue in it. The patient was evaluated with imaging (MRI) which revealed a heterogeneously enhancing mass at the sacrococcygeal region with mild extension into the pre-sacral space suggesting sacrococcygeal teratoma. After a complete evaluation for congenital abnormal abnormalities of other sites, gross total excision of the mass was performed. The post-operative condition of the baby was uneventful. A biopsy of the mass revealed a mature cystic teratoma. The baby is under follow-up at present.

Role of prophylactic surgery in spinal tuberculosis: A retrospective study from Eastern India.

Background: Tuberculosis (TB) is broadly classified into pulmonary and extrapulmonary TB. Skeletal TB is considered to be a form of extrapulmonary TB whose incidence is around 1% of all forms of TB. The incidence of spinal TB is more than 50% of the entire skeletal TB. Methods: A total of 92 consecutive patients, treated over a period of 2 years (January 2021-January 2023), are retrospectively analyzed. Results: In our study, out of 92 patients, the pain had subsided in 90% of cases, and 12 patients showed improvement in motor power postoperatively. In our study, the patients have benefited due to the surgical intervention in decrease of pain and improvement in motor deficits in patients. The 29 patients who were operated on prophylaxis with pain as the only symptom benefitted from the resolution of the symptom of pain and did not develop any morbidity in their long-term follow-up. The sensory and bladder/bowel symptoms did not improve after surgery. Conclusions: In the author's view, prophylactic surgery for spinal TB is safe and effective with confirmation of the disease. However, a well-designed randomized controlled trial, to definitely and objectively prove the usefulness of prophylactic surgery, is needed.

Spinal fungal abscess mimicking as potts spine.

Spinal epidural abscess (SEA) is a rarest form of spinal infections and is characterized by the presence of pus in the space between the dura mater, and the osseoligamentous confines of the vertebral canal. This can cause spinal injury due to direct compression or local ischemia. The major etiology of SEA is bacterial and tuberculous in endemic regions. The incidence of fungal spinal abscess is relatively low and <5% of SEA is attributable to fungi. We, here, report a case of 77-year-old known patient of chronic renal disease on hemodialysis that presented with low-back pain for 15 days and was subsequently diagnosed with SEA by magnetic resonance imaging, and causative organism was identified as Candida by culture. The abscess was surgically drained after laminectomy. The patient improved with surgery and antifungal treatment.

Cerebral Myiasis Secondary to Burr Hole Evacuation: A Rare Illustrative Case Report.

Myiasis (maggot infestation) is a condition in which fly maggots feed off and develop in the tissues of living organisms. Most common in tropical and subtropical regions, human myiasis, is prevalent among individuals in close association of domestic animals and those inhabiting the unhygienic conditions. We, hereby, describe a rare case of cerebral myiasis (17th in the world, 3rd in India) that presented to our institution in Eastern India secondary in the operated site of craniotomy and burr hole few years back. Cerebral myiases are exceedingly rare conditions, especially in high-income countries with only 17 previously published cases with the reported mortality as high as 6 out of 7 cases dying of the disease. We additionally also present a compiled review of previous case literatures to highlight the comparative clinical, epidemiological features and outcome of such cases. Although rare, brain myiasis should be a differential diagnosis of surgical wound dehiscence in developing countries where conditions do exist in this country that permit myiasis. This differential diagnosis should be remembered, particularly when the classic signs of inflammation are not present.

Does follow-up D-dimer level help in predicting oxygenation status, ventilatory support requirement, lung fibrosis, and thromboembolic events in coronavirus disease 2019 pneumonia? A prospective observational study in a tertiary care setting in India.

