Profile of Neurological Manifestations in Children Presenting With Rickettsial Disease

Objective: To study the profile of neurological manifestation of rickettsial disease in children. Methods: Review of hospital records was done in a tertiary care hospital for the period from January to December, 2020. Data of all the children fulfilling the inclusion criteria i.e., clinical criteria and serology were retrieved from the hospital records. Results: Of the total 7974 children admitted over this period, 178 were diagnosed with rickettsial disease wherein 54 (33.3%) had neurological involvement. Convulsions (59%), altered sensorium (56%), headache (44%), meningeal signs (37%), ataxia, (11%), lateral rectus palsy (7.5%) and stroke (7.5%) were the major neurological manifestations. Cerebrospinal fluid (CSF) analysis done in 30 (55%) children showed pleocytosis [median (IQR) cells 15 (3.75, 50)] with lymphocyte predominance [median (IQR) lymphocytes 11.5 (3, 38.75)] and elevated proteins [median IQR 41.5 (29.75,61)]. Neuroimaging abnormalities noticed were cerebral edema (n=7), cerebellar hyperintensities (n=5), basal ganglia infarcts (n=2) and hippocampal hyperintensities (n=1). Conclusion: Early recognition of rickettsial infection as a cause of neurological manifestation would facilitate early specific management.

Prisms in the treatment of diplopia with strabismus of various etiologies

Purpose: We aimed to study the success of prism in regard to diplopia resolution score and associated factors in patients presenting with symptomatic diplopia arising from various etiologies. Methods: In this descriptive, retrospective study diplopia resolution among 31 patients who were prescribed prism were analyzed. Results: Fifty?four patients were evaluated for diplopia and 31 were included for the study done over 3 years. The mean follow?up was 15 months. Esotropia, exotropia, and hypertropia were seen in 39%, 51%, and 19.4% of patients, respectively. Furthermore, 71% received Fresnel prism and 29% were given ground glass prism. The mean prism power prescribed was 13.3 PD. 87% had complete resolution of diplopia; 96.8% continued usage of prism. High success rates were seen among patients with decompensated strabismus, sixth and fourth nerve palsy. Horizontal prism and oblique prism in the form of Fresnel prism yielded complete resolution of diplopia (P = 0.028). There was no association between the success of prism and etiology (P 0.058), history of trauma (P = 0.212), and type of deviation (P = 0.387). The study showed that oblique Fresnel prism can be considered for combined deviation. Conclusion: Our study showed prism to be effective in alleviating diplopia over a varied range of etiologies

Botulinum toxin A in the early treatment of sixth nerve palsy in type 2 diabetes

Purpose: Our study aims at evaluating the efficacy and safety of botulinum toxin A in the early treatment of sixth nerve palsy in type 2 diabetic patients. Methods: This study is a prospective and interventional clinical case series of patients presenting with acute onset of sixth cranial nerve palsy, who received injection botulinum toxin A. Results: Thirty-one cases were included in the study. 58% of the study subjects had incomplete palsy at presentation (abduction deficit -1 to -3) and 42% had complete palsy (-4 and -5). The median dosage of injection was 5 U (range 3--6 U). The median follow-up period is 2 months. The P value shows that there is statistically significant improvement in head turn, ocular deviation in primary position, and improvement in abduction between baseline and 1 week (P-value <0.001), 1 month (P-value <0.001) and 2 month (P-value <0.001) postinjection follow-up visits. 90.3% of patients had full resolution of symptoms in the last follow-up visit. 83.9% of patients were successfully treated. Conclusion: Early injection of botulinum toxin A in select patients with acquired sixth nerve palsy due to diabetes is a safe and efficient treatment option in alleviating symptoms, restoring function and quality of life and reducing need for surgical interventions in future.

