Sarcoidosis is a multisystem, chronic inflammatory disease that is characterized by the development of non-caseating granulomas in multiple body tissues and organ systems. Neurological complications of systemic sarcoi...Sarcoidosis is a multisystem, chronic inflammatory disease that is characterized by the development of non-caseating granulomas in multiple body tissues and organ systems. Neurological complications of systemic sarcoidosis include peripheral and cranial neuropathies, myopathies, seizures, gait dysfunction, and cognitive decline. Because sarcoidosis has a predilection to involve the basilar meninges, cranial neuropathy is the most prevalent neurological deficit seen when the nervous system is involved. Sarcoidosis cranial neuropathy may occur at different stages of the disease and even as the initial clinical manifestation of central nervous system involvement. Attributing a cranial neuropathy to sarcoidosis can be challenging, particularly in the setting of normal imaging studies. In this review, cranial neuropathies in sarcoidosis are discussed in detail.展开更多
Purpose:To observe the clinical and imaging characteristics of radiation-induced optic neuropathy(RION).Methods:We retrospectively reviewed the clinical data of 43 patients(69 eyes)who were diagnosed with RION at the ...Purpose:To observe the clinical and imaging characteristics of radiation-induced optic neuropathy(RION).Methods:We retrospectively reviewed the clinical data of 43 patients(69 eyes)who were diagnosed with RION at the Chinese PLA General Hospital from 2010 to 2021.Results:The latency from radiotherapy to onset of visual loss ranged from 1 to 132(36.33±30.48)months.Optic disc pallor and optic disc edema were found in 27.0%(10/37)and 8.1%(3/37)of the eyes,respectively,within 2 months.After treatment,the best corrected visual acuity(BCVA)was restored in 24.6%(17/69)of the eyes and the final BCVA improved in 13.0%(9/69)of the eyes.An 82.5%(33/40)of the eyes with magnetic resonance imaging(MRI)showed enhancement of the affected optic nerve,mostly(69.7%)in the intracranial segment,and 36.4%(12/33)of the eyes with expansion and T2-high signals also showed enhancement of the affected optic nerve.The superior retinal nerve fiber layer(RNFL)and the outer circle superior quadrant(OS)of the inner limiting membrane to retinal pigment epithelium(ILM-RPE)layer thinned significantly during the first month.The center of the ILM-RPE layer thickened significantly during the first two months and the inner circle temporal quadrant(IT)of the ILM-RPE layer thickened significantly from the third to sixth month.The RNFL thinned significantly after 6 months except for the temporal quadrant,and the average inner circle superior quadrant(IS)and outer circle of the ILM-RPE layer thinned significantly after 6 months.There was no significant difference between hyperbaric oxygen therapy(HBOT)and high-dose intravenous methylprednisolone(IVMP)therapy in improving BCVA recovery or final BCVA(P>0.05).Conclusions:The structural damage of the RNFL and ILM-RPE layer occurred during the first month,the RNFL showed progressive thinning during the follow-up period,while the ILM-RPE layer showed thinning during the first month,thickening from the third to sixth month,and thinning after 6 months.There was a discrete region of enhancement of the optic nerve,often with expansion and high-T2 signals on MRI.HBOT and high-dose IVMP therapy were hardly effective for treating RION in the non-acute stage.展开更多
The intricate anatomy and physiology of cranial nerves have inspired clinicians and scientists to study their roles in the nervous system. Damage to motor cranial nerves may result from a variety of organic or iatroge...The intricate anatomy and physiology of cranial nerves have inspired clinicians and scientists to study their roles in the nervous system. Damage to motor cranial nerves may result from a variety of organic or iatrogenic insults and causes devastating functional impairment and disfigurement. Surgical innovations directed towards restoring function to injured motor cranial nerves and their associated organs have evolved to include nerve repair, grafting, substitution, and muscle transposition. In parallel with this progress, research on tissue-engineered constructs, development of bioelectrical interfaces, and modulation of the regenerative milieu through cellular, immunomodulatory, or neurotrophic mechanisms has proliferated to enhance the available repertoire of clinically applicable reconstructive options. Despite these advances, patients continue to suffer from functional limitations relating to inadequate cranial nerve regeneration, aberrant reinnervation, or incomplete recovery of neuromuscular function. These shortfalls have profound quality of life ramifications and provide an impetus to further elucidate mechanisms underlying cranial nerve denervation and to improve repair. In this review, we summarize the literature on reconstruction and regeneration of motor cranial nerves following various injury patterns. We focus on seven cranial nerves with predominantly efferent functions and highlight shared patterns of injuries and clinical manifestations. We also present an overview of the existing reconstructive approaches, from facial reanimation, laryngeal reinnervation, to variations of interposition nerve grafts for reconstruction. We discuss ongoing endeavors to promote nerve regeneration and to suppress aberrant reinnervation and the development of synkinesis. Insights from these studies will shed light on recent progress and new horizons in understanding the biomechanics of peripheral nerve neurobiology, with emphasis on promising strategies for optimizing neural regeneration and identifying future directions in the field of motor cranial neuron research.展开更多
