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Neurosarcoidosis


Presentation

  • By presenting this case, which to our knowledge is the first to report neurosarcoidosis presenting as hypothermia, our aim is to use it as a teaching moment and for further discussion on clinical presentations and management of such patients.[ncbi.nlm.nih.gov]
  • We present a case series of patients suffering from neurosarcoidosis in whom headache was the presenting symptom.[ncbi.nlm.nih.gov]
  • Neurosarcoidosis has a variety of clinical presentations.[ncbi.nlm.nih.gov]
  • Spinal sarcoidosis is a rare manifestation of sarcoidosis that can present a major diagnostic challenge.[ncbi.nlm.nih.gov]
  • At initial presentation, all patients lacked other cranial neuropathies and systemic sarcoidosis manifestations, making diagnosis difficult.[ncbi.nlm.nih.gov]
Weakness
  • Herein we present a case of a previously healthy 55-year-old man who presented with leg weakness and was found to have diffuse involvement of his spinal cord by neurosarcoidosis. He was treated with steroids and recovered completely.[ncbi.nlm.nih.gov]
  • People may have weakness in their arms and legs, and difficulty walking or controlling their urine or bowels.[ufhealth.org]
  • In June 2006, she suffered from band-like numbness and muscle weakness descending from the abdomen to bilateral lower extremities and urinary difficulty. Spinal magnetic resonance imaging showed an intramedullary lesion in C6 C7 region.[ncbi.nlm.nih.gov]
Pain
  • Yes I'm using it now and does help with my pain. I think it helps better for pain than the hydromorphone Yes I have isolated cardiac sarc (nowhere else) for Reply cont'd.[inspire.com]
  • Our case demonstrates the importance of considering isolated neurosarcoidosis as a potential underlying etiology of painful ophthalmoplegia, even without systemic manifestation of the disease.[ncbi.nlm.nih.gov]
  • While on 20 mg corticosteroids, she experienced subacute paraparesia with right leg pain. A spine MRI revealed a low thoracic hematomyelia at the T10-T11 level. Despite high doses of corticosteroids, her condition continued to worsen.[ncbi.nlm.nih.gov]
  • Pain medication Inflammation of certain nerves can be painful and may be treated with pain medication. Pain medication may include narcotic drugs, which can also cause side effects, such as nausea, dry mouth , and sleepiness.[medicalnewstoday.com]
  • RECENT FINDINGS: The broad range of clinical manifestations of neurosarcoidosis has recently expanded to include painful small fiber neuropathy.[ncbi.nlm.nih.gov]
Fatigue
  • In neurosarcoidosis fatigue is a core symptom, but little is known about the relevance of fatigue on cognitive functions in this disease. Previous results in healthy subjects suggest that fatigue strongly affects cognitive control processes.[ncbi.nlm.nih.gov]
  • A 37-year-old man presented with dysphagia to solids and liquids, dysphonia, fatigue and 50 lb weight loss over 2 months.[ncbi.nlm.nih.gov]
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: January 31, 2019[patientslikeme.com]
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: May 13, 2019[patientslikeme.com]
  • I spent most of the day asleep but no matter how much I slept it never relieved the fatigue. As my deterioration was slow and subtle those around me didn’t really notice it.[bacteriality.com]
Difficulty Walking
  • People may have weakness in their arms and legs, and difficulty walking or controlling their urine or bowels.[medlineplus.gov]
  • People may have weakness in their arms and legs, and difficulty walking or controlling their urine or bowels. In some cases, the spinal cord is so severely affected that both legs are paralyzed.[nlm.nih.gov]
Nausea
  • Symptoms vary as any part of the nervous system can be affected, but they may include fatigue, excessive thirst, dizziness, headaches, loss of vision or sense of smell, speech impairment, loss of movement in any body part and nausea.[independent.ie]
  • Pain medication may include narcotic drugs, which can also cause side effects, such as nausea, dry mouth , and sleepiness. Narcotic pain medication can also be habit-forming and is usually only prescribed in the short term.[medicalnewstoday.com]
