Bing-Neel Syndrome

Classification according to ICD-10
C88.00	Bing von Neel Syndrome
ICD-10 online (WHO version 2019)

The Bing-Neel syndrome (also: Bing-von-Neel syndrome ) describes variable neurological symptoms that can be traced back to the infiltration of the central nervous system by malignant B lymphocytes in Waldenström's disease . Neurological complications occur in approximately 25% of patients with Waldenström's disease, but these affect almost exclusively the peripheral nervous system . The central nervous system is mostly affected by the hyperviscosity of the blood, caused by the IgM immunoglobulins secreted by the B lymphocytes , in the form of headaches , dizziness , somnolence and epileptic seizures . However, only the direct infiltration of the brain and spinal cord by the abnormal B lymphocytes and the deposition of IgM immunoglobulins is referred to as Bing-Neel syndrome .

The disease was first described in 1936 by the Danish internist Jens Bing and the Danish psychiatrist Axel von Neel and later named after them.

Epidemiology

More precise data on the incidence of the disease are not available. Bing-Neel syndrome is a very rare central nervous variant of Waldenström's disease, the incidence of which is given as around one new disease per 100,000 inhabitants per year.

Symptoms

The symptoms are diverse and range from focal neurological deficits ( paresis , increased muscle reflexes ) to epileptic seizures and non-focal deficits ( memory disorders , personality changes, changes in vigilance ).

diagnosis

Diagnosis is based on evidence of intrathecal IgM production. If this does not succeed, the diagnosis can be made using a stereotactic biopsy . The magnetic resonance imaging may provide clues to the existence of an involvement of the brain and spinal cord in Waldenstrom.

therapy

Therapy for Bing-Neel syndrome remains a challenge. Previous attempts at therapy using chemotherapy , radiation therapy and neurosurgery have been carried out with varying degrees of success.

Individual evidence

↑ ^a ^b L Garderet et al .: 'Indolent' Waldenstrom's macroglobulinemia and a cerebrospinal fluid protein level of 16 g / L. In: Eur J Haematol. 2006; 77, pp. 80-82.
^ J. Bing, A. Neel: Two cases of hyperglobulinemia with affection of the central nervous system on a toxi-infection basis. In: Acta Med Scand. 1936; 88, pp. 492-506.
↑ GD Leschziner et al .: Nineteen-year Follow-up of Waldenström's Associated Neuropathy and Bing-Neel Syndrome. In: Muscle Nerve. 2009; 39, pp. 95-100.
↑ J. Drappatz et al .: Imaging of Bing-Neel syndrome. In: Neurology . 2008; 70 (16), p. 1364.
↑ J. Delgado et al .: Radiation therapy and combination of cladribine, cyclophosphamide, and prednisone as treatment of Bing-Neel syndrome: case report and review of the literature. In: Am J Hematol . 2002; 69, pp. 127-131.

[Garderet-1] L Garderet et al .: 'Indolent' Waldenstrom's macroglobulinemia and a cerebrospinal fluid protein level of 16 g / L. In: Eur J Haematol. 2006; 77, pp. 80-82.

[2] J. Bing, A. Neel: Two cases of hyperglobulinemia with affection of the central nervous system on a toxi-infection basis. In: Acta Med Scand. 1936; 88, pp. 492-506.

[3] GD Leschziner et al .: Nineteen-year Follow-up of Waldenström's Associated Neuropathy and Bing-Neel Syndrome. In: Muscle Nerve. 2009; 39, pp. 95-100.

[4] J. Drappatz et al .: Imaging of Bing-Neel syndrome. In: Neurology . 2008; 70 (16), p. 1364.

[5] J. Delgado et al .: Radiation therapy and combination of cladribine, cyclophosphamide, and prednisone as treatment of Bing-Neel syndrome: case report and review of the literature. In: Am J Hematol . 2002; 69, pp. 127-131.