Guest guest Posted January 17, 2007 Report Share Posted January 17, 2007 Sarcoidosis loves the olfactory cranial nerves. This is the the nerve that controls our sense of smell. If smell goes, so does taste. Here is a website that gives a good explanation of cns cranial nerve involvement with sarcoidosis. The 7th Cranial nerve is most often involved-- with the Bell's Palsy (face sags) being the primary culprit. http://www.medscape.com/viewarticle/545953_4 A Woman With Bilateral Bell's Palsy In This Article Presenting Complaint Neurologic Exam Hospital Course Final Diagnosis Patient Course Figures Tables Suggested Reading Final Diagnosis On the basis of the laboratory, neuroradiologic, and biopsy findings, we diagnosed neurosarcoidosis. SarcoidosisSarcoidosis is a multisystem granulomatous disease of unknown etiology that typically presents as a pulmonary disorder. Hutchinson initially described the condition as a cutaneous disorder in 1869 and, in 1875, Boeck named it "Sarkoid" for its pathologic resemblance to sarcoma. The typical pathologic findings are of lymphocytes and mononuclear phagocytes surrounding a noncaseating epithelioid cell granuloma. Incidence of organ system involvement is as follows: lung 85%-90%; lymph nodes 60%-73%; liver 39%-62%; skin 12%-25%; central nervous system (CNS) 5%-16%; eyes 8%-11%. Sarcoidosis is seen more commonly in women than in men, with a peak incidence occurring between the ages of 20 and 40 years. The lifetime risk is 0.85% in whites and 2.4% in blacks. In all, two thirds of patients experience spontaneous remission of the disease within 2 years, while 30% develop chronic disease. Lung involvement is the most frequent manifestation. Lymphohematogenous spread to virtually any organ may occur, and thus many manifestations can be observed. Common constitutional symptoms include malaise, fever, dyspnea, cough, arthralgias, lymphadenopathy, and rash. Diagnosis is typically made after multiple lines of evidence point to sarcoidosis: chest CT demonstrating hilar lymphadenopathy, serum ACE level greater than 50, Galium-67 or fluorodeoxyglucose positron emission tomography (FDG-PET) scan showing increased uptake in a patchy distribution, and pulmonary function tests. The gold standard for diagnosis is biopsy of skin, lymph node, brain, meninges, or muscle showing noncaseating granuloma. The prevalence of clinical involvement of the nervous system in sarcoidosis is estimated to be 5% to 15%. Any part of the CNS can be attacked by sarcoidosis, but cranial nerves, the hypothalamus, and the pituitary gland are most commonly involved. Neurosarcoidosis can manifest in an acute explosive fashion or as a gradual, chronic illness. NeurosarcoidosisIn 1987, Luke and colleagues established these criteria for the diagnosis of neurosarcoidosis: Presence of systemic sarcoidosis and the neurologic illness reasonably attributed to sarcoidosis, with other causes excluded; or Other systemic manifestations of sarcoidosis not established, but pathologic examination of neural or muscle tissue or clinical course consistent with sarcoidosis, and no other cause identified. Cranial nerve involvement is the most common neurologic manifestation. A peripheral 7th nerve palsy (Bell's palsy) is the most common cranial-nerve lesion and is the most common neurologic manifestation of sarcoidosis overall. The 7th nerve can be affected either unilaterally or bilaterally, simultaneously or sequentially. Brain parenchyma involvement is seen when the inflammatory process extends from the meninges through Virchow-Robin perivascular spaces. Spinal cord, peripheral nerve, and muscle may also be involved. Other cranial nerves may be affected as well. Cranial neuropathies may be single or multiple. Heerfordt's syndrome is a cranial neuropathy (affecting mostly the facial nerve) with uveitis, parotid-gland enlargement, and fever. The syndrome is highly suggestive of sarcoidosis. Horner syndrome, Argyll-on pupil, and Adie's pupil have also been described in cases of sarcoidosis. Acute or chronic meningeal signs and symptoms of neurosarcoidosis may occur, including