Treatment of lateral rectus paralysis by transposition of superior rectus to lateral rectus / Tratamento de paralisia de reto lateral pela transposição do reto superior para o reto lateral

ABSTRACT Objective: To evaluate the results of the surgical technique described by Crouch in the correction of lateral rectal paralysis (LR) esotropia. Methods: A study conducted of six patients with VI cranial nerve palsy, with more than three months, and associated contracture of the medial rectus muscle. The causes of paralysis varied in traumatic, congenital and neuropathic. The patients underwent surgical correction, performed with a technique consisting of the instillation of the superior rectus muscle (SR) and its suture above the insertion of the LR muscle, completed with Foster´s suture (suture joining, 8mm of the muscle insertion, the body of the SR and LR). The patients were followed for six months. Results: Five patients presented preoperative deviation between 30 and 50 prismatic diopter (PD), and one patient presented a deviation greater than 100 PD, and therefore, the patient required surgical reintervention due to residual deviation. The other five patients presented postoperative orthoppy, with better visual acuity and no vertical deviations. Conclusion: The surgery proposed by Crouch has been shown to be a good alternative to conventional techniques, such as Carlson-Jampolsky surgery, being a technically simpler procedure with good results.

RESUMO Objetivo: Avaliar resultados da técnica cirúrgica descrita por Crouch na correção de esotropia por paralisia do reto lateral (RL). Métodos: Estudo realizado com seis pacientes diagnosticados com paralisia de IV par craniano, com mais de três meses, e com contratura associada do musculo reto medial. As causas da paralisia variaram em traumática, congênita e neuropática. Os pacientes foram submetidos à correção cirúrgica, realizada com técnica que consiste na desinserção do músculo reto superior (RS) e sua sutura acima da inserção do músculo RL, completada com ponto de Foster (sutura unindo, a 8mm da inserção muscular, o corpo do RS e RL). Os pacientes foram seguidos por seis meses. Resultados: Cinco pacientes apresentaram, no pré-operatório, desvio entre 30 e 50 dioptrias prismáticas (DP), e um paciente apresentava desvio maior que 100DP, sendo, portanto o paciente que necessitou de reintervenção cirúrgica, por desvio residual. Os outros cinco pacientes apresentaram ortotropia no pós-operatório, com melhor da acuidade visual e sem desvios verticais. Conclusão: A cirurgia proposta por Crouch demostrou-se uma boa alternativa às técnicas convencionais, como cirurgia de Carlson-Jampolsky, sendo um procedimento tecnicamente mais simples e com bons resultados.

Pituitary Apoplexy Presenting as Isolated Bilateral Oculomotor Nerve Palsy

PURPOSE: To report a case of pituitary apoplexy presenting as isolated bilateral oculomotor nerve palsy. CASE SUMMARY: A 46-year-old male presented with bilateral ptosis and acute severe headaches for 6 days. He underwent head surgery and bilateral vitrectomy 12 years prior to his visit because of ocular and head trauma. He mentioned that previous visual acuities in both eyes were not good. The initial corrected visual acuity was finger counting in the right eye and 20/500 in the left eye. Ocular motility testing revealed the limitation of adduction, supraduction, and infraduction with complete bilateral ptosis in both eyes, and his left pupil was dilated. He was diagnosed with an isolated bilateral oculomotor nerve palsy. Magnetic resonance imaging indicated pituitary gland hemorrhage with a tumor, which was suspicious of pituitary apoplexy. The patient was treated intravenous with 1.0 g methylprednisolone to prevent the corticotropic deficiency. In addition, he underwent surgical decompression using a navigation-guided transsphenoidal approach and aspiration biopsy. He was confirmed with pituitary adenoma using a pathological examination. The patient's ocular movements began to dramatically improve by the third day postoperatively. At 4 months postoperative follow-up, his ocular movement and double vision were completely recovered. CONCLUSIONS: This was a rare case of pituitary apoplexy with bilateral isolated oculomotor nerve palsy, which was the first report in the Republic of Korea. A full recovery was achieved after early surgical treatment.

Weber syndrome secondary to brain stem tuberculoma

This case report describes a rare presentation of presumed brain stem tuberculoma in a 28-year-old male who presented with acute onset of third cranial nerve palsy with contralateral hemiparesis (Weber syndrome) and upgaze palsy. Isolated midbrain tuberculoma is rare, presenting with varied clinical manifestations and radiological findings posing as a diagnostic dilemma. Weber syndrome is commonly caused by midbrain infarct secondary to occlusion of branches of the posterior cerebral artery and rarely from a tuberculoma. The patient is a case of disseminated tuberculosis with granuloma in midbrain causing pressure effect, thereby presenting with features consistent with Weber syndrome and upgaze palsy. The patient had good recovery with antitubercular treatment and systemic steroids.

