gms | German Medical Science

Introduction: Anti-IgLON5 disease was described in 2014. It is caused by auto-antibodies against IgLON5, a neural cell adhesion molecule of unknown function. The disease is characterized by a complex sleep disorder including abnormal behaviors and vocalization during non-REM and REM sleep, gait instability, bulbar symptoms such as stridor, dysphagia or central hypoventilation. These symptoms can develop over months to years.

In general, 4 clinical types can be distinguished according to predominant symptoms:

1.

prominent sleep disorder

2.

progressive bulbar dysfunction

3.

gait instability with abnormal eye movements reminiscent

4.

and cognitive deterioration, associated with chorea

Methods: We describe the case of a 58-year-old man, who was admitted to our clinic for respiratory failure due to bilateral vocal cord paralysis in September 2017.

In the past 2 years he was admitted to the hospital several times for the same reason and was diagnosed with vocal cord dysfunction. Besides that, he was diagnosed with arterial hypertension, obstructive sleep apnea and arterial hypertension before.

Results: Clinical examination showed bilateral vocal cord paralysis and the patient was transferred to the operating room for permanent tracheostomy. Further imaging (CT neurocranium/neck/thorax and MRI of the neurocranium) showed no pathological findings that could explain the bilateral paralysis.

Analysis of blood and cerebrospinal fluid revealed the presence antibodies against IgLON5. In the beginning, neurological examination and polysomnography showed no pathological findings. However, 2 months later the patient developed oculomotoric disorders, dysphagia, central hypoventilation and REM-sleep-disorders. He was subsequently diagnosed with bulbar anti-IgLON5 encephalopathy.

The patient received corticoids and immunoadsorption in November 2017. BIPAP ventilation was started to treat the hypoventilation. In addition to this, he received Rituximab 1000 mg every 6 months. These measures led to a significant improvement of the neurological symptoms.

Discussion: Vocal cord dysfunction should be seen as a diagnosis of exclusion. In cases of patients with multiple admissions to hospital due to bilateral vocal cord paralysis, further investigation and other possible diagnoses should be taken into consideration.

A bilateral vocal cord paralysis has to be treated immediately. Imaging and laryngoscopy are obligatory. Furthermore, neurological examination can point towards the correct diagnosis. In addition to this, analysis of blood as well as cerebrospinal fluid are important to confirm diagnosis, especially since the results of a neurological exam could be false negative.