Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.
A 71-year-old Caucasian woman had a 1-month history of imbalance, forgetfulness and disinhibition. She had been previously well and was a recently retired linguistics academic, but had recently neglected self-care and had become housebound. On examination, there was mild right upper limb dysmetria and sway on the Romberg’s test. Neuropsychological testing identified marked deficits in executive function, recall and tasks requiring new verbal and visual learning.
Laboratory investigations showed mild neutrophilic leucocytosis (white cell count 10.9×109/L), hypoalbuminaemia (30 g/L) and elevated serum gamma-glutamyl transferase (133 U/L). Chest X-ray was normal. Serum antineuronal antibodies were negative but serum anti-GABA-B antibodies were positive. Cerebrospinal fluid (CSF) white cell count was 8x109/µL (≤5) and red cell count was 19x1012/µL (0) (presumed traumatic tap), but with normal protein (0.43 g/L) and glucose (3.4 mmol/L). Oligoclonal bands were detected as was an IgG lambda paraprotein, too small to quantify. The serum free light chain kappa/lambda ratio was 1.23 (normal). …
Contributors SD and RP contributed equally to this paper.Conception of report: SD, RP, HB and EA. Drafting of manuscript: SD, RP, HB and AC. Contribution of relevant expertise for inclusion in manuscript: HB, MF, SJ, RA, ZT, MH, EA and AC. Critical review of manuscript for intellectual content: SD, RP, HB, MF, MH and EA. All listed authors were involved in the care of the patient listed.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned. Externally peer reviewed by Johann Selvarajah, Glasgow, UK.
Read the full text or download the PDF:
Other content recommended for you
- An unusual case of interstitial lung disease in a patient with cardiopulmonary syndrome as the initial presentation of Erdheim-Chester disease
- Peritoneal or mesenteric tumours revealing histiocytosis
- Erdheim-Chester disease: a rare cause of bilateral renal artery stenosis, mimicking large vessel vasculitis
- 4CPS-122 Efficacy and safety of cobimetinib used in monotherapy for erdheim–chester disease
- Efficacy of infliximab in the treatment of Erdheim-Chester disease
- The multifaceted clinical presentations and manifestations of Erdheim–Chester disease: comprehensive review of the literature and of 10 new cases
- Erdheim-Chester disease: an unusual aetiology of bilateral lipomatous perinephric masses
- Atypical form of non-Langerhans histiocytosis with disseminated brain and leptomeningeal lesions
- Erdheim-Chester disease with Rosai-Dorfman-like lesions: treatment with methotrexate, anakinra and upadacitinib
- Erdheim-Chester disease with isolated craniocerebral involvement