scholarly journals Early Aggressive Immunotherapy Improves Functional Outcome in Chronic Immune Sensory Polyradiculopathy

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Jasmine Shimin Koh ◽  
James Wei Min Tung ◽  
Genevieve Lynn Yu Tan-Yu ◽  
Thirugnanam Umapathi
Keyword(s):  
Sensory Nerve ◽  
Lower Limbs ◽  
Inflammatory Disorder ◽  
Dramatic Improvement ◽  
Independent Mobility ◽  
Nerve Roots ◽  
Sensory Ataxia ◽  
Unsteady Gait ◽  
History Of ◽  
Normal Strength

Chronic immune sensory polyradiculopathy (CISP) is an uncommon and treatable inflammatory disorder of the proximal sensory nerve roots. Patients typically present with severe sensory ataxia, normal motor examination, unsteady gait, and normal nerve conduction studies (NCS). We describe an elderly man who presented with a two-week history of progressive numbness of both legs and recurrent falls. He had hyporeflexia, normal strength, severe proprioceptive, and vibration sense loss in both lower limbs and was unable to stand or walk because of severe sensory ataxia. The NCS and MR scan of the spine were normal. Tibial somatosensory evoked potentials revealed proximal conduction defect and localized the pathology to the lumbar sensory nerve roots proximal to the dorsal root ganglion. Cerebrospinal fluid showed cytoalbuminergic dissociation suggestive of inflammation. CISP was diagnosed; he was given aggressive immunotherapy consisting sequentially of corticosteroids with mycophenolate mofetil and three cycles of intravenous immunoglobulin after which he regained independent mobility. Unlike previous reports where patients presented months-years after symptom onset and improved after single-line immunotherapy, our patient presented fairly acutely and made dramatic improvement only after aggressive combination therapy. We urge physicians to recognize this uncommon neurologic cause of sensory ataxia where early aggressive treatment is crucial for better functional outcomes.

scholarly journals Parotid carcinoma following chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids: a case report

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Yilun Deng ◽  
Bi Zhao ◽  
Bing Wei ◽  
Shihong Zhang ◽  
Ming Liu
Keyword(s):  
Clinical Symptoms ◽  
Brain Mri ◽  
Pulse Therapy ◽  
Inflammatory Disorder ◽  
Residual Symptoms ◽  
Parotid Carcinoma ◽  
Unsteady Gait ◽  
History Of ◽  
Nosologic Entity

Abstract Background Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disorder with unclear causes. Paraneoplastic etiology may be a cause. We report a case of CLIPPERS with parotid carcinoma. Case presentation A 54-year-old man with a history of lymphoma was hospitalized with a pontocerebellar syndrome. Brain MRI revealed that the pons and cerebellum were “peppered” with punctate and curvilinear enhancement lesions that supported the diagnosis of CLIPPERS. The relapse of lymphoma was excluded by a further cerebellum biopsy revealing predominantly CD3+ T cells in white matter. The patient was relieved after pulse therapy with intravenous methylprednisolone and a large dose of corticosteroids, but he complained of a worsening gait problem when corticosteroids were tapered to a lower dose. Although the clinical symptoms gradually improved again by increasing the dosage of corticosteroids with Azathioprine, the patient still had a slight unsteady gait during follow-up. At the 7-month follow-up, a parotid mass was detected by MRI and was verified as carcinoma by biopsy. After resection of parotid carcinoma, the residual symptoms and previous MRI lesions disappeared, and no relapse occurred. Conclusions CLIPPERS may not be a distinct nosologic entity but an overlapping diagnosis with other diseases. Some cases of CLIPPERS might be a subtype of paraneoplastic neurological syndromes (PNS) due to the similar mechanism of antibody-mediated encephalitis. Tumor screening and serum paraneoplastic autoantibody tests are recommended for patients with CLIPPERS, especially for those who relapse when corticosteroids treatment is stopped or tapered.