Introduction: Coronavirus disease 2019 (COVID-19) pneumonia is a heterogeneous disease with variable effects on lung parenchyma, airways, and vasculature, leading to long-term effects on lung functions. Materials and Methods: This multicentric, prospective, observational, and interventional study included 1000 COVID-19 cases confirmed with reverse transcription-polymerase chain reaction. All cases were assessed with high-resolution computed tomography thorax, oxygen saturation, inflammatory marker as D-dimer at the entry point, and follow-up. Age, gender, comorbidity, use of bilevel positive airway pressure/noninvasive ventilation (BiPAP/NIV), and outcome as with or without lung fibrosis as per CT severity were key observations. In selected cases, we have performed lower limb venous Doppler and computed tomography (CT) pulmonary angiography to rule out deep-vein thrombosis (DVT) or pulmonary thromboembolism (PTE) respectively. Statistical analysis is performed by using Chi-square test. Observations and Analysis: Age (<50 and >50 years) and gender (male vs. female) has a significant association with D-dimer level (P < 0.00001 and P < 0.010, respectively). CT severity score at the entry point with the D-dimer level has a significant correlation (P < 0.00001). The D-dimer level has a significant association with the duration of illness before hospitalization (P < 0.00001). Comorbidities have a significant association with D-dimer levels (P < 0.00001). D-dimer level has a significant association with oxygen saturation (P < 0.00001). BIPAP/NIV requirement has a significant association with the D-dimer level (P < 0.00001). Timing of BIPAP/NIV requirement during hospitalization has a significant association with D-dimer level (P < 0.00001). Follow-up D-dimer titer during hospitalization as compared to normal and abnormal to entry point level has a significant association with post-COVID lung fibrosis, DVT, and PTE (P < 0.00001). Conclusions: D-dimer has documented a very crucial role in COVID-19 pneumonia in predicting the severity of illness and assessing response to treatment during hospitalization, and follow-up titers have a significant role in step-up or step-down interventions in a critical care setting.

Résumé Introducción: La neumonía por enfermedad por coronavirus 2019 (COVID 19) es una enfermedad heterogénea con efectos variables sobre el parénquima pulmonar, las vías respiratorias y la vasculatura, lo que lleva a efectos a largo plazo sobre las funciones pulmonares. Materiales y métodos: este estudio multicéntrico, prospectivo, observacional e intervencionista incluyó 1000 casos de COVID 19 confirmados con reacción en cadena de la polimerasa con transcriptasa inversa. Todos los casos fueron evaluados con tomografía computarizada de tórax de alta resolución, saturación de oxígeno, marcador inflamatorio como dímero D en el punto de entrada y seguimiento. La edad, el sexo, la comorbilidad, el uso de presión positiva en las vías respiratorias de dos niveles/ventilación no invasiva (BiPAP/NIV) y el resultado con o sin fibrosis pulmonar según la gravedad de la TC fueron observaciones clave. En casos seleccionados, hemos realizado Doppler venoso de miembros inferiores y angiografía pulmonar por tomografía computarizada (TC) para descartar trombosis venosa profunda (TVP) o tromboembolismo pulmonar (TEP) respectivamente. El análisis estadístico se realiza utilizando la prueba de Chi cuadrado. Observaciones y análisis: la edad (50 años) y el sexo (hombre vs. mujer) tienen una asociación significativa con el nivel de dímero D (P < 0,00001 y P < 0,010, respectivamente). La puntuación de gravedad de la TC en el punto de entrada con el nivel de dímero D tiene una correlación significativa (P < 0,00001). El nivel de dímero D tiene una asociación significativa con la duración de la enfermedad antes de la hospitalización (P < 0,00001). Las comorbilidades tienen una asociación significativa con los niveles de dímero D (P < 0,00001). El nivel de dímero D tiene una asociación significativa con la saturación de oxígeno (P < 0,00001). El requerimiento de BIPAP/NIV tiene una asociación significativa con el nivel de dímero D (P < 0.00001). El momento del requerimiento de BIPAP/NIV durante la hospitalización tiene una asociación significativa con el nivel de dímero D (P < 0.00001). El título de dímero D de seguimiento durante la hospitalización en comparación con el nivel normal y anormal al punto de entrada tiene una asociación significativa con la fibrosis pulmonar, la TVP y la TEP posteriores a la COVID (P < 0,00001). Conclusiones: el dímero D ha documentado un papel muy importante en la neumonía por COVID 19 para predecir la gravedad de la enfermedad y evaluar la respuesta al tratamiento durante la hospitalización, y los títulos de seguimiento tienen un papel importante en las intervenciones de aumento o reducción en un entorno de cuidados críticos. Mots-clés: Palabras clave: enfermedad por coronavirus 2019, neumonía, dímero D, trombosis venosa profunda, marcador inflamatorio, fibrosis pulmonar, embolia pulmonar.