  • Clinical Presentation Case 1 A 25 years male presented to Emergency Department with complaint of headache and altered consciousness for 3 days; he has nausea, vomiting without visual or behavioral problem.[omicsonline.org]
  • One patient was taking methotrexate only briefly and was changed to azathioprine because of severe nausea.[jamanetwork.com]
  • Five months later, the patient presented to the outside hospital with a headache, nausea, vomiting, ataxia, dizziness, and blurred vision.[nnjournal.net]
Vomiting
  • The most frequent features were headaches, visual failure, ataxia and vomiting.[jnnp.bmj.com]
  • Clinical Presentation Case 1 A 25 years male presented to Emergency Department with complaint of headache and altered consciousness for 3 days; he has nausea, vomiting without visual or behavioral problem.[omicsonline.org]
  • Five months later, the patient presented to the outside hospital with a headache, nausea, vomiting, ataxia, dizziness, and blurred vision.[nnjournal.net]
  • The headaches continued and I began having daily nausea and vomiting. Also, I began developing double vision and my balance was terrible. It seriously affected my quality of life.[steadyhealth.com]
  • She has lost a huge amount of weight as one of her symptoms was vomiting all the time. She also has sarcoid in her bones. She is being treated with infliximab which is being administered by IV every 4 weeks.[healthypages.com]
Tinnitus
  • The second is a 36-year-old woman with rapid-onset horizontal diplopia, left mixed severe sensorineural hearing loss (SNHL) and tinnitus, diffuse leptomeningeal enhancement on MRI, and progressive palsy of the left CNs IV, VI, VII, IX, X and XI, with[ncbi.nlm.nih.gov]
  • Patient and observation A 46-year-old lady with no significant medical history, was referred to our department after a 7 months history of gradually worsening bilateral hearing loss without tinnitus, vertigo or aural discharge.[panafrican-med-journal.com]
  • Weakness of jaw muscles and muscle twitch Loss of sight or defects in visual perception Impaired taste and smell Sensorineural deafness (“brain deaf”), tinnitus and vertigo Involuntary tongue movements causing speech abnormalities and problems with swallowing[healthhype.com]
  • I developed a host of new symptoms – severe photosensitivity, compression-like headaches, tinnitus, changes in proprioreception, blackouts, hair loss, mild fever, night sweats, anxiety, edema, abdominal pain and discomfort, and, swollen lymph nodes in[bacteriality.com]
Myalgia
  • Guillain-Barré syndrome variant: presenting with myalgias and acute facial diplegia . Mil Med. 2008 ;173(5): 507 – 508 . Google Scholar Crossref Medline ISI 18. Elias, MK, Mateen, FJ, Weiler, CR.[journals.sagepub.com]
Polyuria
  • A 22-year-old man was referred to the endocrinology service with profound weight gain, polyuria and lethargy.[ncbi.nlm.nih.gov]
  • Signs and symptoms of CNS involvement can include polyuria, polydipsia, obesity, impotence, amenorrhea, confusion/amnesia (short and long term memory), meningitis, and seizures (focal seizures).[encyclopedia.com]
Secondary Amenorrhea
  • In this report, we describe abrupt onset of secondary amenorrhea in a woman with history of chronic systemic sarcoidosis.[ncbi.nlm.nih.gov]
Seizure
  • Common manifestations include leptomeningeal inflammation with seizures, headache, cranial nerve palsies, hydrocephalus, or focal neurological deficits with white matter lesions or mass lesions.[ncbi.nlm.nih.gov]
  • On the day of admission, he had a witnessed seizure. Laboratory studies were significant only for mild leukopenia and erythrocyte sedimentation rate of 17 mm/h.[ncbi.nlm.nih.gov]
  • (iii) A 36-year-old man with raised intracranial pressure headaches presented with a seizure and a frontal haemorrhage. Hilar lymph node biopsy confirmed sarcoidosis, and he was treated successfully.[ncbi.nlm.nih.gov]
  • Whilst cranial neuropathy is the commonest presenting feature in adults with neurosarcoidosis, paediatric patients are more likely to present with seizures. Diagnosis presents a clinical challenge as a result of its protean manifestations.[ncbi.nlm.nih.gov]
