Théophile Alajouanine

Théophile Alajouanine (1890-1980) portrait 1

Théophile Antonin Joseph Alajouanine (1890-1980) was an

Alajouanine was a clinician, and scholar whose career bridged the disciplines of medicine, literature, and the arts. Best remembered for his contributions to the understanding of spinal vascular malformations, aphasia, and epilepsy, Alajouanine exemplified the humanist physician, integrating clinical rigour with cultural sensitivity.

He trained under Pierre Marie (1853–1940) and later joined the neurological unit of Georges Guillain (1876–1961) at the Salpêtrière, where he would remain for much of his professional life. A gifted clinician and teacher, Alajouanine developed detailed characterisations of progressive myelopathy, contributing to the description of what is now termed Foix–Alajouanine syndrome—a necrotising myelopathy due to spinal dural arteriovenous fistulas.

Alajouanine also made significant contributions to the study of aphasia and language disorders, including the influential monograph L’Aphasie et le langage pathologique. His approach combined neuroanatomical localisation with a nuanced appreciation for expressive and cognitive disruption in neurological illness, influencing generations of speech pathologists and neurolinguists.

Beyond the clinic, Alajouanine maintained a deep interest in the interface between neurology and the creative arts. He famously investigated the neurological conditions of composer Maurice Ravel and author Fyodor Dostoevsky, producing works that explored how brain disease influenced artistic expression. His later writings fused clinical experience with philosophical and literary reflection, earning him a unique place in the history of medical humanities.


Biography
  • 1890 – Born June 12 in Verneix, Allier, France.
  • 1910s – Studied medicine in Paris; interned under Pierre Marie at Bicêtre and Salpêtrière.
  • 1915 – Published first paper, co-authored with Clovis Vincent.
  • 1920 – Joined the neurological unit of Georges Guillain; also worked closely with Raymond Garcin (1897–1971).
  • 1924 – Published “Contribution à l’étude anatomique et clinique de la syringomyélie.”
  • 1935 – Appointed chef de service at Salpêtrière; remained there until retirement.
  • 1937 – Described spinal arteriovenous malformations (AVMs) with Charles Foix (1882-1927) and Dorothea Campagne—now known as Foix–Alajouanine syndrome.
  • 1939–1945 – Maintained teaching and clinical duties throughout WWII.
  • 1947 – Chairman of the Clinique des Maladies du Système Nerveux at La Salpêtrière – the fifth successor to Jean-Martin Charcot after Fulgence Raymond, Jules Dejerine, Pierre Marie and Georges Guillain
  • 1950s – Conducted pioneering studies on musicians and neurological disorders; consulted on the illnesses of Maurice Ravel and Fyodor Dostoevsky.
  • 1955 – Published on temporal lobe epilepsy and automatisms.
  • 1960s – Published essays on medicine and the humanities, including Du bon usage de la parole (1963).
  • 1967 – Retired from Salpêtrière.
  • 1948 – Co-authored Aphasie et langage with Georges-Heuyer.
  • 1977 – Final publications on language and expression in neurological illness.
  • 1980 – Died October 2 in Paris.

Medical Eponyms
Foix-Alajouanine syndrome (Syndrome de Foix-Alajouanine) 1926

Rare subacute necrotizing myelopathy, historically associated with thrombosis of spinal medullary veins, now understood to stem from congestive myelopathy due to spinal dural arteriovenous fistulas (AVFs). This progressive myelopathy leads to paraplegia, sensory disturbances, sphincter dysfunction, and eventual death if untreated. The condition affects primarily middle-aged adults, with a strong male predominance.

First described by Charles Foix and Alajouanine in 1926, it was termed “myélite nécrotique subaiguë” and believed to represent spinal cord ischaemia due to vascular occlusion. Modern neuroimaging and angiography have since clarified the underlying pathophysiology as venous hypertension resulting from dural AVFs, causing spinal cord edema and infarction—especially in the thoracolumbar region.

In contemporary neurology, the term “Foix–Alajouanine syndrome” is generally reserved for non-haemorrhagic spinal AVMs or dural AVFs that present with progressive myelopathy, often mimicking other cord pathologies such as cauda equina or MS.

1926 – Foix and Alajouanine publish La myélite nécrotique subaiguë describing two fatal cases of progressive paraplegia with spinal cord necrosis and vascular congestion.

1989Criscuolo et al. redefine the syndrome’s pathogenesis as reversible congestive myelopathy secondary to dural AVFs rather than irreversible spinal artery thrombosis.

2000s–2020s – MRI and DSA become standard for diagnosis; successful treatments via embolization or surgical ligation improve prognosis significantly when diagnosed early.

