Sjogren's syndrome (SS) is an autoimmune disorder characterized by lymphocytic and plasma cell infiltration of exocrine glands, resulting in dry mouth and keratoconjunctivitis sicca. The clinical symptoms may range from mucosal dryness to more systemic complaints. We report a 42-year-old man presenting with rapid cognitive decline and memory impairment for 2 months. Magnetic resonance imaging of the cranium revealed abnormal signals at the bilateral medial temporal lobe and hippocampal region. The autoimmune panel and paraneoplastic antibodies were negative. The patient was labeled as a case of primary SS based on the vasculitis profile and pathological examination of lip biopsy. This case illustrates a rare, heralding manifestation of SS as limbic encephalitis.A case of resection of a large dumbbell-shaped nerve sheath tumor at L1/L2 via a direct lateral minimally invasive approach is described. The tumor was removed via a lateral, subdiaphragmatic, rib-sparing, retropleural, retroperitoneal approach. The thoracolumbar junction can be challenging via the lateral approach and to the best of our knowledge this is the first report of excision of a paravertebral tumor via this approach. We believe this provided the benefits of direct operative trajectory and exposure, excellent tumor access, and avoided the need for facetectomy and extended spinal instrumentation across the thoracolumbar junction. Complete tumor excision was achieved with preserved motor function and improvement in radicular pain and sensory neurological deficit.Stiff-person syndrome (SPS) is a rare neurological disorder that causes muscle rigidity and stiffness of the trunk and proximal limb muscles, leading to movement difficulties and impaired function. Due to the rarity of the disease, studies on the benefit of rehabilitation for this disorder are quite limited. A 46-year-old female patient diagnosed with SPS complained of imbalance and movement difficulty. We prescribed therapeutic exercises aimed to reduce the stiffness of the trunk and proximal limbs and improve her function. Baseline measurement of the patient's range of motion, muscle power and tone, balance and functional abilities were taken pre- and post-program. Outcome measures showed a general improvement in the patient's muscle flexibility, balance, and functionality.A 67-year-old patient with a history of fully treated bowel carcinoma presented with a short history of unilateral lumbosacral radiculopathy. No neurological deficit was found on examination. Magnetic resonance imaging (MRI) demonstrated initially L4/5 foraminal narrowing that was believed to be the cause for the patient's symptoms; however, a nerve root block led to no improvement in symptoms. MRI was repeated at 5 months and demonstrated a mass causing compression of the S1 nerve. However, the patient declined surgical decompression. MRI at 14 months subsequently showed rapid growth of the lesion suggestive of an aggressive process such as a metastatic lesion or even a nerve sheath tumor such as a Schwannoma. Open biopsy and decompression revealed the lesion to be a facet joint cyst and the patient recovered well and had satisfactory postoperative imaging at 3 months follow-up. There are no reports in the literature of facet joint cysts growing this quickly and thus mimicking other forms of lesion.Cannabinoids have been found to be effective in controlling seizures and the highly purified form of cannabinoid derived for Cannabis sativa . Cannabidiol (CBD) is now approved for Lennox-Gastaut syndrome (LGS) and Dravet syndrome. CBD was used in a 9-year-old boy with LGS (unknown etiology) with very good results. The electroencephalography (EEG) response was very dramatic with near normalization of EEG background and complete control of seizures. The effect of CBD on EEG with such an improvement has not been described previously. Also, this adds to evidence that early intervention in LGS with CBD might be more helpful and improve outcomes.Gait disorders are common in the elderly as there are various causes of neurological and non-neurological conditions. On the other hand, most of the gait parameters do change with advancing age which is identified as age-related physiological changes in gait. At this point, the discrimination between age-related physiological changes and gait disorders may be strictly challenging. After identifying gait as an abnormal pattern, classification of it and making the responsible pathophysiology also require high-level expertise in this regard. Herein, we present a rare patient with corticobasal degeneration (CBD) who had admitted initially due to complaints of gait problems. Over a long time, the patient had received the misdiagnosis of gait abnormality due to musculoskeletal problems by multiple physicians. However, the detailed neurological exam showed a higher level gait disorder (HLGD). Further investigations at this point yielded the diagnosis of CBD.Objective  Antiepileptic drug (AED) therapy remains the primary form of treatment for epilepsy, noncompliance to which can result in breakthrough seizure, emergency department visits, fractures, head injuries, and increased mortality. Various tools like self-report measures, pill-counts, medication refills, and frequency of seizures can assess compliance with varying extent. Thus, assessment of compliance with AEDs is crucial to be studied. Materials and Methods  Compliance was assessed using pill-count and Morisky medication adherence scale (MMAS) during home visits. A pill-count (pills dispensed-pills remaining)/(pills to be consumed between two visits) value of 0.85 to ≤1.15 was recorded as appropriate compliance. Underdose (1.15) was labeled as noncompliance. https://www.selleckchem.com/products/lonafarnib-sch66336.html Score of 1 was given to each positive answer in MMAS. Score of ≥1 was labeled as noncompliance. Statistical analysis Relationship of demographic factors between compliant and noncompliant patients was analyzed using Chi-square test (SPSS version 21.0, IBM). Rest of the data was analyzed with the help of descriptive statistics using Microsoft Excel. p less then 0.05 was considered statistically significant. Results  Out of 105 patients, 54 patients were noncompliant with both pill-count and MMAS. 10 patients were noncompliant with pill-count only, while 10 were noncompliant with MMAS. Conclusion  Both tools complement each other when used in combination, as use of a single tool was not able to completely detect compliance.