Pedunculated Chiari 3 malformation with proatlas defect.

Chiari III malformation is considered to be a rare congenital abnormality in human with very high mortality rates. Seventy percent of Chiari III is found to be associated with C1 arch defect as reported by Cakirer (Clin Imaging 27:1-4, 2003). The herniation of posterior fossa elements or dysplastic neural tissue is a must to stamp it as Chiari 3 malformation. The malformation is a result of the abnormal development of craniovertebral junction (CVJ). The CVJ developed from the occipital somites and the first spinal sclerotome. The major role in the development of the CVJ is played by the fourth occipital somite, which is otherwise known as "proatlas." The Chiari III anomalies are due to a result of proatlas defect, which results from failures of segmentation, failures of fusion of different components of each bone, or hypoplasia and ankylosis. We are presenting a case of a 1-year 4-month-old female child who presented with pedunculated swelling at the suboccipital region. The swelling was cystic and with pulsation. On evaluation, we found Chiari III anomaly with C1 posterior arch deficiency (proatlas defect). He was surgically managed. The outcome of the patient was good. Despite literature concluding Chiari 3 malformation with an unfavorable outcome, however, meticulous management and good pre- and postoperative care, physical therapy, and follow-up are necessary for good outcome.

Thoracic bony human tail in a newborn baby: a rare case and review of literature.

BACKGROUND: The dorsal cutaneous appendage or so-called human tail is considered to be a cutaneous marker of underlying occult dysraphism. CASE PRESENTATION: We are presenting an unusual case of spinal dysraphism with a bony human tail at midthoracic region occurring in a newborn baby with tethered cord (conus at L4). Physical examination was unremarkable except for a thoracic appendage and a dermal sinus over coccyx region. Magnetic resonance imaging (MRI) scan of spine revealed bony outgrowth arising from D7 posterior element with multiple butterfly-shaped vertebrae found at D2 D4 D8 D9 D10 with low-lying conus at L4-L5 level. Surgical excision of the tail and untethering of the spinal cord along with excision of dermal sinus were performed. The infant had an uneventful postoperative period and was unchanged neurologically. CONCLUSION: To our knowledge, no similar case has been reported in English literature till date. DISCUSSION: The specific features of this rare case of human tail treated surgically are discussed in light of the available literature.

Von-Hippel Lindau (VHL) syndrome with bilateral cerebellar hemangioblastomas and retinal angiomas: A rare presentation.

INTRODUCTION AND IMPORTANCE: Hemangioblastomas are benign vascular neoplasms which originate almost exclusively from central nervous system (CNS). They account for 2 % of all intracranial neoplasms, 7 %-12 % of posterior fossa tumours. 60-75 % cases of hemangioblastomas occur sporadically and rest 25 % to 40 % occur in genetically inherited in the autosomal dominant neoplasia syndrome known as Von Hippel-Lindau (VHL) disease with mutation occurring on chromosome 3p. CASE PRESENTATION: We, hereby, report a case of one such rare case of a 30-year old male who presented to us with the complaint of holocranial headache for past 2 months associated with vomiting along with bilateral cerebellar signs and symptoms. MRI Brain revealed multiple cysts with enhancing mural nodules present on both cerebellar hemispheres. Due to recurrence and multi-focality of lesions, VHL syndrome was suspected. CLINICAL DISCUSSION: VHL is a rare autosomal dominant disease. MRI is the gold standard test. It demonstrates specific appearances depending on the tumour anatomy. Hemangioblastoma of the CNS in VHL usually develop from childhood at an age of <10 years or early teen until the age of 30 years. The most common locations for hemangioblastomas in VHL are cerebellum and spinal cord as seen in our patient. CONCLUSION: VHL being a lifelong disease with no cure till date, the patients should be frequently screened for lesions at various potential sites in his/her lifetime so as to provide required intervention at the earliest. Hence, a genetic screening for VHL (VHL tumour suppressor gene), should be performed in cases of hemangioblastoma.