Aspergillus Spondylodiscitis After Spinal Stenosis Surgery: A Case Report

2020 ◽  
Vol 5 (3 And 4) ◽  
pp. 155-160
Author(s):  
Mohsen Aghapoor ◽  
◽  
Babak Alijani Alijani ◽  
Mahsa Pakseresht-Mogharab ◽  
◽  
...  
Keyword(s):  
Antifungal Drugs ◽  
The Body ◽  
Lower Limbs ◽  
Lumbar Pain ◽  
Case Presentation ◽  
Delay In Diagnosis ◽  
Death Case ◽  
Therapeutic Results ◽  
History Of ◽  
Probable Diagnosis

Background and Importance: Spondylodiscitis is an inflammatory disease of the body of one or more vertebrae and intervertebral disc. The fungal etiology of this disease is rare, particularly in patients without immunodeficiency. Delay in diagnosis and treatment of this disease can lead to complications and even death. Case Presentation: A 63-year-old diabetic female patient, who had a history of spinal surgery and complaining radicular lumbar pain in both lower limbs with a probable diagnosis of spondylodiscitis, underwent partial L2 and complete L3 and L4 corpectomy and fusion. As a result of pathology from tissue biopsy specimen, Aspergillus fungi were observed. There was no evidence of immunodeficiency in the patient. The patient was treated with Itraconazole 100 mg twice a day for two months. Pain, neurological symptom, and laboratory tests improved. Conclusion: The debridement surgery coupled with antifungal drugs can lead to the best therapeutic results.

Minocycline-Induced Agranulocytosis Presenting as Ecthyma Gangrenosum

2017 ◽  
Vol 1 (3) ◽  
pp. 161-164 ◽  
Author(s):  
Katherine Nolan ◽  
Reema Ishteiwy ◽  
John Alexis ◽  
Martin Zaiac ◽  
Anna Nichols
Keyword(s):  
Care Facility ◽  
Urgent Care ◽  
Severe Neutropenia ◽  
Dramatic Improvement ◽  
Therapy Discontinuation ◽  
Incision And Drainage ◽  
Ecthyma Gangrenosum ◽  
Minimal Improvement ◽  
History Of ◽  
Acute And Chronic Inflammation

A 51-year-old female with a history of rheumatoid arthritis was admitted for progressive fevers, chills and malaise. Five weeks prior, she started minocycline for an RA exacerbation. Two weeks after starting minocycline she developed an abscess on her right ankle that was treated at an urgent care facility with ceftriaxone and trimethoprim-sulfamethoxazole. She had minimal improvement so was switched to clindamycin. She developed additional abscesses on her right ankle and right axilla and spiking fevers so she was treated with incision and drainage under general anesthesia. Routine blood work obtained prior to surgery revealed severe neutropenia (0.74 103/ul) and the patient was urgently referred to the emergency department.  Skin biopsy was obtained on admission and revealed ulceration, necrosis, acute and chronic inflammation, vasculitis with vascular thrombosis and rod-shaped bacteria in blood vessel walls and lumina consistent with ecthyma gangrenosum. The following day tissue and blood cultures confirmed the growth of Pseudomonas aureginosa. Bone-marrow biopsy showed decreased granulopoiesis and hematopoiesis, and a diagnosis of minocycline-induced agranulocytosis presenting as ecthyma gangrenosum was made.  The patient had dramatic improvement with appropriate antibiotic therapy, discontinuation of minocycline and initiation of filgrastrim. She has remained healthy without recurrence for 17 months.    

scholarly journals Sirenomelia: two case reports

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Asiyeh Shojaee ◽  
Firooze Ronnasian ◽  
Mahdiyeh Behnam ◽  
Mansoor Salehi
Keyword(s):  
Case Reports ◽  
Three Dimensional ◽  
First Trimester ◽  
Mendelian Inheritance ◽  
Lower Limbs ◽  
Congenital Deformity ◽  
White Family ◽  
Her Family ◽  
Caudal Regression ◽  
History Of