Chronic inflammatory demyelinating polyneuropathy(CIDP) is a progressive autoimmune disorder that targets peripheral nerves. It commonly presents with motor-predominant dysfunction and enlargement of cranial nerves. W...Chronic inflammatory demyelinating polyneuropathy(CIDP) is a progressive autoimmune disorder that targets peripheral nerves. It commonly presents with motor-predominant dysfunction and enlargement of cranial nerves. With regards to hearing loss, a few cases of sensorineural loss have been described. We present a novel case of conductive hearing loss caused by a mass on the tympanic segment of the facial nerve in the setting of CIDP.展开更多
Objective: To determine the effectiveness of low saturated fat diet (LSFD) in patients with trigeminal neuralgia (TN). Design: 1) Internet forum where patients could request a 10 page LSFD plan, and 2) Follow-up asses...Objective: To determine the effectiveness of low saturated fat diet (LSFD) in patients with trigeminal neuralgia (TN). Design: 1) Internet forum where patients could request a 10 page LSFD plan, and 2) Follow-up assessment done by a retrospective 20-item questionnaire. Duration of treatment—2 months to 13 years. Pain rated on a Visual Analogue Scale and reported as typical, atypical, or both, defining typical TN as intermittent quick jolts/stabbing pain and atypical TN as continuous never ending discomfort. Setting: General community. Patients: 55 unselected patients, most with unilateral, severe and daily symptoms in V2 and V3 distribution for a mean duration of 8 years and on medications. 84% had pain level 9-10 before treatment, 89% had daily attacks and 31% had undergone surgical procedures. Intervention: LSFD for 2 months-13 years (mean 20 months). Main outcome measure: VAS score and medication use before and after LSFD treatment. Results: Reported SF content was 3-25 gm. With treatment 96% typical TN (p Conclusions: LSFD is effective in TN with high compliance, few adverse effects and may result in reduction/ elimination of medications even in most severe cases.展开更多
Here we report a patient with uncontrolled diabetes, who presented with giddiness and fall due to an episode of seizure. On evaluation with CT and MRI scans, he was found to have pan sinusitis with erosions of the sku...Here we report a patient with uncontrolled diabetes, who presented with giddiness and fall due to an episode of seizure. On evaluation with CT and MRI scans, he was found to have pan sinusitis with erosions of the skull base in the floor of sphenoid near lateral recess. PET-CT showed evidence of increased metabolism. He was operated upon by functional endoscopic sinus surgery and debridement of lesion near skull base. The histopathological examination revealed evidence of inflammation with no granulomas or fungal elements or tubercle bacilli. No organisms were grown in microbiological cultures. He started on empirical antibiotics for 3 months and showed improvement. We are reporting this case due to rarity to skull base osteomyelitis.展开更多
文摘Sarcoidosis is a multisystem, chronic inflammatory disease that is characterized by the development of non-caseating granulomas in multiple body tissues and organ systems. Neurological complications of systemic sarcoidosis include peripheral and cranial neuropathies, myopathies, seizures, gait dysfunction, and cognitive decline. Because sarcoidosis has a predilection to involve the basilar meninges, cranial neuropathy is the most prevalent neurological deficit seen when the nervous system is involved. Sarcoidosis cranial neuropathy may occur at different stages of the disease and even as the initial clinical manifestation of central nervous system involvement. Attributing a cranial neuropathy to sarcoidosis can be challenging, particularly in the setting of normal imaging studies. In this review, cranial neuropathies in sarcoidosis are discussed in detail.
基金supported by the National Key Research and Development Program. (Project code:2018YFE0113900 to S⋅W.)the National Natural Science Foundation of China (Project code:81870662 to S⋅W.)on the study.
文摘Purpose:To observe the clinical and imaging characteristics of radiation-induced optic neuropathy(RION).Methods:We retrospectively reviewed the clinical data of 43 patients(69 eyes)who were diagnosed with RION at the Chinese PLA General Hospital from 2010 to 2021.Results:The latency from radiotherapy to onset of visual loss ranged from 1 to 132(36.33±30.48)months.Optic disc pallor and optic disc edema were found in 27.0%(10/37)and 8.1%(3/37)of the eyes,respectively,within 2 months.After treatment,the best corrected visual acuity(BCVA)was restored in 24.6%(17/69)of the eyes and the final BCVA improved in 13.0%(9/69)of the eyes.An 82.5%(33/40)of the eyes with magnetic resonance imaging(MRI)showed enhancement of the affected optic nerve,mostly(69.7%)in the intracranial segment,and 36.4%(12/33)of the eyes with expansion and T2-high signals also showed enhancement of the affected optic nerve.The superior retinal nerve fiber layer(RNFL)and the outer circle superior quadrant(OS)of the inner limiting membrane to retinal pigment epithelium(ILM-RPE)layer thinned significantly during the first month.The center of the ILM-RPE layer thickened significantly during the first two months and the inner circle temporal quadrant(IT)of the ILM-RPE layer thickened significantly from the third to sixth month.The RNFL thinned significantly after 6 months except for the temporal quadrant,and the average inner circle superior quadrant(IS)and outer circle of the ILM-RPE layer thinned significantly after 6 months.There was no significant difference between hyperbaric oxygen therapy(HBOT)and high-dose intravenous methylprednisolone(IVMP)therapy in improving BCVA recovery or final BCVA(P>0.05).Conclusions:The structural damage of the RNFL and ILM-RPE layer occurred during the first month,the RNFL showed progressive thinning during the follow-up period,while the ILM-RPE layer showed thinning during the first month,thickening from the third to sixth month,and thinning after 6 months.There was a discrete region of enhancement of the optic nerve,often with expansion and high-T2 signals on MRI.HBOT and high-dose IVMP therapy were hardly effective for treating RION in the non-acute stage.
