Novel mechanism for benign essential blepharospasm.

Journal ArticlePURPOSE: The purpose of this study is to test the hypothesis that the photophobia of benign essential blepharospasm (BEB) is caused by sympathetically maintained pain. METHODS: Nineteen patients with photophobia and BEB were enrolled in an unblinded prospective treatment trial. The intervention was blockade of the superior sympathetic ganglion with local anesthetic. Outcome measures included the patient's subjective report of ocular surface dryness, foreign body sensation, and eyelid spasm. We also obtained video recordings of eyelid movements. RESULTS: Of the 19 patients, 13 reported subjective improvement in BEB symptoms after cervical sympathetic blockade (CSB). Thirteen of 19 patients also had objective evidence of decreased light-induced eyelid spasm after CSB. Ocular surface disease was present in 18 of 19 patients. CONCLUSION: These data support the hypothesis that in many patients with BEB there is a sympathetically maintained pain syndrome associated with external ocular disease. We speculate on a neurologic circuit that may explain these findings

Idiopathic intracranial hypertension. Relationship to depression, anxiety, and quality of life

Journal ArticleOBJECTIVE: To explore the incidence of depression and anxiety and to measure quality of life in women with idiopathic intracranial hypertension (IIH), a matched group cross-sectional study was conducted. Women with IIH (n = 28) were compared with control groups of weight- and age-matched women not diagnosed with IIH (n = 30) and with age-matched women of normal weight (n = 30). METHODS: Eighty-eight women completed a questionnaire soliciting health information and standardized questionnaires measuring depression, anxiety, and quality of life. The groups were compared using analysis of variance and chi2 tests. Where appropriate, post hoc comparisons were made using Fisher's test. RESULTS: Patients with IIH reported a greater number of adverse health problems than either of the control groups. Non-health-related psychosocial concerns were equally prevalent among the three groups, but IIH patients were significantly more affected by hardships associated with health problems than the other two groups. The patient group also had higher levels of depression and anxiety than the control groups. These adverse health conditions were reflected in decreased quality of life measures for the IIH patients. CONCLUSIONS: This study supports previous reports that link obesity and psychosocial difficulties, but obesity alone is not the explanation for the higher levels of depression and lower levels of quality of life

Autosomal dominant cerebellar ataxia with retinal degeneration: clinical, neuropathologic, and genetic analysis of a large kindred.

Journal ArticleThe autosomal dominant cerebellar ataxias (ADCA) comprise a heterogeneous group of neurologic disorders characterized by degeneration of the cerebellum, spinal cord, and brainstem. Genetic analysis has revealed two loci, SCA1 on chromosome 6p, and SCA2 on chromosome 12q, responsible for some ADCA. We present a four-generation kindred of 42 individuals, 12 of whom were clinically affected with ADCA and an associated cone dystrophy. Early loss of color discrimination with retinal and macular signs is followed by gradual progression of cerebellar dysfunction and development of pyramidal signs. Pathology shows degeneration of cerebellum, basis pontis, inferior olive, and retinal ganglion cells. For genetic analysis, we used polymorphic markers D6S89 and D12S79; linkage analysis gave negative results, excluding linkage to both SCA1 and SCA2. The data strongly support genetic heterogeneity consistent with the unique clinicopathologic features of the form of ADCA displayed in this large family

Not so benign intracranial hypertension.

Journal ArticleDespite many controversies, intracranial hypertension due to use of doxycycline does occur. Practitioners prescribing the tetracyclic antibiotics should be aware of the syndrome of increased intracranial pressure, and pay particular attention to the ocular fundus for papilloedema. Appropriate referral for visual testing should be made, and treatment directed at stopping the drug and instituting symptomatic treatment that lowers the intracranial pressure

Photophobia in a blind patient: An alternate visual pathway. Case report.

Journal ArticlePhotophobia is a common neurological and ophthalmological symptom that has been associated with a growing number of neurosurgical conditions, especially compressive lesions. The exact signaling pathways and neurophysiological features of the disorder are not well understood; however, data from multiple studies have shown the significance of the trigeminal system and the pretectal nuclei in its pathophysiology. The Author's report on a rare case of a blind patient who presented with photophobia without evidence of light perception. They also review the literature and early experimental data in an effort to understand the possible neuronal pathways and structures involved in photophobia

Carcinomatous meningitis as the presenting manifestation of gallbladder carcinoma: case report and review of the literature

Journal ArticleThe primary tumors that typically cause carcinomatous meningitis include lung cancer, breast cancer, leukemia, lymphoma and melanoma. A variety of neurological signs and symptoms can be seen depending on the extent and location of the meningeal metastasis. Once the diagnosis of carcinomatous meningitis is confirmed, the search for the primary tumor can be a challenge and at times may require extensive radiographic or even surgical evaluation to obtain specimen for pathological confirmation. Here we report a patient who presented with bilateral cranial nerve VIII and cerebellar symptoms, and was diagnosed with carcinomatous meningitis. Only after an exploratory laporatomy did it become clear that the initial symptoms were related to a metastatic gallbladder carcinoma

Compressive optic neuropathy caused by renal osteodystrophy. Case report.

Journal ArticleCompressive optic neuropathy with acute or chronic vision loss has been associated with various skull base tumors, aneurysms, Graves disease, trauma, and, less commonly, fibrous dysplasia and osteopetrosis. The Author's present a case of acute visual deterioration in a 25-year-old woman who had massive calvarial hypertrophy with optic canal stenosis secondary to renal osteodystrophy (uremic leontiasis ossea [ULO]: bighead disease). Significant visual field restoration was achieved with high-dose corticosteroids followed by optic nerve decompression. This is the first case report of cranial neuropathy associated with ULO

Neurologic conditions presenting as psychiatric disorders.

Journal ArticleUnderstanding underlying neuroanatomic function helps physicians to localize defects and search for treatable neurologic conditions. Neurologic conditions such as Huntington's chorea, Wilson's disease, Gille de la Tourette syndrome, brain tumors, encephalitis and meningitis, neurodegenerative conditions and metabolic or toxic conditions can have psychiatric manifestations

Vitamin A in the cerebrospinal fluid of patients with and without idiopathic intracranial hypertension

Journal ArticleWe quantified vitamin A in the cerebrospinal fluid of patients with idiopathic intracranial hypertension, elevated intracranial pressure of other causes and normal intracranial pressure. Vitamin A could be detected by high-pressure liquid chromatography in most of the specimens. There was a significantly higher level of vitamin A in the cerebrospinal fluid of some patients with idiopathic intracranial hypertension. Vitamin A toxicity may play a role in the pathogenesis of idiopathic intracranial hypertension

Selective MR imaging approach for evaluation of patients with Horner's syndrome.

Journal ArticlePURPOSE: To assess the usefulness of MR in the evaluation of patients with Horner's syndrome. PATIENTS AND METHODS: We prospectively performed MR imaging in 33 patients with Horner's syndrome (13 preganglionic and 20 postganglionic) using a protocol specifically designed for pre- and postganglionic varieties of this syndrome. Assignment of patients to pre- or postganglionic categories was performed on the basis of pharmacologic testing. RESULTS: Abnormalities in one-half of the patients with preganglionic Horner's syndrome included lateral medullary infarct, spinal cord/root disease, apical lung tumor, and paravertebral metastatic mass. Three of 20 patients with postganglionic Horner's syndrome had carotid artery dissection. CONCLUSION: Routine scanning of patients who have postganglionic Horner's syndrome with cluster headaches was not helpful in our small serie