A case of spinal tuberculosis presenting with cauda equina syndrome.

Tuberculosis (TB) of the central nervous system accounts for only 1% of all cases of TB but 50% of these occur in the spine. Common clinical manifestations of spinal TB include fever, weight loss, back pain, lower limb weakness (either upper or lower motor neuron), and skeletal deformities such as kyphosis or gibbus. Infective pathology caused by Mycobacterium tuberculosis with epidural collection can present as acute cauda equina syndrome. Here, we describe a 28-year-old female patient who presented with low back pain and ascending weakness of both lower limbs (Medical Research Council grade 4/5 with absent ankle reflex) for 2 months with sudden-onset loss of bladder and bowel control for 1 day without any localized spinal tenderness, swelling, or discharging sinus in the back. Magnetic resonance imaging (MRI) spine was suggestive of an intraosseous collection with spondylodiscitis at the L4 level vertebral level. MRI can help identify the involvement of the vertebral bodies on either side of the disk, disk destruction, cold abscess, vertebral collapse, and the presence of vertebral column deformities making it a sensitive tool for the detection of spinal TB. Antituberculous treatment remains the cornerstone of treatment. Surgery is required in selected cases, for example, large abscess formation, severe kyphosis, an evolving neurological deficit, or lack of response to medical treatment. A combined intervention of laminectomy with drainage of the collection with antitubercular therapy after microbiological evidence helped in the resolution of the patient's condition. Early diagnosis and appropriate treatment generally result in a good prognosis.

Dorsal medullary cavernous hemangioma presenting as obstinate hiccups and its surgical treatment: illustrative case.

BACKGROUND: Hiccups are characterized by involuntary, intermittent, repetitive, myoclonic, and spasmodic contractions of the diaphragm. Hiccups are termed "intractable" when they last for over 1 month. OBSERVATIONS: A rare case of intractable hiccups due to an uncommon location of cavernous hemangioma in the dorsal medulla is illustrated. With respect to the management, surgical excision was performed, and postsurgical complete recovery was witnessed, which has been reported only in six cases worldwide to date. LESSONS: A mechanism of the hiccups reflex arc is discussed in detail with special reference to the need for equal emphasis on evaluating central nervous system causes and peripheral etiologies for pertinent hiccups.

Complete intraventricular migration of ventriculo-peritoneal shunt: A rare case report.

INTRODUCTION AND IMPORTANCE: A ventriculoperitoneal (VP) shunt is a cerebral shunt used to treat hydrocephalus. This is used to remove the excessive accumulation of cerebrospinal fluid inside the ventricles. CASE PRESENTATION: We are reporting a rare case of complete intracranial migration of a ventriculoperitoneal shunt, a potentially fatal complication, presenting to us with hydrocephalous. The baby was taken up for surgery with endoscopic assisted shunt removal and shunt revision. CLINICAL DISCUSSION: Ventriculoperitoneal shunt is one of the common procedures used in infants and children, however, it is used in all age groups for hydrocephalous. The shunt is associated with multiple large number of complications like obstruction, infection, migration, and separation from the connected site. Etc. Shunt migration is a less common complication of which cranial migration is still less common. CONCLUSION: Optimum creation of the sub-galeal space for the shunt chamber, a smaller burr hole, a smaller dural opening, and proper anchorage of the chamber to the pericranium, are some of the measures that may be useful in obviating this complication.

