The next stage is hypoventilation (inadequate breathing) requiring intubation, orofacial dyskinesia and autonomic instability (dramatic fluctuations in blood pressure, temperature and heart rate). Investigation
Cerebrospinal fluid (CSF)
Examination of cerebrospinal fluid (CSF) shows elevated numbers of lymphocytes (but usually < 100 cells/μl); elevated CSF protein (but usually <1.5 g/L), normal glucose, elevated IgG index and oligoclonal bands. Patients with antibodies to voltage-gated potassium channels may have a completely normal CSF examination. Neuroimaging
Brain MRI is the mainstay of initial investigation pointing to limbic lobe pathology revealing increased T2 signal involving one or both temporal lobes in most cases.Serial MRI in LE starts as an acute disease with uni- or bilateral swollen temporomesial structures that are hyperintense on fluid attenuation inversion recovery and T2-weighted sequences. Swelling and hyperintensity may persist over months to years, but in most cases progressive temporomesial atrophy develops.PET-CT is not an essential investigation but can help in suspected cases with MRI negative for early diagnosis. Neuro-electrophysiology
EEG is mostly nonspecific slowing and epileptiform activity arising from temporal lobes. Treatment
Limbic encephalitis is a rare condition with no randomised-controlled trials to guide treatment. Treatments that have been tried include intravenous immunoglobulin, plasmapheresis, corticosteroids, cyclophosphamide and rituximab.If an associated tumour is found, then recovery is not possible until the tumour is removed. Unfortunately, this is not always possible, especially if the tumour is malignant and advanced. History
References


== External links ==
Diagnosis
Treatment
Treatment is symptomatic, often addressing indicators associated with peripheral pulmonary artery stenosis. Laryngotracheal calcification resulting in dyspnea and forceful breathing can be treated with bronchodilators including the short and long-acting β2-agonists, and various anticholinergics. Prognosis
Prognosis is  good, yet life expectancy depends on the severity and extent of diffuse pulmonary and arterial calcification. History
Keutel Syndrom was first described by the pediatric Cardiologist Jürgen Keutel and Colleagues in 1972 in Göttingen, Germany. References
External links
Keutel syndrome at NIHs Office of Rare Diseases