fever, headache, neck rigidity, and sterile cerebrospinal fluid (CSF) with pleocytosis (particularly lymphocytes). CSF glucose levels may be low in about a fifth of patients. Mental status changes and polyradiculopathy are present sometimes. The basal meninges are most commonly affected, resulting in cranial neuropathy. Chronic meningitis will commonly recur . Neuroimaging studies, especially MRI, are the most sensitive diagnostic tools for the detection and localization of neurologic lesions. Specific Neurologic ManifestationsCranial nerve involvement Impaired taste and smell Blindness, blurry vision, double vision, visual field defects, pupillary abnormalities, dry and sore eyes Facial droop, slurred speech, impaired swallowing, hoarseness Vertigo, sensorineural deafness, tinnitus Weakness of trapezius and sternocleidomastoid muscles Tongue deviation and atrophy Meningeal involvement Headache is a prominent feature Aseptic meningitis (mononuclear pleocytosis with increased protein) Hydrocephalus (secondary to granuloma obstructing cerebral aqueduct) Intraparenchymal involvement Hypothalamus/pituitary disruption (electrolyte imbalance, changes in appetite, temperature, sleep and libido, diabetes insipidus, and syndrome of inappropriate secretion of antidiuretic hormone) Diffuse encephalopathy (delirium, psychiatric disorders, cognitive impairment) Seizure disorder (secondary to mass lesion, hydrocephalus, encephalitis, hypercalcemia) Spinal cord involvement Intra-, extramedullary; intra-, extradural process (myelopathy) Peripheral nerve involvement Mononeuropathy, mononeuropathy multiplex, polyneuropathy Sensory neuropathy - characterized by loss of sensation or abnormal sensation (eg, tingling, numbness, painful patches over the thorax) Motor neuropathy - characterized by weakness Muscle involvement Usually asymptomatic Symptomatic myopathy seen in less than 8 % of systemic sarcoidosis patients May observe elevated creatine kinase Differential DiagnosisThe differential for neurosarcoidosis is extensive and includes infectious, granulomatous, carcinomatous, inflammatory, and other neurologic processes ( Table ). TreatmentTherapeutic medical options for neurosarcoidosis are similar to those for systemic sarcoidosis. Corticosteroids represent the drugs of first choice. Doses for patients with neurosarcoidosis are higher than those advised for the treatment of other forms of sarcoidosis, including pulmonary. In general, the initial recommended dose of prednisone is 60 mg daily, whereas for neurosarcoidosis an initial dose of 1 mg/kg/day is typically recommended. In severe cases, high doses of intravenous methylprednisolone may be used for a few days to obtain a high initial loading dose. Previous Page Section 4 of 5 Next Page: Patient Course Medscape General Medicine. 2006;8(4):23. ©2006 Medscape Quote Link to comment Share on other sites More sharing options...
Guest guest Posted January 18, 2007 Report Share Posted January 18, 2007 Tracie, Big, big hugs! You are a genius! I was unfamiliar with `Weakness of trapezius and sternocleidomastoid muscles' so I did some research. Low & behold, I found a reference for symptoms resulting from a compromised sternocleidomastoid muscle ( http://www.round- earth.com/SCM.html ). Bingo! The article describes many of the symptoms I've experienced during my most recent flare. The severe pain emanating from the mastoid process which extends behind the ear was attributed to the confirmatory biopsy done on one of the large granulomas behind my ear. While this may be a contributor, the prime suspect is the sternocleidomastoid muscle. Like everything else, if I know what it is I can deal with it or possibly treat it. The website referenced above describes a massage & stretching routine which may ease the pain & alleviate some of the symptoms. I'll give it a go to see if it helps. Thank you so much! I hope your bout with the flu is short lived. Please know that you are in my thoughts & prayers. Stay well my friend. Tony Quote Link to comment Share on other sites More sharing options...
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