Sarcoidosis of nervous system

Sarcoidosis is a multisystem disease of unknown cause. It most often affects young and middle-aged people. It is often manifested by bilateral hilar adenopathy, pulmonary infiltrates, and ocular and skin lesions. The liver, spleen, lymph nodes, salivary glands, heart,  nervous system  ', muscles, bones and other organs can also be affected.

Epidemiology
It occurs in less than 10% of patients, in 0.8% of cases.

Symptoms
Symptoms include brain-based predilection, common facial nerve disorders (most commonly paralysis nervus facialis, less commonly hypothalamic and pituitary lesions with endocrinopathy, and  intracranial hypertension. Facial nerve disorders appear early and respond well to therapy. Other expansive disorders, peripheral nerve disorders and neuromuscular disorders, appear later and tend to be chronic.

Diagnostics
Diagnosis is made using CT and Nuclear Magnetic Resonance (NMR). We do not require a biopsy due to the high risk. Necessary NMR checks 3-6 months after the start of therapy. Lumbar puncture findings are non-specific. Cerebrospinal fluid with lymphocytosis and elevated protein levels in 80% of patients. There is usually an increase in ACE (50% of patients) and  CD4 / CD8 in cerebrospinal fluid.

Treatment
There are no controlled studies or clear guidelines for treatment. Systemic corticoids are added with the addition of hydroxychloroquine or azathioprine, followed by lifelong treatment. In patients with corticosteroid refractory disease (30-50% of patients), pulsed cyclophosphamide or newly described successful infliximab treatment is recommended. An exception is facial nerve palsy, which tends to spontaneous remission and whose course is not affected by corticosteroids. Therefore, mere observation is recommended in this case.

Related articles

 * Sarcoidosis
 * Sarcoidosis (pathology)
 * Sarcoidosis (internal)

Source

 * ANTON, Jan. Lecture materials "Sarkoidóza".