The case of the Marquis de Causan (1804): an early account of visual loss associated with spinal cord inflammation

Abstract

The recent discovery of disease specific and pathogenic autoantibodies in neuromyelitis optica (NMO, Devic's disease) has revived the interest in this intriguing yet often devastating condition. While the history of classic multiple sclerosis has been studied extensively, only very little is known so far about the early history of NMO. Here we discuss a now forgotten report by the famous French anatomist and pathologist Antoine Portal (1742-1832), first physician to Louis XVIII and founding and lifelong president of the Académie Nationale de Médecine. Portal's report, which fascinated some of the most renowned 19th century pioneers in the field of neurology but fell into oblivion later, represents the first account of visual loss in a patient with spinal cord inflammation but no brain pathology in the Western literature known so far--published more than 60 years prior to Thomas Clifford Allbutt's much cited note on a patient with myelitis and a "sympathetic eye disorder".

Finding NMO: The Evolving Diagnostic Criteria of Neuromyelitis Optica

“…The first clinical account of presumed NMO is often attributed to Sir Clifford Allbutt, a pioneering physician who promoted the adoption of the direct ophthalmoscope in clinical practice (3). However, even before Albutt’s seminal publication, “On the Ophthalmoscopic Signs of Spinal Disease,” clinicopathologic reports of individuals with concurrent vision loss and myelitis by Antoine Portal in 1804, Giovanni Pescetto in 1844, and Jacob Clarke in 1865 likely represent the earliest accounts of NMO in the literature (4). The term “neuro-myélite optique aiguë” was originally coined in 1894 by Eugene Devic and Fernand Gault when they presented a case of concurrent ON and TM and reviewed 16 additional cases from the literature (5).…”

Evaluation of aquaporin‐4 antibody assays

“…1,2 Almost a century later, Eugène Devic and his student, Fernand Gault, reported one case and reviewed 16 patients reported in the medical literature that presented with optic neuritis (ON) and myelitis, and coined the term “neuromyélite optique aigue” based on the clinical phenotype. 3 However, the most significant discovery in the understanding of this disease occurred in the early 2000s, when Lennon and colleagues at the Mayo Clinic identified an antibody in patients with neuromyelitis optica (NMO) that binds to the water channel called aquaporin-4 (AQP4).…”

The history of neuromyelitis optica

“…26 years prior to the first case referenced by Devic and Gault, the Genoese physician Giovanni Battista Pescetto (1806–1884) had reported on a 42-year-old man who simultaneously developed acute amaurosis and cervical myelitis, with complete recovery following extensive bloodletting [10]; in 1850, the British physician Christopher Mercer Durrant (1814–1901) had described a case of tetraparesis and (partly reversible) bilateral amaurosis in the precursor of the British Medical Journal [20]; and in 1862 the British neuroanatomist, neuropathologist, and neurologist Jacob Augustus Lockhart Clarke (1817–1880), known to many as the eponym of Clarke’s column, the posterior thoracic nucleus, had reported the case of a 17-year-old girl with bilateral optic neuritis and longitudinally extensive transverse myelitis in The Lancet [15]. Finally, a report by Antoine Portal (1742–1832), first physician to Louis XVIII and founding and lifelong president of the Académie Nationale de Médecine, deserves to be mentioned here: it represents the first account of visual loss in a patient with spinal cord inflammation but no brain pathology in the Western literature known so far [16]. However, none of these previous authors had ever used the term ‘neuromyelitis optica’ or a similar one.…”