Sjogren's syndrome (SS) is an autoimmune disorder characterized by lymphocytic and plasma cell infiltration of exocrine glands, resulting in dry mouth and keratoconjunctivitis sicca. The clinical symptoms may range from mucosal dryness to more systemic complaints. We report a 42-year-old man presenting with rapid cognitive decline and memory impairment for 2 months. Magnetic resonance imaging of the cranium revealed abnormal signals at the bilateral medial temporal lobe and hippocampal region. The autoimmune panel and paraneoplastic antibodies were negative. The patient was labeled as a case of primary SS based on the vasculitis profile and pathological examination of lip biopsy. This case illustrates a rare, heralding manifestation of SS as limbic encephalitis.A case of resection of a large dumbbell-shaped nerve sheath tumor at L1/L2 via a direct lateral minimally invasive approach is described. The tumor was removed via a lateral, subdiaphragmatic, rib-sparing, retropleural, retroperitoneal approach. The thoracolumbar junction can be challenging via the lateral approach and to the best of our knowledge this is the first report of excision of a paravertebral tumor via this approach. We believe this provided the benefits of direct operative trajectory and exposure, excellent tumor access, and avoided the need for facetectomy and extended spinal instrumentation across the thoracolumbar junction. Complete tumor excision was achieved with preserved motor function and improvement in radicular pain and sensory neurological deficit.Stiff-person syndrome (SPS) is a rare neurological disorder that causes muscle rigidity and stiffness of the trunk and proximal limb muscles, leading to movement difficulties and impaired function. Due to the rarity of the disease, studies on the benefit of rehabilitation for this disorder are quite limited. A 46-year-old female patient diagnosed with SPS complained of imbalance and movement difficulty. We prescribed therapeutic exercises aimed to reduce the stiffness of the trunk and proximal limbs and improve her function. Baseline measurement of the patient's range of motion, muscle power and tone, balance and functional abilities were taken pre- and post-program. Outcome measures showed a general improvement in the patient's muscle flexibility, balance, and functionality.A 67-year-old patient with a history of fully treated bowel carcinoma presented with a short history of unilateral lumbosacral radiculopathy. No neurological deficit was found on examination. Magnetic resonance imaging (MRI) demonstrated initially L4/5 foraminal narrowing that was believed to be the cause for the patient's symptoms; however, a nerve root block led to no improvement in symptoms. MRI was repeated at 5 months and demonstrated a mass causing compression of the S1 nerve. However, the patient declined surgical decompression. MRI at 14 months subsequently showed rapid growth of the lesion suggestive of an aggressive process such as a metastatic lesion or even a nerve sheath tumor such as a Schwannoma. Open biopsy and decompression revealed the lesion to be a facet joint cyst and the patient recovered well and had satisfactory postoperative imaging at 3 months follow-up. There are no reports in the literature of facet joint cysts growing this quickly and thus mimicking other forms of lesion.Cannabinoids have been found to be effective in controlling seizures and the highly purified form of cannabinoid derived for Cannabis sativa . Cannabidiol (CBD) is now approved for Lennox-Gastaut syndrome (LGS) and Dravet syndrome. CBD was used in a 9-year-old boy with LGS (unknown etiology) with very good results. The electroencephalography (EEG) response was very dramatic with near normalization of EEG background and complete control of seizures. The effect of CBD on EEG with such an improvement has not been described previously. Also, this adds to evidence that early intervention in LGS with CBD might be more helpful and improve outcomes.Gait disorders are common in the elderly as there are various causes of neurological and non-neurological conditions. On the other hand, most of the gait parameters do change with advancing age which is identified as age-related physiological changes in gait. At this point, the discrimination between age-related physiological changes and gait disorders may be strictly challenging. After identifying gait as an abnormal pattern, classification of it and making the responsible pathophysiology also require high-level expertise in this regard. Herein, we present a rare patient with corticobasal degeneration (CBD) who had admitted initially due to complaints of gait problems. Over a long time, the patient had received the misdiagnosis of gait abnormality due to musculoskeletal problems by multiple physicians. However, the detailed neurological exam showed a higher level gait disorder (HLGD). Further investigations at this point yielded the diagnosis of CBD.Objective  Antiepileptic drug (AED) therapy remains the primary form of treatment for epilepsy, noncompliance to which can result in breakthrough seizure, emergency department visits, fractures, head injuries, and increased mortality. Various tools like self-report measures, pill-counts, medication refills, and frequency of seizures can assess compliance with varying extent. Thus, assessment of compliance with AEDs is crucial to be studied. Materials and Methods  Compliance was assessed using pill-count and Morisky medication adherence scale (MMAS) during home visits. A pill-count (pills dispensed-pills remaining)/(pills to be consumed between two visits) value of 0.85 to ≤1.15 was recorded as appropriate compliance. Underdose (1.15) was labeled as noncompliance. https://www.selleckchem.com/products/lonafarnib-sch66336.html Score of 1 was given to each positive answer in MMAS. Score of ≥1 was labeled as noncompliance. Statistical analysis Relationship of demographic factors between compliant and noncompliant patients was analyzed using Chi-square test (SPSS version 21.0, IBM). Rest of the data was analyzed with the help of descriptive statistics using Microsoft Excel. p less then 0.05 was considered statistically significant. Results  Out of 105 patients, 54 patients were noncompliant with both pill-count and MMAS. 10 patients were noncompliant with pill-count only, while 10 were noncompliant with MMAS. Conclusion  Both tools complement each other when used in combination, as use of a single tool was not able to completely detect compliance.
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