Paresia recurrente de sexto nervio craneano asociado a hipoplasia de sexto nervio / Recurrent paresis of sixth cranial nerve associated with sixth nerve hypoplasia

La paresia de sexto nervio craneano recurrente es una entidad ampliamente conocida en la población pediátrica que ha sido asociado a múltiples etiologías, siendo las más frecuentes las infecciones virales o postinmunizaciones. A continuación, exponemos el caso de una niña de 5 años que presenta 2 episodios de déficit de abducción del ojo izquierdo debido a paresia de sexto nervio craneano, que se resolvieron espontáneamente. El estudio con resonancia magnética reveló hipoplasia del sexto nervio craneano izquierdo.(AU)

Recurrent sixth nerve palsy is a well-known entity in pediatric population associated to multiple causes, most frequently viral infection or immunization. We report a case of a 5 years old girl who presents two episodes of left abduction deficit due to sixth nerve palsy that partially resolved spontaneously. A magnetic resonance imaging study revealed a hypoplastic left sixth nerve.(AU)

Cranial Nerve Palsy after Onyx Embolization as a Treatment for Cerebral Vascular Malformation

The Onyx liquid embolic system is a relatively safe and commonly used treatment for vascular malformations, such as arteriovenous fistulas and arteriovenous malformations. However, studies on possible complications after Onyx embolization in patients with vascular malformations are limited, and the occurrence of cranial nerve palsy is occasionally reported. Here we report the progress of two different types of cranial nerve palsy that can occur after embolization. In both cases, Onyx embolization was performed to treat vascular malformations and ipsilateral oculomotor and facial nerve palsies were observed. Both patients were treated with steroids and exhibited symptom improvement after several months. The most common types of neuropathy that can occur after Onyx embolization are facial nerve palsy and trigeminal neuralgia. Although the mechanisms underlying these neuropathies are not clear, they may involve traction injuries sustained while extracting the microcatheter, mass effects resulting from thrombi and edema, or Onyx reflux into the vasa nervorum. In most cases, the neuropathy spontaneously resolves several months following the procedure.

The Neuro-ophthalmic Presentation of Intracranial Aneurysms

PURPOSE: To investigate the neuro-ophthalmic diagnosis and clinical manifestations of intracranial aneurysm. METHODS: A retrospective survey of 33 patients who were diagnosed with intracranial aneurysm and underwent neuro-ophthalmic examination from April 2008 to December 2016. Frequency of the first diagnosis of intracranial aneurysm in ophthalmology, neuro-ophthalmic diagnosis, location of intracranial aneurysm, examination of intracranial aneurysm rupture, and neurologic prognosis of Terson's syndrome patients were analyzed by image examination, neurosurgery, and ophthalmology chart review. RESULTS: Of the 33 patients, most patients (n = 31, 94%) were diagnosed with intracranial aneurysm at the neurosurgical department and only 2 patients were diagnosed initially at the ophthalmology department. Causes and association were: Terson's syndrome (n = 10, 30%), third cranial nerve palsy (n = 10, 30%), internclear ophthalmoplegia (n = 4, 12%), visual field defect (n = 3, 9%), optic atrophy (n = 3, 9%), sixth cranial nerve palsy (n = 2, 6%), and nystagmus (n = 1, 3%). The location of intracranial aneurysms were: anterior communicating artery (n = 13, 39%), medial communicating artery (n = 12, 36%), and posterior communicating artery (n = 5, 15%). Ten of 33 patients had Terson's syndrome, and 6 patients (60%) with Terson's syndrome had apermanent neurological disorder such as agnosia, gait disorder and conduct disorder. CONCLUSIONS: Third cranial nerve palsy was the most common neuro-ophthalmic disease in patients presenting with intracranial aneurysm. The neuro-ophthalmic prognoses for those diseases were relatively good, but, if Terson's syndrome was present, neurological disorders (agnosia, gait disorder, conduct disorder) were more likely to remain after treatment.