基金supported by the United States National Institute of Health grant 1K08DC012535(to MJB)Program for Neurology Research and Discoverythe Sinai Medical Staff Foundation Neuroscience Scholar Fund(to ELF)
文摘The intricate anatomy and physiology of cranial nerves have inspired clinicians and scientists to study their roles in the nervous system. Damage to motor cranial nerves may result from a variety of organic or iatrogenic insults and causes devastating functional impairment and disfigurement. Surgical innovations directed towards restoring function to injured motor cranial nerves and their associated organs have evolved to include nerve repair, grafting, substitution, and muscle transposition. In parallel with this progress, research on tissue-engineered constructs, development of bioelectrical interfaces, and modulation of the regenerative milieu through cellular, immunomodulatory, or neurotrophic mechanisms has proliferated to enhance the available repertoire of clinically applicable reconstructive options. Despite these advances, patients continue to suffer from functional limitations relating to inadequate cranial nerve regeneration, aberrant reinnervation, or incomplete recovery of neuromuscular function. These shortfalls have profound quality of life ramifications and provide an impetus to further elucidate mechanisms underlying cranial nerve denervation and to improve repair. In this review, we summarize the literature on reconstruction and regeneration of motor cranial nerves following various injury patterns. We focus on seven cranial nerves with predominantly efferent functions and highlight shared patterns of injuries and clinical manifestations. We also present an overview of the existing reconstructive approaches, from facial reanimation, laryngeal reinnervation, to variations of interposition nerve grafts for reconstruction. We discuss ongoing endeavors to promote nerve regeneration and to suppress aberrant reinnervation and the development of synkinesis. Insights from these studies will shed light on recent progress and new horizons in understanding the biomechanics of peripheral nerve neurobiology, with emphasis on promising strategies for optimizing neural regeneration and identifying future directions in the field of motor cranial neuron research.
文摘Chronic inflammatory demyelinating polyneuropathy(CIDP) is a progressive autoimmune disorder that targets peripheral nerves. It commonly presents with motor-predominant dysfunction and enlargement of cranial nerves. With regards to hearing loss, a few cases of sensorineural loss have been described. We present a novel case of conductive hearing loss caused by a mass on the tympanic segment of the facial nerve in the setting of CIDP.
文摘Objective: To determine the effectiveness of low saturated fat diet (LSFD) in patients with trigeminal neuralgia (TN). Design: 1) Internet forum where patients could request a 10 page LSFD plan, and 2) Follow-up assessment done by a retrospective 20-item questionnaire. Duration of treatment—2 months to 13 years. Pain rated on a Visual Analogue Scale and reported as typical, atypical, or both, defining typical TN as intermittent quick jolts/stabbing pain and atypical TN as continuous never ending discomfort. Setting: General community. Patients: 55 unselected patients, most with unilateral, severe and daily symptoms in V2 and V3 distribution for a mean duration of 8 years and on medications. 84% had pain level 9-10 before treatment, 89% had daily attacks and 31% had undergone surgical procedures. Intervention: LSFD for 2 months-13 years (mean 20 months). Main outcome measure: VAS score and medication use before and after LSFD treatment. Results: Reported SF content was 3-25 gm. With treatment 96% typical TN (p Conclusions: LSFD is effective in TN with high compliance, few adverse effects and may result in reduction/ elimination of medications even in most severe cases.
文摘Here we report a patient with uncontrolled diabetes, who presented with giddiness and fall due to an episode of seizure. On evaluation with CT and MRI scans, he was found to have pan sinusitis with erosions of the skull base in the floor of sphenoid near lateral recess. PET-CT showed evidence of increased metabolism. He was operated upon by functional endoscopic sinus surgery and debridement of lesion near skull base. The histopathological examination revealed evidence of inflammation with no granulomas or fungal elements or tubercle bacilli. No organisms were grown in microbiological cultures. He started on empirical antibiotics for 3 months and showed improvement. We are reporting this case due to rarity to skull base osteomyelitis.
