Théophile Alajouanine
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.
1989 – Criscuolo 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.
2024 – Atallah 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 hemiplegia, paraplegia, 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
- Marie P, Foix C, Alajouanine T. De l’atrophie cérébelleuse tardive à prédominance corticale.
Revue neurologique 1922; 38(7): 849-885 and 1922; 38(8): 1082-1111. [Marie-Foix-Alajouanine syndrome] - Guillain G. Alajouanine Th. Le réflexe médio-pubien. Comptes rendus des séances de la Société de biologie, Paris, 1923; 89: 874, 1215. [Guillain sign]
- Guillain G. Alajouanine Th, Garcin R. Le syndrome paralytique unilatéral global des nerfs crâniens. Bulletins et mémoires de la Société médicale des hôpitaux de Paris, 1926; 50: 456-460 [Garcin – Guillain syndrome]
- Foix C, Alajouanine T. La myélite nécrotique subaiguë (Myélite centrale angiohypertrophique à évolution progressive). Paraplégie amyotrophique lentement ascendante d’abord spasmodique, puis flasque. Revue neurologique 1926; 2: 1-42. [Foix-Alajouanine disease]
- Alajouanine T, Akerman A. Attitude de la main dans une poussée monobrachiale astéréognosique de la sclérose en plaques. Revue neurologique 1931; 1: 318–322.
- Alajouanine T, Ombredane A, Durand M. Le Syndrome De Desintegration Phonetique Dans L’aphasie. 1939
- Alajouanine T. Aphasia and artistic realization. Brain. 1948 Sep;71(Pt. 3):229-41
- Alajouanine T. Le syndrome tétanie. 1958
- Alajouanine T. Bases physiologiques et aspects cliniques de l’épilepsié. 1958
- Alajouanine T, L’Hermitte F. Les désorganisations des activités expressives du langage dans l’aphasie. L’Encéphale, 1963
- Alajouanine T. Dostoiewski’s epilepsy. Brain. 1963 Jun;86:210-8.
- Alajouanine T. L’Aphasie et le langage pathologique. 1968
- Alajouanine T. Raymond Garcin (1897-1971). Rev Neurol (Paris). 1971 Apr;124(4):257-60.
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
- Titres et Travaux scientifiques du docteur Th. Alajouanine. 1927
- Lhermitte F, Lecours AR, Signoret JL. Obituary: Théophile Alajouanine (1890-1980). Brain Lang. 1981 May;13(1):191-6.
- Boller F. Modern neuropsychology in France: Théophile Alajouanine (1890-1980). Cortex. 2006 Jan;42(1):3-4.
- Vitturi BK. Théophile Alajouanine (1890–1980). J Neurol 2025; 272: 497
- Théophile Alajouanine (1890-1980) – Toutes ses œuvres. BNF
Eponymous terms
- Richter RB. Late cortical cerebellar atrophy, a form of hereditary cerebellar ataxia. Am J Hum Genet. 1950 Mar;2(1):1-29.
- Criscuolo GR, Oldfield EH, Doppman JL. Reversible acute and subacute myelopathy in patients with dural arteriovenous fistulas. Foix-Alajouanine syndrome reconsidered. J Neurosurg. 1989 Mar;70(3):354-9.
- Bhatt N, Bhatt N. Foix-Alajouanine syndrome: a case report. Eur J Neurol. 2007 Aug;14(8):e4-5
- Ferrell AS, Tubbs RS, Acakpo-Satchivi L, Deveikis JP, Harrigan MR. Legacy and current understanding of the often-misunderstood Foix-Alajouanine syndrome. Historical vignette. J Neurosurg. 2009 Nov;111(5):902-6.
- Vitturi BK, Sanvito WL. What do Flaubert, Dostoevsky and Machado de Assis have in common with neurology? Arq Neuropsiquiatr. 2017 Dec;75(12):892-894.
- Levy G, Engelhardt E. Is the unstable ataxic hand of Alajouanine and Akerman a distinct contribution? Rev Neurol (Paris). 2021 Oct;177(8):852-858.
- Vitturi BK, Gagliardi RJ. Post-stroke aphasia in famous writers: when Neurology left geniuses speechless. Arq Neuropsiquiatr. 2021 Mar;79(3):251-253.
- Atallah O, Almealawy YF, Arian R, Dwebi A, Badary A, Abdul Hussein AF, Sanker V, Zafarshamspour S, Chaurasia B, Agrawal A, Pastrana Brandes S, Azab MA. Foix-Alajouanine syndrome: a comprehensive overview of rare but relevant diagnosis. Ann Med Surg (Lond). 2024 Sep 30;86(11):6636-6644.
Eponym
the person behind the name