AbstractBackgroundSirenomelia, also called mermaid syndrome, is a rare lethal multi-system congenital deformity with an incidence of one in 60,000–70,000 pregnancies. Sirenomelia is mainly characterized by the fusion of lower limbs and is widely associated with severe urogenital and gastrointestinal malformations. The presence of a single umbilical artery derived from the vitelline artery is the main anatomical feature distinguishing sirenomelia from caudal regression syndrome. First-trimester diagnosis of this disorder and induced abortion may be the safest medical option. In this report, two cases of sirenomelia that occurred in an white family will be discussed.Case presentationWe report two white cases of sirenomelia occurring in a 31-year-old multigravid pregnant woman. In the first pregnancy (18 weeks of gestation) abortion was performed, but in the third pregnancy (32 weeks) the stillborn baby was delivered by spontaneous vaginal birth. In the second and fourth pregnancies, however, she gave birth to normal babies. Three-dimensional ultrasound imaging showed fusion of the lower limbs. Neither she nor any member of her family had a history of diabetes. In terms of other risk factors, she had no history of exposure to teratogenic agents during her pregnancy. Also, her marriage was non-consanguineous.ConclusionThis report suggests the existence of a genetic background in this mother with a Mendelian inheritance pattern of 50% second-generation incidence in her offspring.

scholarly journals Atypical cutaneous and musculoskeletal manifestation of SARS-CoV-2: ‘COVID-19 toes’ and spasticity in a 48-year-old woman

2021 ◽  
Vol 14 (3) ◽  
pp. e241410
Author(s):  
Avery Kopacz ◽  
Cameron Ludwig ◽  
Michelle Tarbox
Keyword(s):  
Nervous System ◽  
Adult Patient ◽  
Early Identification ◽  
Lower Limbs ◽  
Rare Occurrence ◽  
Cutaneous Manifestations ◽  
Crucial Component ◽  
History Of ◽  
Screening Questions ◽  
Insight Into

Establishing accurate symptomatology associated with novel diseases such as COVID-19 is a crucial component of early identification and screening. This case report identifies an adult patient with a history of clotting dysfunction presenting with rare cutaneous manifestations of COVID-19, known as ‘COVID-19 toes’', previously described predominantly in children. Additionally, this patient presented with possible COVID-associated muscle spasticity of the lower limbs, as well as a prolonged and atypical timeline of COVID-19 infection. The rare occurrence of ‘COVID-19 toes’' in this adult patient suggests that her medical history could have predisposed her to this symptom. This supports the coagulopathic hypothesis of this manifestation of COVID-19 and provides possible screening questions for patients with a similar history who might be exposed to the virus. Additionally, nervous system complaints associated with this disease are rare and understudied, so this novel symptom may also provide insight into this aspect of SARS-CoV-2.

Lumbosacral intersegmental epispinal axons and ectopic ventral nerve rootlets

1987 ◽  
Vol 67 (2) ◽  
pp. 269-277 ◽  
Author(s):  
Wesley W. Parke ◽  
Ryo Watanabe
Keyword(s):  
Nerve Root ◽  
Sensory Nerve ◽  
Conus Medullaris ◽  
Spinal Nerve ◽  
Nerve Fibers ◽  
Ventral Horn ◽  
Spinal Nerve Roots ◽  
Nerve Roots ◽  
Content Type ◽  
Almost All

✓ An epispinal system of motor axons virtually covers the ventral and lateral funiculi of the human conus medullaris between the L-2 and S-2 levels. These nerve fibers apparently arise from motor cells of the ventral horn nuclei and join spinal nerve roots caudal to their level of origin. In all observed spinal cords, many of these axons converged at the cord surface and formed an irregular group of ectopic rootlets that could be visually traced to join conventional spinal nerve roots at one to several segments inferior to their original segmental level; occasional rootlets joined a dorsal nerve root. As almost all previous reports of nerve root interconnections involved only the dorsal roots and have been cited to explain a lack of an absolute segmental sensory nerve distribution, it is believed that these intersegmental motor fibers may similarly explain a more diffuse efferent distribution than has previously been suspected.

scholarly journals An infantile alantoaxial dislocation with patent foramen ovale managed with titanium cabling and allogenic bone grafts

2018 ◽  
Vol 10 (1) ◽  
Author(s):  
Seidu A. Richard ◽  
Zhi Gang  Lan ◽  
Xiao Yang ◽  
Siqing Huang
Keyword(s):  
Action Potentials ◽  
Patent Foramen Ovale ◽  
Early Stage ◽  
Sensory Nerve ◽  
Odontoid Process ◽  
Bone Destruction ◽  
Lower Limbs ◽  
Foramen Ovale ◽  
Compound Muscle Action Potentials ◽  
Patient Reported