  • We present a 45-year-old man with newly generalized tonic-clonic seizures due to a contrast-enhancing frontal lesion with perifocal edema suggestive for high-grade glioma (HGG).[ncbi.nlm.nih.gov]
Headache
  • In the remaining three cases headache was the only neurological symptom found in association with systemic features of sarcoidosis, and had the clinical features of tension-type headache.[ncbi.nlm.nih.gov]
  • Common manifestations include leptomeningeal inflammation with seizures, headache, cranial nerve palsies, hydrocephalus, or focal neurological deficits with white matter lesions or mass lesions.[ncbi.nlm.nih.gov]
  • (iii) A 36-year-old man with raised intracranial pressure headaches presented with a seizure and a frontal haemorrhage. Hilar lymph node biopsy confirmed sarcoidosis, and he was treated successfully.[ncbi.nlm.nih.gov]
  • […] both of the following: a) headache has significantly worsened in parallel with worsening of the neurosarcoidosis b) headache has significantly improved in parallel with improvement in the neurosarcoidosis headache is accompanied by one or more cranial[ichd-3.org]
  • A 28 years old female, presented with headache for 6 months. Her MRI Brain showed multiple ring enhancing lesions in the left cerebellum and vermis.[ncbi.nlm.nih.gov]
Stroke
  • Stroke is relatively rare, and hemorrhage is much less common than ischemia due to vasculitis.[ncbi.nlm.nih.gov]
  • It can mimic stroke or multiple sclerosis. Due to the variation in clinical presentation, diagnosis is difficult and often delayed.[ncbi.nlm.nih.gov]
  • A 62-year-old woman had diabetic mellitus and an old lacunar stroke in 1996. In 2003, she received steroid therapy for one year for the mediastinal mass lesion with a good response.[ncbi.nlm.nih.gov]
  • Symptomatic palatal tremor (SPT) is the result of a structural lesion, in the form of stroke, trauma or demyelinating disease.[ncbi.nlm.nih.gov]
  • Neurosarcoidosis and stroke. Stroke 1990 ; 21 : 152 –53 Brown MM, Thompson AJ, Wedzicha JA, et al. Sarcoidosis presenting with stroke. Stroke 1989 ; 20 : 400 –05 Ketonen L, Oksanen V, Kuuliala I.[ajnr.org]
Confusion
  • In the latter, it can be a source of considerable confusion, given the non-specific imaging appearance.[ncbi.nlm.nih.gov]
  • If the condition affects other parts of the brain or cranial nerves, symptoms may include: Headache Confusion, disorientation, and dementia Hearing or vision loss Bell’s palsy , which is characterized by weakness or drooping of the facial muscles Seizures[sarcoidosisnews.com]
  • If the condition is affecting the pituitary gland, symptoms may include: Changes in menstrual periods Fatigue Excessive thirst Excessive urination If the condition is affecting the brain or cranial nerves, symptoms may include: Confusion, disorientation[cedars-sinai.edu]
  • If it affects the brain or cranial nerves, you may have: headaches inability or change in your ability to smell or taste loss of hearing confusion dementia seizures speech difficulties psychiatric issues hallucinations irritability palsy in the facial[healthline.com]
  • While hepatic encephalopathy may have accounted for the patient's confusion and seizure disorder, it is less likely that it could account for MRI findings in the cerebellum and leptomeninges, both of which were suggestive of neurosarcoidosis.[path.upmc.edu]
Vertigo
  • . * Vertigo, dizzines and drowsiness. * Tongue deviation and atrophy. * Tingling, numbness on extremities. * Generalized weakness, leading to immobility and joint stiffness. * Changes in appetite. * Changes in sleep cycle (somnolence; insomnia). * Impaired[inspire.com]
  • […] gland, symptoms may include: Changes in menstrual periods Fatigue Excessive thirst Excessive urination If the condition is affecting the brain or cranial nerves, symptoms may include: Confusion, disorientation Hearing loss Dementia Delirium Dizziness, vertigo[cedars-sinai.edu]
  • Involvement of the brain or cranial nerves can cause: Confusion , disorientation Decreased hearing Dementia Delirium Dizziness , vertigo , or abnormal sensations of movement Double vision or other vision problems Facial palsy (weakness, drooping) Headache[ufhealth.org]

