Neurosarcoidosis: sarcoidosis affecting the nervous system

The NHS explains that sarcoidosis is a rare condition that causes small patches of swollen tissue, called granulomas, to develop in organs of the body. It most often affects the lungs, skin and lymph nodes, but because it can affect any organ, symptoms vary widely depending on which organs are involved.

Neurosarcoidosis is sarcoidosis involving the nervous system. The clinical article on Patient.info, written for medical professionals, says the nervous system is among the less commonly affected sites after the thorax, skin and eyes, and describes nerve lesions that can affect any part of the central or peripheral nervous system, causing a huge variety of neurological disease.

The sources read for this guide describe neurosarcoidosis only briefly within general sarcoidosis information, so this page cannot give detailed figures for how often it occurs. Specialist neurology and sarcoidosis teams manage the condition.

According to the Patient.info clinical article, Bell’s palsy and lymphocytic meningitis are common manifestations of neurological involvement, and diabetes insipidus is also seen. The following symptoms are described as relatively common.

• Facial numbness and facial weakness (cranial nerve involvement).

• Difficulty swallowing, hoarseness and hearing impairment.

• Headache.

• Visual field defects, including from involvement of the optic chiasm.

• Excessive thirst and passing a lot of urine (polydipsia), linked to diabetes insipidus.

• Seizures, stroke-like events or TIA, and peripheral neuropathy.

Neurological symptoms always need assessment, whatever the cause. A GP appointment is the first step for persistent headache, double vision, facial weakness or numbness, while a seizure or a sudden change such as a drooping face, weakness or slurred speech should be treated as an emergency: call 999.

If you already have sarcoidosis, the NHS says to ask your doctor for advice if symptoms change, and that help is available for people with severe, persistent sarcoidosis, including through Sarcoidosis UK and Asthma + Lung UK.

The NHS says a number of tests may be carried out depending on which organs are affected, such as chest X-ray, CT scan, endoscopy with a biopsy of lung tissue, and scans or examinations of other organs. The Patient.info article says a diagnosis requires histological evidence of granulomatous inflammation, exclusion of alternative causes and evidence of systemic disease, and that all patients must be thoroughly assessed for additional disease manifestations because isolated involvement of a single organ is rare.

It lists blood tests including calcium and serum ACE, and a formal neurological examination when there are relevant symptoms, including checking cranial nerve function and peripheral sensory and motor nerve function. For neurosarcoidosis specifically, the sources read do not describe the imaging or spinal fluid tests used, so ask your neurology team how the diagnosis is being confirmed.

The NHS says that many people with sarcoidosis do not need treatment as it often goes away on its own, and that if treatment is recommended steroid tablets are usually used to relieve symptoms and prevent organ damage by reducing inflammation and preventing scarring. You may be given a high dose for a short time, then a low dose for months or years, with monitoring. Side effects can include weight gain, mood swings and bone weakening, and the NHS says steroids are only used when necessary. Other medicines such as immunosuppressants may be used if steroids are not enough or there are concerns about side effects.

The Patient.info article says neurosarcoidosis is relatively refractory to corticosteroids and that some authorities recommend a higher initial dose. It also describes antimetabolite drugs such as methotrexate and azathioprine as second-line agents, and anti-TNF antibodies such as infliximab and adalimumab as third-line agents in sarcoidosis generally. Treatment of neurological disease is decided by specialist teams, and doses and drug choices are individual.

The NHS suggests lifestyle measures: stop smoking if you smoke, eat a balanced diet, maintain a healthy weight, try to reduce stress, take regular exercise and get enough sleep. It says symptoms can come and go and can often be managed with over-the-counter painkillers so they do not affect everyday life, and that most people with the condition find symptoms have disappeared within a few years of diagnosis.

The NHS adds that, for some people, the condition slowly gets worse over time and they end up with organ damage. It points to the Sarcoidosis UK website, which has a list of support groups, and to Asthma + Lung UK. The NHS also says clinical teams pass information to the National Congenital Anomaly and Rare Disease Registration Service, which helps research, and you can opt out at any time.

Sarcoidosis care in the NHS may be shared between several specialists, and the nervous system is looked after by neurology teams where it is involved. The sources read do not describe the NHS pathway in detail.

Some people seek a private neurology or sarcoidosis opinion for a second view. As treatment can include powerful immunosuppressive medicines needing regular monitoring, ask who will prescribe and monitor them, and make sure your GP and specialist teams share information.