"Serial ferritin titer" monitoring in COVID-19 pneumonia: valuable inflammatory marker in assessment of severity and predicting early lung fibrosis - prospective, multicentric, observational, and interventional study in tertiary care setting in India.

Introduction: The COVID-19 pneumonia is a heterogeneous disease with variable effect on lung parenchyma, airways, and vasculature leading to long-term effects on lung functions. Materials and methods: Multicentric, prospective, observational, and interventional study conducted during July 2020 to May 2021, in the MIMSR Medical College and Venkatesh Hospital Latur India, included 1000 COVID-19 cases confirmed with RT-PCR. All cases were assessed with lung involvement documented and categorized on HRCT thorax, oxygen saturation, inflammatory marker, ferritin at entry point, and follow-up during hospitalization. Age, gender, comorbidity, and use of BIPAP/NIV and outcome as with or without lung fibrosis as per CT severity were key observations. CT severity scoring is done as per universally accepted standard scoring tool as score < 7 as mild, 7-14 as moderate, and score > 15 as severe affection of the lung. Statistical analysis is done by using chi-square test. Observations and analysis: In study of 1000 COVID-19 pneumonia cases, age (< 50 and > 50 years) and gender (male versus female) have significant association with ferritin in predicting severity of COVID-19 pneumonia (p < 0.00001) and (p < 0.010), respectively. CT severity score at entry point with ferritin level has significant correlation in severity scores < 8, 8-15, and > 15 documented in normal and abnormal ferritin level as in 190/110, 90/210, and 40/360, respectively (p < 0.00001). Ferritin level has significant association with duration of illness, i.e., DOI < 7 days, 8-15 days, and > 15 days of onset of symptoms documented normal and abnormal ferritin levels in 30/310, 160/300, and 130/70 cases, respectively (p < 0.00001). Comorbidity as diabetes mellitus, hypertension, COPD, IHD, and obesity has significant association in COVID-19 cases with normal and abnormal ferritin level respectively (p < 0.00001). Ferritin level has significant association with oxygen saturation in COVID-19 pneumonia cases; cases with oxygen saturation > 90%, 75-90%, and < 75% are observed as normal and abnormal ferritin level in 110/100, 150/340, and 60/240 cases, respectively (p < 0.00001). BIPAP/NIV requirement during the course of COVID-19 pneumonia in critical care setting has significant association with ferritin level; cases received BIPAP/NIV during hospitalization were documented normal and abnormal ferritin level in 155/445 and 165/235 cases, respectively (p < 0.00001). Timing of BIPAP/NIV requirement during course of COVID-19 pneumonia in critical care setting has significant association with ferritin level; cases received BIPAP/NIV at entry point < 1 day, 3-7 days, and after 7 days of hospitalization were documented significance in fourfold raised ferritin level in 110/70, 150/160, and 30/80 cases, respectively (p < 0.00001). Follow-up of ferritin titer during hospitalization as compared to entry point abnormal ferritin has significant association in post-COVID lung fibrosis (p < 0.00001). Follow-up of ferritin titer during hospitalization as compared to entry point normal ferritin has significant association in post-COVID lung fibrosis (p < 0.00001). Conclusion: Ferritin is easily available, sensitive and reliable, cost-effective, and universally acceptable inflammatory marker in COVID-19 pandemic. Ferritin has very crucial role in COVID-19 pneumonia in predicting severity of illness and assessing response to treatment during hospitalization. Follow-up of ferritin titer during hospitalization and at discharge can be used as early predictor of post-COVID lung fibrosis.

A rare case of intramedullary tuberculosis with paraparesis.