Atlantoaxial dislocation is a disorder that is characterized with loss of stability of the atlas and axis (C1-C2) with consequential loss of usual articulation. Although this condition is very common, no one has reported a case as young as our patients. We present a 7-month infant with bilateral paralysis of the lower limbs for four (4) months with no history of trauma. Computer tomographic (CT-scan) imaging revealed alantoaxial dislocation with severe cervical spinal cord compression. The odontoid process is displaced outwardly with no bone destruction. Doppler echocardiogram done revealed patent foramen ovale. Thorough physical examination as well as radiological evaluation revealed no feather malformations. Electrophysio - logical studies reveal normal compound muscle action potentials (CMAP) and sensory nerve action potentials (SNAPs) in all the limbs. Electromyography (EMG) also revealed normal nerves in the limbs and the trunk. We attained a stable fusion and anatomical reduction using a posterior titanium wire and an iliac bone graft harvested from his mother. This is the youngest patient reported in literature. Infantile alantoaxial dislocation should be managed at early stage to prevent long-term neurologic disorders.

scholarly journals Pulmonary Crohn's Disease in Down Syndrome: A Link or Linkage Problem

2016 ◽  
Vol 10 (2) ◽  
pp. 206-211
Author(s):  
Danyal Thaver ◽  
Mirza Beg
Keyword(s):  
Down Syndrome ◽  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Pediatric Population ◽  
Pulmonary Nodules ◽  
Pulmonary Involvement ◽  
Lung Nodules ◽  
Inflammatory Disorder ◽  
Ct Guided ◽  
History Of

Crohn’s disease (CD) is an autoimmune inflammatory disorder that primarily affects the gastrointestinal tract. It may have pulmonary involvement, which has been rarely reported in pediatric patients. Down syndrome (DS) has been associated with increased frequency of autoimmune diseases. However, associations between CD and DS have been rarely reported. We present the case of a 5-year-old girl with known DS and a history of chronic intermittent abdominal pain who presented with persistent pneumonia. Her workup included a chest computed tomography (CT) scan that showed multiple noncalcified pulmonary nodules. An extensive infectious workup was done that was negative. CT-guided needle biopsy of the lung nodules showed necrotizing granulomas. This raised concern for primary CD with extraintestinal pulmonary manifestation. An esophagogastroduodenoscopy and colonoscopy were performed, and colon biopsies showed scattered epithelioid granulomas. Based on this information, there was consensus that her lung nodules were secondary to CD. She was started on standard therapy for CD, and her abdominal and respiratory symptoms gradually improved. However, she continues to have mild residual lung calcification and fibrosis. To our knowledge, this is the first reported case of pulmonary CD in a child with DS. The natural history of pulmonary CD in the pediatric population is not very well studied. Furthermore, since DS has been well known to be associated with increased frequency of malignancies and autoimmune conditions due to immune dysregulation, it is difficult to predict the severity and possible complications in this patient.

scholarly journals Multiple Cartilaginous Exostoses In A Pediatric Dog

2019 ◽  
Vol 47 ◽  
Author(s):  
José Artur Brilhante Bezerra ◽  
Ramon Tadeu Galvão Alves Rodrgues ◽  
Isabelle De Oliveira Lima ◽  
Luanda Pâmela César De Oliveira ◽  
Carlos Eduardo Bezerra De Moura ◽  
...  
Keyword(s):  
Physical Examination ◽  
Neurological Dysfunction ◽  
Lateral Part ◽  
Histopathological Analysis ◽  
Neoplastic Disease ◽  
Nerve Roots ◽  
Palpable Mass ◽  
Histopathological Findings ◽  
Adjacent Structures ◽  
History Of

