Man has unusual case of sarcoidosis affecting brain, multiple organs

[Joachim]

For a young man in Belgium, a month-long headache combined with slurred speech and walking difficulties were the result of neurosarcoidosis, or sarcoidosis affecting the nervous system, a case study shows.

The man’s neurosarcoidosis was part of a widespread form of sarcoidosis affecting multiple other organs, including the lymph nodes, lungs, and testes.

Diagnosis of this “unusual form of neurosarcoidosis” was complicated by his neurological symptoms and history of a similar episode that resolved on its own, researchers wrote.

“Overall, this case report highlights the importance of a multidisciplinary approach to the diagnosis and management of sarcoidosis,” they wrote.

The case report, “Neurosarcoidosis With Multi-Organ Involvement: A Case Report and Literature Review,” was published in the journal Cureus.

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Sarcoidosis is a disease where clusters of inflammatory cells, called granulomas, form in one or more organs, leading to damage. The lungs and lymphatic (drainage) system are most often affected, but granulomas can appear anywhere in the body.

In up to 15% of cases, these granulomas form in the brain and/or other parts of the nervous system, and the disease is then called neurosarcoidosis. This rare form of sarcoidosis can occur on its own or as part of a more widespread form of sarcoidosis.

“The presentation of neurosarcoidosis varies widely and depends on the location and extent of the involvement,” the researchers wrote.

Symptoms can appear gradually or emerge suddenly, and include headache, vision or hearing loss, cognitive problems, seizures, and stroke-like symptoms. Because these symptoms are non-specific, reaching a neurosarcoidosis diagnosis can be difficult.

Now, a team of researchers in Brussels, Belgium, described the case of 27-year-old man who developed an unusual form of neurosarcoidosis that affected multiple organs.

The man was admitted to the emergency department due to a month-long headache that had worsened in the past couple of days. He had also been experiencing slurred speech and walking problems in the previous three months.

A neurological examination revealed unequal pupil size in response to light. Motor and sensory assessments were unremarkable, and the patient showed normal reflexes.

However, tests assessing the function of his cerebellum, which is a brain region involved in motor movement regulation and balance control, revealed lack of coordination on a finger-to-nose test and gait ataxia, or an abnormal walking pattern with a widened stance. He had difficulty walking in a straight line.

His speech was slurred, suggesting speech problems resulting from cerebellar damage.

Samples of blood and cerebrospinal fluid (the liquid around the brain and spinal cord) tested negative for a panel of bacteria, viruses, and fungi, as well as for self-reactive antibodies linked to autoimmune diseases and tumor cells.

“He had a similar episode one year ago but with spontaneous remission,” the researchers wrote.

Headaches can be a potential symptom of neurosarcoidosis and may warrant further evaluation especially if other symptoms or physical findings are present.

Stroke-like symptoms indicated need for brain imaging

His “stroke-like symptoms, such as [slurred speech] and [walking] disorders, further underscored the indication of brain imaging,” the researchers wrote.

A computed tomography (CT) scan of the brain showed diffuse leptomeningeal enhancement, a non-specific imaging feature that indicates inflammation and “is associated with a more severe and progressive disease course,” the team wrote.

MRI also revealed lesions in the pons, a group of nerves that work as a connection between the brain, the cerebellum, and the spinal cord. “Involvement of the pons is considered an unusual presentation” of neurosarcoidosis, the researchers wrote.

These findings suggested most of his neurological symptoms were due to problems in the cerebellum and/or pons.

A CT scan of the chest and abdomen revealed multiple swollen lymph nodes and the presence of signs typical of sarcoidosis in the lungs. Further imaging scans revealed lesions in both testes.

These findings suggested the presence of neurosarcoidosis with multi-organ sarcoidosis.

A lymph node biopsy confirmed the presence of granulomas typically seen in sarcoidosis, which, together with no signs of infection, was “consistent with the diagnosis of sarcoidosis,” the researchers wrote.

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Patient started on immunosuppressants

The man was started on immunosuppressive treatment with corticosteroids and methotrexate. Imaging scans taken three months later showed resolution of leptomeningeal enhancement and a reduction in lymph node swelling. However, the man still showed lesions in the pons.

“With ongoing treatment, significant neurological improvement was noted, including the resolution of headaches and [slurred speech],” the researchers wrote.

However, his walking problems persisted, even though they showed signs of reduction.

“The positive outcome observed in our patient, following the combined treatment of corticosteroids and methotrexate, aligns with current recommendations for managing neurosarcoidosis,” the researchers wrote.

“However, persistent gait ataxia is a reminder of the possibility of long-term impairment, underlining the importance of rapid diagnosis and treatment,” they added.

The researchers also noted “headaches can be a potential symptom of neurosarcoidosis and may warrant further evaluation especially if other symptoms or physical findings are present.”

However, their “patient’s presentation with a combination of headaches, cerebellar [slurred speech], and gait ataxia, against the backdrop of spontaneous remission from a similar episode a year prior, heightened the complexity of the diagnosis,” the team concluded.

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