Intramedullary tuberculoma (IMT) is considered to be a rare form of spinal tuberculosis (TB). Overall, TB of the central nervous system accounts for approximately 1% of all cases of TB and 50% of these involve the spine. The clinical presentation of spinal intramedullary TB is similar to an intramedullary spinal cord tumor mass. The factors attributable could be malnutrition, poor socioeconomic conditions, and immunodeficiency syndromes. As per the reports, the incidence of primary intramedullary TB is 2 in 100,000 cases among patients with TB. We describe one such patient who presented with progressive asymmetrical paraparesis due to histologically confirmed intraspinal tuberculoma. Paraparesis in spinal IMT is considered to be rare. Hereby, we present the case of a 29-year-old female who presented with asymmetric onset paraparesis of 6 months with associated numbness and tingling began in the left foot 3 months which was ascending in nature. There was no history of stiffness, involuntary movements, flexor spasms, thinning, or fasciculations of muscles. There was a loss of sensation pain, touch, and temperature below L3 with normal reflexes. Power in both the lower limbs was 1/5 as per Medical Research Council (MRC) grading. She underwent a contrast magnetic resonance imaging spine which was suggestive of an intramedullary SOL at D12 vertebral level. The patient underwent surgical intervention with resection of the SOL. Histopathology was confirmed to be an IMT. She was started on Category 1 (antitubercular drugs) and further investigated for primary source, which was found to be negative. We want to emphasize that TB can involve any part of the body. It should be kept as a differential diagnosis of any chronic inflammatory lesion involving the bony skeleton, especially in endemic countries where combined surgical and medical treatment is usually sufficient to provide a cure.

A rare case of calvarial tuberculosis in post-COVID-19 scenario.

Isolated calvarial involvement with tuberculosis (TB) is a very rare entity, with the incidence of only 0.01% of all patients with mycobacterial infections. The factors attributable could be malnutrition, poor socioeconomic conditions, and immunodeficiency syndromes. We hereby present the case of a 35-year-old male who had recently recovered from coronavirus disease 2019 and a diagnosed case of Evan's syndrome with secondary hemophagocytic lymphohistiocytosis who presented with a scalp swelling on the right frontotemporal region. He presented to the emergency department with acute-onset generalized tonic - clonic seizures with high-grade fever. Clinically, the swelling appeared like a cystic swelling of the scalp. On evaluation, there was a collection present below the scalp communicating with the extradural space, involving the underlying skull bone. The patient was operated with drainage of the abscess plus excision of the pathological underlying skull bone. The pus revealed florid amount of acid-fast bacillus on Ziehl-Neelsen staining. The patient was started on four drugs Category 1 antitubercular regimen. The patient responded well to the combined surgical and medical treatment. It should be emphasized that TB can involve any part of the body. It should be kept as differential diagnosis of any chronic inflammatory lesion involving the bony skeleton, especially in endemic countries where combined surgical and medical treatment is usually sufficient to provide a cure.

Gastric tuberculosis masquerading as advanced malignancy in a young male.

Gastrointestinal tuberculosis usually involves ileum and cecum in three-fourth of cases. Isolated gastric involvement is uncommon in the absence of pulmonary tuberculosis or immunodeficiency in affected individuals. Here, we describe a case of tuberculosis involving stomach and colon in an immunocompetant young patient who presented to us with dyspeptic symptoms, pain abdomen, and melena. Morphologically, the lesion mimicked as advanced malignancy, but laparoscopic biopsy confirmed the diagnosis. The patient responded well to medical treatment. It should be emphasized that tuberculosis can involve any part of gastrointestinal tract including stomach even in immunocompetent individuals, and it should be kept as differential diagnosis of any chronic inflammatory lesion of stomach, especially in endemic countries as medical treatment is usually sufficient to provide a cure.

Cutaneous lymphangiosarcoma following chronic lymphedema of filarial origin.

We are reporting a case of 36 yr old female with chronic filarial lymphedema of both legs. Subsequently she developed lymphangiosarcoma of her left leg which was confirmed histologically and on immunohistochemistry. Only three more cases of lymphagiosarcoma occurring in patients with filarial lymphedema are reviewed from available literature.