Clinical Features of Acquired Paralytic Strabismus

PURPOSE: The purpose of our study was to evaluate the cause of acquired third, fourth, and sixth nerve palsy while also establishing recovery rates and important factors for recovery. METHODS: A retrospective chart review was performed for 92 patients who visited the ophthalmologic department of Konyang University Hospital with acquired third, fourth, and sixth nerve palsy from March 2015 to February 2016. Recovery rates and factors for recovery were evaluated in only 66 patients who received first ocular exam within 2 weeks of onset and who were followed up for at least 6 months. Complete recovery was defined as both complete recovery of the angle of deviation and the restoration of eye movement in all directions. For the degree of ocular motor restriction, −4 was defined as not crossing the midline and −2 was defined as 50% eye movement. The degree of ocular motor restriction was analyzed from −1/2 to 4. RESULTS: The fourth nerve was affected most frequently (n = 37, 40.2%), followed by the sixth cranial nerve (n = 33, 35.9%), the third cranial nerve (n = 18, 19.6%), and a combination of 2 or more cranial nerves (n = 4, 4.3%). Vasculopathy (n = 44, 47.8%) was the most common etiology, followed by trauma (n = 14, 15.2%), idiopathic (n = 13, 14.1%), inflammation(n = 10, 10.9%), neoplasm (n = 9, 9.8%), and aneurysm (n = 2, 2.2%). Complete recovery rate occurred for 66.7% (n = 44) of patients, and the overall recovery rate (i.e., at least partial recovery) was 86.3% (n = 57). Significant factors for complete recovery were the initial deviation angle and the limitation of extraocular movement (p < 0.001, p = 0.005, respectively, according to univariate analysis). CONCLUSIONS: In this study, paralytic strabismus due to vasculopathy was the most common etiology, and a lower degree of initial deviation resulted in an improved complete recovery rate. In addition, a high overall recovery rate was possible through quick diagnosis and early treatment of cranial nerve palsy.

Central Skull Base Osteomyelitis: 10-Case Series in a Single Center / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Skull base osteomyelitis (SBO) typically evolves as a complication of malignant otitis externa (MOE) in diabetic patients and involves the temporal bone. Central SBO (CSBO), which mainly involves the sphenoid or occipital bones, has clinicaland radiological characteristics similar to those of SBO but without coexisting MOE. We investigated a group of patients with CSBO and studied the clinical course of CSBO. SUBJECTS AND METHOD: Medical records of patients who were diagnosed with CSBO were retrospectively analyzed from 1999 to 2014. RESULTS: Ten patients (mean age; 60.5 years) were identified. There were five males and five females. All patients suffered from headache, and six patients had cranial nerve palsy including oculomotor (20%), abducens (10%), vestibulocochlear (10%), glossopharyngeal (20%), vagus (30%) and hypoglossal (10%) nerve. Patients had underlying diseases including diabetes mellitus (40%), immunosuppression status after liver transplantation (10%) and cardiovascular disease (40%). Four patients received endoscopic biopsy and debridement for diagnostic and curative intent. Patients were treated with intravenous antibiotics for 5.1 weeks in average and oral antibiotics for 17 weeks. Mean follow-up period was 12.4 months and the mortality rate was zero. 40% of patients had residual neurologic deficit. The earliest sign of improving CSBO was headache (mean; 3.1 weeks) and the erythrocyte sedimentation rate was the latest improving sign (mean; 4 months). CONCLUSION: CSBO was diagnostic and therapeutic challenge to the clinicians. The timely diagnosis and long-term antibiotics therapy could avoid a mortality case and minimize the permanent neurologic deficit.

The diagnostic yield of neuroimaging in sixth nerve palsy - Sankara Nethralaya Abducens Palsy Study (SNAPS): Report 1.