Workup

  • Laryngeal electromyography revealed high vagal injury, prompting workup on brain magnetic resonance imaging. On magnetic resonance imaging, 2 cases showed basilar leptomeningeal inflammation and 1 case showed a brainstem mass.[ncbi.nlm.nih.gov]
  • Based on our findings, we propose a diagnostic path for neurosarcoidosis, including spinal magnetic resonance imaging (MRI) as a mandatory and early step during diagnostic workup. [Indexed for MEDLINE] Free full text[ncbi.nlm.nih.gov]
  • Further workup failed to reveal any evidence of systemic disease. Intramedullary sarcoidosis without systemic sarcoidosis is extremely rare.[ncbi.nlm.nih.gov]
  • This case highlights the importance of tissue diagnosis and the diagnostic workup for sarcoidosis in cases of cryptogenic hydrocephalus.[nnjournal.net]
  • Therefore, a CT of the chest should be done on high clinical suspicion and moreover extensive workups like a MRI of the brain should be performed for patients who present with neurological symptoms as seen in our Case#01.[omicsonline.org]
Mediastinal Mass
  • In 2003, she received steroid therapy for one year for the mediastinal mass lesion with a good response.[ncbi.nlm.nih.gov]
Gliosis
  • Pathology demonstrated gliosis consistent with a low-grade central nervous system (CNS) glioma.[ncbi.nlm.nih.gov]

Treatment

  • We identified a total of 34 case reports on effective infliximab treatment of therapy-resistant neurosarcoidosis through PubMed search. Nineteen of the 34 cases reported the duration until treatment response.[ncbi.nlm.nih.gov]
  • TREATMENT OF NEUROSARCOIDOSIS: Although a lot of drugs have proved useful in treating neurosarcoidosis, corticosteroids are still the gold standard in treatment of these patients. Therapeutic protocols differ regarding the dose of these drugs.[ncbi.nlm.nih.gov]
  • At present, there are no set standards for treatment of patients suffering from NS. Early therapy of symptomatic patients is recommended. Corticosteroids still are the first line of treatment for NS patients.[ncbi.nlm.nih.gov]
  • Thirty-one of the 46 cases receiving treatment (67%) improved, 13 cases (28%) stabilized, and 2 cases (4%) deteriorated. First-line treatment with corticosteroids resulted in satisfactory reduction of symptoms in 21 of 43 patients (49%).[ncbi.nlm.nih.gov]
  • New treatments have emerged, but nevertheless one third of patients do not respond to treatment. Prospective cohort studies and RCTs on treatment are urgently needed.[ncbi.nlm.nih.gov]

Prognosis

  • Furthermore, future studies are needed to evaluate the prognosis and the optimal treatment strategy.[ncbi.nlm.nih.gov]
  • Neurosarcoidosis is difficult to diagnose early and might be associated with a poor prognosis.[ncbi.nlm.nih.gov]
  • This is particularly important because neurosarcoidosis has a poor prognosis and aggressive immunosuppressive treatment is generally recommended.[ncbi.nlm.nih.gov]
  • TNFα blockers have drastically improved rheumatoid arthritis prognosis by preventing joint destruction in DMARD resistant patients. Altering cytokine balance in immune diseases may expose to paradoxical adverse events.[ncbi.nlm.nih.gov]
  • Prognosis The prognosis for patients with neurosarcoidosis varies. Approximately two-thirds of those with the condition will recover completely; the remainder will have a chronically progressing or on-and-off course of illness.[ninds.nih.gov]

