Paroxysmal localized Hyperhidrosis, a case-report: When excessive sweating occurs in combination with severe headaches.

BACKGROUND: Paroxysmal localized hyperhidrosis is a rare disorder of the central autonomic nervous system. No association between paroxysmal hyperhidrosis and severe headache has been previously described in literature.Case description: A 65-year-old woman with idiopathic paroxysmal localized hyperhidrosis combined with severe holocranial headache attacks is described in this case report. Extensive diagnostic testing by means of laboratory examinations, 24-hour urinalyses, chest X-ray, abdominal ultrasound and computed tomography scans, and brain and spinal cord magnetic resonance imaging could not identify an underlying disorder. A diagnosis of idiopathic paroxysmal localized hyperhidrosis was made, and the patient was successfully treated with clonidine 0.075 mg three times a day, without any side effects. CONCLUSION: Paroxysmal localized hyperhidrosis is a rare central autonomic nervous system disorder that can occur in combination with severe headache. Both the headache and paroxysmal hyperhidrosis complaints were treated effectively with clonidine in the patient described in this case-report.

Pathologically confirmed autoimmune encephalitis in suspected Creutzfeldt-Jakob disease.

OBJECTIVE: To determine the clinical features and presence in CSF of antineuronal antibodies in patients with pathologically proven autoimmune encephalitis derived from a cohort of patients with suspected Creutzfeldt-Jakob disease (CJD). METHODS: The Dutch Surveillance Centre for Prion Diseases performed 384 autopsies on patients with suspected CJD over a 14-year period (1998-2011). Clinical information was collected from treating physicians. Antineuronal antibodies were tested in CSF obtained postmortem by immunohistochemistry on fresh frozen rat brain sections, by Luminex assay for the presence of well-characterized onconeural antibodies, and by cell-based assays for antibodies against NMDAR, GABABR1/2, GABAAR GLUR1/2, LGI1, Caspr2, and DPPX. RESULTS: In 203 patients, a diagnosis of definite CJD was made, while in 181 a variety of other conditions were diagnosed, mainly neurodegenerative. In 22 of these 181, the neuropathologist diagnosed autoimmune encephalitis. One patient was excluded because of lack of clinical information. Inflammatory infiltrates were predominantly perivascular and consisted mainly of T cells. The predominant locations were basal ganglia and thalamus (90%) and temporal lobes and hippocampus (81%). In 6 patients (29%), antineuronal antibodies were detected in postmortem CSF, directed against Hu, NMDAR, GABABR1/2, Caspr2, and an unidentified synaptic antigen in 2. The most frequent symptoms were dementia (90%), gait disturbance (86%), cerebellar signs (67%), and neuropsychiatric symptoms (67%). Immunopathologic and clinical findings did not differ between autoantibody-negative patients and patients with antineuronal antibodies. CONCLUSIONS: It is important to consider immune-mediated disorders in the differential diagnosis of rapidly progressive neurologic deficits.