Neurologist
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Cervical spondylosis is part of the aging process and affects most people if they live long enough. Degenerative changes affecting the intervertebral disks, vertebrae, facet joints, and ligamentous structures encroach on the cervical spinal canal and damage the spinal cord, especially in patients with a congenitally small cervical canal. Cervical spondylotic myelopathy (CSM) is the most common cause of myelopathy in adults. ⋯ Neurologists should be familiar with this very common condition. Patients with mild signs and symptoms of CSM can be monitored. Surgical decompression from an anterior or posterior approach should be considered in patients with progressive and moderate to severe neurologic deficits.
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Patients with lower extremity sensory symptoms and pain but without clinical or standard neurophysiological examination abnormalities may have a small fiber neuropathy. Skin biopsy with intraepidermal nerve fiber density (IENFD) assessment has been promoted as a diagnostic tool for such patients. ⋯ Detection of reduced IENFD using skin biopsy may be sensitive and specific for clinically-defined syndromes consistent with small fiber neuropathy. Skin biopsy appears to have greater diagnostic utility than the neurologic examination and quantitative sensory testing, both of which rely heavily on subjective patient perception. Prospective studies that evaluate quantitative methodology (rather than modalities that rely on patient report) and do not include the diagnostic tests in the reference standard are needed. Consensus is needed regarding a reference standard definition for small fiber neuropathy.
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Sarcoidosis affects the central nervous system more frequently than previously appreciated. The diagnosis of neurosarcoidosis is often delayed, potentially leading to serious complications. Symptoms, when present, are not specific, may be subtle and resemble those of other neurologic diseases. ⋯ Neurologists should be aware of the varying presentations of neurosarcoidosis since early recognition of neurologic involvement in patients with undiagnosed or proven sarcoidosis is currently possible and critical to the prevention of disabling complications.
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A 24-year-old woman presented with right face pain and blurry vision. Examination revealed right pupil-sparing third nerve palsy and decreased sensation in the second and third divisions of the right trigeminal nerve. MRI demonstrated right cavernous sinus enhancement. ⋯ High-dose corticosteroid therapy again led to resolution of symptoms. This case demonstrates that Tolosa-Hunt syndrome can rarely recur on the contralateral side. MRI of this presentation has not been previously reported in the literature.
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The trigeminal autonomic cephalalgias (TACs) are a group of primary headache syndromes characterized by strictly unilateral head pain that occurs in association with ipsilateral cranial autonomic features. The group includes cluster headache, paroxysmal hemicrania, and short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. These syndromes differ in attack duration and frequency as well as the response to therapy. Most of the cases of these syndromes are primary headaches, though numerous symptomatic cases have been described albeit that it is difficult to establish a causal relationship with the underlying pathology in most cases. ⋯ Forty cases of symptomatic TACs were identified. These symptomatic headaches were associated with atypical phenotypes, abnormal examination, and poor treatment response though a significant minority had a typical presentation. A relatively high proportion of all TACs were secondary to pituitary tumors. It is difficult to draw up guidelines for the diagnostic workup required on the basis of this small retrospective case series. It remains unclear whether every TAC patient requires neuroimaging, though if it is considered then magnetic resonance imaging is the preferred modality. In addition, we suggest that all TAC patients should be carefully assessed for pituitary disease related symptoms but further investigations with magnetic resonance imaging of the pituitary gland and pituitary hormonal profile should only be undertaken in patients with atypical features, abnormal examination, or those resistant to the appropriate medical treatment.