2024Atallah et al. publish a pooled analysis of 26 confirmed FAS cases, clarifying diagnostic criteria, typical imaging findings, and best outcomes with early surgical ligation.


Marie–Foix–Alajouanine syndrome (1922)

A progressive cortical cerebellar atrophy typically seen in older adults, with a strong association to chronic alcohol use. Clinically, it manifests with gait ataxia, dysarthria, intention tremor, and truncal instability — classic features of cerebellar dysfunction. Histologically, the condition involves degeneration of Purkinje cells, particularly in the anterior lobe of the cerebellum.

Originally described in 1922 by Pierre Marie, Charles Foix, and Théophile Alajouanine as l’atrophie cérébelleuse tardive à prédominance corticale, the syndrome was framed as a late-onset, predominately cortical cerebellar degeneration. While most commonly seen in alcoholics, a hereditary form was later described by Richter in 1950, suggesting an autosomal dominant inheritance in select families.

In modern neurology, the term is used less frequently, with most cases now subsumed under alcoholic cerebellar degeneration or spinocerebellar ataxias (SCAs), depending on aetiology and family history.


Guillain sign (1923)

Guillain sign — originally termed Le réflexe médio-pubien — was described in 1923 by Georges Guillain and Théophile Alajouanine. It is a bone reflex elicited by percussion over the pubic symphysis, which produces a double muscular response: (1) contraction of the abdominal muscles (especially rectus abdominis), and (2) contraction of the hip adductors (notably the pectineus and adductor longus).

The reflex was proposed as a tool for segmental spinal cord localization, particularly in the lower thoracic and upper lumbar regions. Guillain emphasized that it was absent or asymmetric in cases of hemiplegiaparaplegia, or spinal cord compression, and particularly useful in differentiating flaccid from spastic paraplegia. It also served as a proxy to explore automated medullary responses when traditional reflexes were absent.

Though once a topic of intense neurophysiological interest, the reflex is rarely used in modern practice. Its use has largely been replaced by imaging and more standardised neurological testing.


Garcin-Guillain syndrome (1927)

Garcin–Guillain syndrome, more commonly referred to as Garcin syndrome, describes the rare occurrence of unilateral cranial nerve palsies — involving most or all of the twelve cranial nerves on one side — without signs of long tract involvement or raised intracranial pressure. It typically results from infiltrative lesions at the skull base, most commonly due to lymphoepithelioma or nasopharyngeal carcinoma.

The syndrome was initially mentioned by Georges Guillain in 1926 in collaboration with Théophile Alajouanine (1890–1980) and Raymond Garcin (1897–1971). However, it was Garcin’s 1927 Paris thesis that gave the first comprehensive clinical description, hence the naming predominance as “Garcin syndrome.”

The condition reflects peripheral cranial nerve involvement, usually from extracranial or parameningeal malignancy, with sparing of the brain parenchyma. Though exceedingly rare, it remains a key diagnostic consideration in progressive cranial neuropathies with imaging evidence of skull base pathology.

1926 – Guillain, Alajouanine, and Garcin co-author a note on a new form of cranial nerve paralysis – Le syndrome paralytique unilatéral global des nerfs crâniens

1927 – Raymond Garcin publishes his landmark thesis – Le syndrome paralytique unilatéral global des nerfs craniens: contribution à l’étude des tumeurs de la base du crâne

1930s–1950s – The syndrome is further observed in association with nasopharyngeal carcinomas, including the Schmincke type, and base of skull sarcomas.

Later 20th century – Often used synonymously with cranial polyneuropathy of neoplastic origin, the eponym persists in European neurology.


Major Publications

Key Medical Contributions
Neurology and the Arts

Alajouanine had a lifelong interest in the interplay between neurology and creative expression. He investigated Maurice Ravel’s aphasia and consulted on Dostoevsky’s epilepsy, interpreting how neurological disease shaped their output. His essays combined medical insight with literary depth and contributed to the field of neuroaesthetics.

Aphasia and Language

Alajouanine explored aphasia beyond Broca and Wernicke, characterising expressive and receptive impairments through a clinical–linguistic lens. Their work influenced mid-20th-century European approaches to neurolinguistics and speech therapy.


References

Biography

Eponymous terms


Eponym

the person behind the name

BA MA (Oxon) MBChB (Edin) FACEM FFSEM. Emergency physician, Sir Charles Gairdner Hospital. Passion for rugby; medical history; medical education; and asynchronous learning #FOAMed evangelist. Co-founder and CTO of Life in the Fast lane | Eponyms | Books |

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