Aims: The aim was to assess the etiology of sixth nerve palsy and on the basis of our data, to formulate a diagnostic algorithm for the management in sixth nerve palsy. Design: Retrospective chart review. Results: Of the 104 neurologically isolated cases, 9 cases were attributable to trauma, and 95 (86.36%) cases were classified as nontraumatic, neurologically isolated cases. Of the 95 nontraumatic, isolated cases of sixth nerve palsy, 52 cases were associated with vasculopathic risk factors, namely diabetes and hypertension and were classified as vasculopathic sixth nerve palsy (54.7%), and those with a history of sixth nerve palsy from birth (6 cases) were classified as congenital sixth nerve palsy (6.3%). Of the rest, neuroimaging alone yielded a cause in 18 of the 37 cases (48.64%). Of the other 19 cases where neuroimaging did not yield a cause, 6 cases were attributed to preceding history of infection (3 upper respiratory tract infection and 3 viral illnesses), 2 cases of sixth nerve palsy were found to be a false localizing sign in idiopathic intracranial hypertension and in 11 cases, the cause was undetermined. In these idiopathic cases of isolated sixth nerve palsy, neuroimaging yielded no positive findings. Conclusions: In the absence of risk factors, a suggestive history, or positive laboratory and clinical findings, neuroimaging can serve as a useful diagnostic tool in identifying the exact cause of sixth nerve palsy. Furthermore, we recommend an algorithm to assess the need for neuroimaging in sixth nerve palsy.

Spheniodal mucocele causing bilateral optic neuropathy and ophthalmoplegia.

Sphenoid sinus mucocele comprises only 2% of all paranasal sinus mucoceles. In literature, there is a case report on sphenoidal mucocele causing bilateral optic neuropathy, with unilateral partial recovery and cranial nerve palsy, but we did not come across any literature with bilateral optic neuropathy and ophthalmoplegia together caused by spheno-ethmoidal mucocele. We present such a rare case of spheno-ethmoidal mucocele causing bilateral optic neuropathy and unilateral sixth nerve palsy who had postsurgery, unilateral good vision recovery, and complete resolution of sixth nerve palsy.

Associated Injuries and Prognosis in Traumatic Isolated 3rd, 4th, and 6th Cranial Nerve Palsies

PURPOSE: To analyze the relationship between prognosis and the severity of associated injuries in traumatic isolated 3rd, 4th and 6th cranial nerve palsies. METHODS: The records of 39 patients (39 eyes) who were diagnosed with isolated 3rd, 4th or 6th cranial nerve palsy following trauma were reviewed retrospectively to analyze the etiology of trauma, the degree of associated injuries, the degree of paralysis, and the prognosis. RESULTS: The 4th cranial nerve was affected most frequently (19 patients, 48.7%), followed by the 6th nerve (12 patients, 30.8%) and the 3rd nerve (8 patients, 20.5%). Traffic accidents were the most frequent etiology of traumatic cranial nerve palsies. Loss of consciousness, intracranial hemorrhage, craniofacial fracture, c-spine injury, and optic nerve injury were among the most common accompanying conditions. The 3rd cranial nerve was the most severely paralyzed and showed the highest number of associated injuries. The recovery rate of the all cranial nerve palsies was 46.2%. By nerve, the 3rd cranial nerve palsy showed the lowest recovery rate of 25%, followed by the 4th nerve at 47.4%, and the 6th nerve at 58.3%. CONCLUSIONS: The prognosis was worse in patients with intracranial hemorrhage, compared with those without intracranial hemorrhage. There was a higher average number of associated injuries and the degree of paralysis was more severe in 3rd nerve palsies.

A case of Ophthalmic Zoster Presenting as Multiple Cranial Nerve Palsies, Ultimately Leading to Death

No abstract available.

Risk Factors and Prognosis of Isolated Ischemic 3rd, 4th, 6th Cranial Nerve Palsy