Etiology

  • Our case demonstrates the importance of considering isolated neurosarcoidosis as a potential underlying etiology of painful ophthalmoplegia, even without systemic manifestation of the disease.[ncbi.nlm.nih.gov]
  • Sarcoidosis is a multisystem granulomatous disorder of unknown etiology first named and described in the 19th century.[ncbi.nlm.nih.gov]
  • Neurosarcoidosis was diagnosed pathologically by proximal dorsal root biopsy after systemic investigations for inflammatory, infectious, and neoplastic etiologies were found to be negative.[ncbi.nlm.nih.gov]
  • Sarcoidosis is a multisystem granulomatous disease of unknown etiology, with prevalence ranging from 1 to 50 per 100,000.[ncbi.nlm.nih.gov]
  • Sarcoidosis is a multisystem inflammatory disease of unknown etiology. Isolated neurosarcoidosis without signs of systemic involvement has rarely been reported in the literature.[ncbi.nlm.nih.gov]

Epidemiology

  • DISCUSSION: Fully comprehension of neurosarcoidosis is still a challenge due to its rarity and limited number of large published series, which renders the epidemiological study of this disease very difficult.[ncbi.nlm.nih.gov]
  • PURPOSE OF REVIEW: The aims of this article are to discuss the epidemiology, pathophysiology and clinical phenomenology of neurosarcoidosis, as well as current approaches to diagnosis and treatment.[ncbi.nlm.nih.gov]
  • Electronic address: P.Ungprasert@gmail.com. 2 Division of Rheumatology, Department of Internal Medicine, Mayo Clinic College of Medicine and Science, 200 First Avenue Southwest, Rochester, MN 55905, USA; Division of Epidemiology, Department of Health[ncbi.nlm.nih.gov]
  • Overview In this article, the authors present the current understanding of the epidemiology, diagnostic findings, proposed pathogenesis, relevant genetics, and therapeutic options for neurosarcoidosis.[medlink.com]
  • Epidemiology While sarcoidosis can be found world wide, it has a higher prevelance in Americans of West African descent and in North European Caucasions than in other populations. The age of onset varies by ethnicity.[docneuro.com]
Sex distribution
Age distribution

Pathophysiology

  • PURPOSE OF REVIEW: The aims of this article are to discuss the epidemiology, pathophysiology and clinical phenomenology of neurosarcoidosis, as well as current approaches to diagnosis and treatment.[ncbi.nlm.nih.gov]
  • The pathophysiology responsible for the unusual CT appearance and the differential diagnosis are discussed. Article History Published in print: 1986[pubs.rsna.org]
  • Pathophysiology Sarcoidosis is a disease of unknown cause that leads to the development of granulomas in various organs. While the lungs are typically involved, other organs may equally be affected.[wikidoc.org]
  • Neurosarcoidosis Pathophysiology The Immune System The body’s immune defense is a well coordinated system. Cells target and destroy foreign agents that enter the body.[healthhype.com]

Prevention

  • It is important that neurosarcoidosis be in the differential diagnosis of mononeuritis multiplex when other causes cannot be found because early treatment could help prevent progression to other nerves.[ncbi.nlm.nih.gov]
  • The goals of available treatments include: halting inflammation, prevention of disease worsening and restoring neurological functions whenever possible. With immunosuppression, clinical remission of NS occurs in the majority of patients.[ncbi.nlm.nih.gov]
  • TNFα blockers have drastically improved rheumatoid arthritis prognosis by preventing joint destruction in DMARD resistant patients. Altering cytokine balance in immune diseases may expose to paradoxical adverse events.[ncbi.nlm.nih.gov]
  • Immuno-modulating agents must be envisaged in severe neurosarcoidosis, to prevent complications.[ncbi.nlm.nih.gov]
  • OBJECTIVE: To compare the efficacy of methotrexate (MTX) and mycophenolate mofetil (MMF) in the prevention of relapses in neurosarcoidosis.[ncbi.nlm.nih.gov]

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