Background: Multiple cartilaginous exostosis (MCE) is a rare neoplastic disease in dogs that is characterized by the presence of osteochondromas in multiple bones. MCE is a disorder of development during growth and maturation, the real etiology of which has yet to be elucidated, but is believed to have a genetic basis. Usually affected animals have a history of a palpable mass on the bone surface, causing anatomical deformities and compression of adjacent structures. Since MCE is a rare neoplastic disease in dogs it may be difficult to recognize in the clinic. The aim of this study was to report a case of MCE in a pediatric dog.Case: A female 4-month-old Rottweiler dog with a history of bone neoformation on the left hind limb, anorexia, weight loss, and difficulty walking received clinical care. The disease had its initial onset 2 months prior. At physical examination, the animal showed paresis of the hind limbs and a bony tumor on the lateral part of the left tibia. A complete blood cell count and serum biochemical profile were obtained. The only abnormality diagnosed was hyperproteinemia due to hyperglobulinemia. Other diagnostic tests were not authorized and the animal was euthanized. At the necropsy, an intrathoracic neoformation was observed from the sixth to the eighth rib on the right antimer. Some tumors were also observed on the fourth lumbar vertebra and between the first and the second sacral vertebra, and a left tibial tumor, which had been observed at physical examination. The sagittal section of the spine revealed the presence of extradural compression of the spinal cord due to vertebral proliferations with compression of the nerve roots. All of the proliferations had macroscopic similarities, being firm, sessile, irregular, and with complete adherence to deep planes. Tumor samples were submitted to histopathological analysis and the tissue morphology was compatible with osteochondroma. Based on the clinical, necroscopical, and histopathological findings, a diagnosis of MCE was established.Discussion: In MCE, animals less than one year of age are the most frequently affected, as was observed in the case reported. Clinical signs in MCE usually result from poor bone and cartilaginous anatomical formations and from compression of adjacent tissue structures. In the described patient, the proliferations located in the vertebrae and tibia and the consequent compression of nerve roots and nerves caused the neurological deficits. Unlike in canines, the etiology of MCE in humans is already well established and it is classified as an autosomal dominant genetic disorder. The diagnosis is usually obtained through the association of clinical examination with radiographic and histopathological findings. However, in the animal studied, since it was not possible to perform radiographs, confirmation was obtained only after performing histopathological examination of the neoformations, which is considered the standard for the diagnosis of MCE. Although it corresponds to a benign neoplastic process affecting dogs during their growth stage, it may significantly decrease the animal’s quality of life. In this situation, the prognosis of MCE is considered to be reserved to bad, and animals are often referred for euthanasia, as occurred in the animal under discussion, due to the progression of neurological dysfunction. Early recognition of MCE in routine pet hospital practice is of fundamental importance in order to properly institute treatment and monitoring.

scholarly journals Vermal cerebellar hemorrhage as the initial manifestation of undiagnosed hypertension

2016 ◽  
Vol 5 (1) ◽  
pp. 357 ◽  
Author(s):  
Alagoma Iyagba ◽  
Arthur Onwuchekwa
Keyword(s):  
Sudden Onset ◽  
Lower Limbs ◽  
Neck Stiffness ◽  
Cerebellar Haemorrhage ◽  
Initial Manifestation ◽  
Truncal Ataxia ◽  
Accident And Emergency ◽  
Undiagnosed Hypertension ◽  
History Of ◽  
Clonic Seizures

Vermal cerebellar haemorrhage is bleeding affecting the midline region of the cerebellum. It is uncommon, accounting for 5-13% of all intracerebral haemorrhages. It is a grave condition with a high mortality. The diagnosis must be made early, if not, most patients would die within 48 hours. A 70-year-old lady presented to the accident and emergency department with a six hours history of sudden onset generalized tonic-clonic seizures following activity and loss of consciousness. She was unconscious with a Glasgow coma score of 3/15 with neck stiffness, generalized hypotonia and globally diminished reflexes. Her blood pressure was 240/120 mmHg with a mean arterial pressure of 160 mmHg. The brain computerized tomographic scan showed a hyperdense area in the cerebellar vermis with extension to both hemispheres and fourth ventricle. Consciousness was regained after three weeks of conservative management following which she became extremely restless and noticed to have developed titubations with nystagmus of both eyes. She also developed intention tremors, scanning dysarthria, generalized hypotonia, dysmetria, dysdiadochokinesia and intentional bilateral tremors. Her recovery was slow and gradual. Once she was able to stand with support, she was noticed to have florid truncal ataxia, lower limbs ataxia, and titubations.

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