PURPOSE: To investigate the clinical features and risk factors of ischemic third, fourth, sixth cranial nerve palsy. METHODS: Retrospectively, we reviewed the medical records of 46 eyes of 46 patients who were diagnosed with ischemic third, fourth, sixth nerve palsy alone such as age of onset, risk factors, recovery rate and recovery time. RESULTS: The mean age of onset was 64.9 years. Of the 46 patients, 15 patients (32.6%) in third cranial nerve palsy group, 15 patients (32.6%) in fourth cranial nerve palsy group, 16 patients (34.8%) in sixth cranial nerve palsy group. The risk factor of hypertension in 30 patients (65.2%) was the most common than other risk factors such as diabetes, hyperlipidemia, elevated blood hematocrit, ischemic heart disease, left ventricular hypertrophy, smoking. The mean number of risk factors was 2.3 +/- 0.5 in third cranial nerve palsy group, 1.6 +/- 1.1 in sixth cranial nerve palsy group, 1.4 +/- 1.1 in fourth cranial nerve palsy group. Of the 46 patients, 42 patients (91.3%) were recovered. There was no significant difference in recovery rate by cranial nerve palsy. Recovery time of intracranial abnormalities group (10.5 +/- 2.9 weeks) in brain imaging study was late as compared with that of no intracranial abnormalities group (7.5 +/- 5.1 weeks). CONCLUSIONS: The overall recovery rate of isolated ischemic third, fourth, sixth cranial nerve was high. But if there are intracranial abnormalities in imaging study, it took a long time to recover. Also ischemic third cranial nerve palsy had multiple risk factors characteristically.

Risk Factors and Prognosis of Isolated Ischemic 3rd, 4th, 6th Cranial Nerve Palsy

PURPOSE: To investigate the clinical features and risk factors of ischemic third, fourth, sixth cranial nerve palsy. METHODS: Retrospectively, we reviewed the medical records of 46 eyes of 46 patients who were diagnosed with ischemic third, fourth, sixth nerve palsy alone such as age of onset, risk factors, recovery rate and recovery time. RESULTS: The mean age of onset was 64.9 years. Of the 46 patients, 15 patients (32.6%) in third cranial nerve palsy group, 15 patients (32.6%) in fourth cranial nerve palsy group, 16 patients (34.8%) in sixth cranial nerve palsy group. The risk factor of hypertension in 30 patients (65.2%) was the most common than other risk factors such as diabetes, hyperlipidemia, elevated blood hematocrit, ischemic heart disease, left ventricular hypertrophy, smoking. The mean number of risk factors was 2.3 +/- 0.5 in third cranial nerve palsy group, 1.6 +/- 1.1 in sixth cranial nerve palsy group, 1.4 +/- 1.1 in fourth cranial nerve palsy group. Of the 46 patients, 42 patients (91.3%) were recovered. There was no significant difference in recovery rate by cranial nerve palsy. Recovery time of intracranial abnormalities group (10.5 +/- 2.9 weeks) in brain imaging study was late as compared with that of no intracranial abnormalities group (7.5 +/- 5.1 weeks). CONCLUSIONS: The overall recovery rate of isolated ischemic third, fourth, sixth cranial nerve was high. But if there are intracranial abnormalities in imaging study, it took a long time to recover. Also ischemic third cranial nerve palsy had multiple risk factors characteristically.

Ichthyosiform sarcoidosis revisited.

Cutaneous manifestations of sarcoidosis are present in approximately one-third of the cases. Ichthyosiform lesion is one of the extremely rare cutaneous manifestations of sarcoidosis. It is a uncommon, but specifi c cutaneous manifestation of sarcoidosis that may precede or appear simultaneously with the diagnosis of systemic sarcoidosis. Approximately 20 cases of ichthyosiform sarcoidosis have been reported in the PubMed database. We report here a case of sarcoidosis with ichthyosiform skin lesions along with central nervous system (CNS) and pulmonary involvement for its rarity and interesting clinical presentation.

Tapia Syndrome after Cervical Spine Surgery

Tapia syndrome is a rare entity characterized by unilateral paralysis of the tongue and vocal cord caused by Xth and XIIth cranial nerve lesions. However, there has been no report of Tapia syndrome immediately following spine surgery. A 47-year-old man underwent posterior decompressive laminectomy for cervical stenosis. The surgery took about 117 minutes and it was uneventful. Postoperatively he developed hoarseness of voice during speech, with deviation of tongue protrusion. On laryngoscopic examination, paralysis of the left side of the tongue and the soft palate was found and complete palsy of the left vocal cord was noted. After excluding surgical cause and craniocervical lesion, a clinical diagnosis of Tapia syndrome was made. Here we report a rare case of Tapia syndrome developed after posterior approach for cervical spine surgery and discuss the possible mechanisms of this uncommon syndrome.