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Archive image Neurosciences and History 2014; 2(2):79-83

José Ramón Ricoy, neuropathologist

M. BalcellsDepartment of Neurology. Hospital Universitari del Sagrat Cor, Barcelona, Spain.

ABSTRACT

José Ramón Ricoy, who studied under Ludo van Bogaert at the Bunge Institute in Antwerp, is a very influentialprofessor who has trained specialists in general anatomical pathology and neuropathology throughout hisprofessional career. He has published more than 130 scientific papers in both national and international journals. Stellar contributionsinclude his 1974 doctoral thesis on orthochromatic (or sudanophilic) leukodystrophies. ey also include hisstudies of other leukodystrophies, Pelizaeus-Merzbacher disease, mitochondrial behaviour in myopathies, andCSF and serum studies in patients with subacute sclerosing panencephalitis. Additional examples are hishistochemical and ultrastructural studies of striated muscle fibres in Prader-Willi syndrome, the case series ofneuronal ceroid lipofuscinosis or Batten disease, and his clinical and neuropathological examination of progressivemyoclonus epilepsy of Unverricht-Lundborg. I should also mention his contribution to the study of toxic oilsyndrome, a neurological disease of great social concern that struck Spain in 1981.is article also presents the anatomical pathology collection which the doctor recently donated to the SEN'shistorical archive. is collection consists of 56 histological slides illustrating various nervous system diseases. Itis particularly interesting from a didactic point of view since it showcases rare diseases and an excellent slidepreparation technique. Key examples are the slides presenting Huntington disease, tuberous sclerosis, andBinswanger disease because their image quality is especially sharp.

KEYWORDSJosé Ramón Ricoy, neuropathology, Huntington disease, tuberous sclerosis, Bourneville disease, Binswangerdisease

myoclonic variant of non-ganglioside familial amauroticidiocy according to its clinical and pathological features.Based on cellular neuropathological findings, the authorshighlight the selective localisation and nature of build-upin neurons.1

Ricoy returned to Spain and was hired as an associateprofessor of anatomical pathology by UniversidadAutónoma, Madrid, in 1973, and in 1976, by UniversidadComplutense. He held these positions until 1974 and1984, respectively.In 1985, he was made a senior lecturer in pathology atUniversidad Complutense, Madrid, later becoming thechair of anatomical pathology at that university’s Facultyof Medicine.

Corresponding author: Dr Miquel Balcells RibaE-mail: balcellsriba@gmail.com © 2014 Sociedad Española de Neurología

Introduction

José Ramón Ricoy Campo was born in 1942 in AsErmidas, Ourense province. He attended medical schoolin Santiago de Compostela and graduated in 1966.Neuropathology was his calling from an early age. A yearaer earning his medical degree he began working at theBunge Institute in Antwerp, where he was trained in thatspecialty by Ludo van Bogaert in the three years from1967 to 1970.

He rapidly acquired a solid background, which isexemplified by his study, with J.H. Bruens as co-author,of a case of familial amaurotic idiocy. e conclusions ofthis article address the challenges in characterising this

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Lines of research

Dr Ricoy Campo has presented more than 30 oralcommunications on a wide variety of diseases at theannual meetings held by the Spanish Society ofNeurology (SEN). His contributions includedescriptions of leukodystrophies, Pelizaeus-Merzbacher disease, mitochondrial behaviour inmyopathies, and CSF and serum studies in patientswith subacute sclerosing panencephalitis. We also findhis histochemical and ultrastructural studies of striatedmuscle fibres in Prader-Willi syndrome, the study ofseven cases of neuronal ceroid lipofuscinoses or Battendisease, and his clinical and neuropathological study oftwo patients with the Unverricht-Lundborg form ofprogressive myoclonus epilepsy.

Special mention should be made of his first oralpresentation, given with A. Vázquez and G. Moya at the1969 SEN Annual Meeting, on the neurological aspectsof hypoglycaemic comas. Here, a neuropathology studyof two autopsy cases revealed mild neural loss withhomogenising necrosis and diffuse demyelination, withno accompanying glial reaction.2

In the same meeting, he also presented his own studyof the description of the myoclonic variant of familialamaurotic idiocy. He concluded that this variant wassimply a less severe form of the histopathologicalprocess that takes place in diseases of this type.3

At the 1970 annual meeting, Ricoy was the lead authorand presenter of a comparative study of theultrastructure of striated muscle fibres in two motorneuron diseases: amyotrophic lateral sclerosis (ALS)and Wohlfart-Kugelberg-Welander (WKW). Here, hepresented the results from a study of striated muscle inten cases of ALS and in one patient with WKW. Theconclusions from this study are that in ALS, theobserved myofibrillar alterations are secondary to Z-band changes that indicate denervation. In WKWdisease, the entire myofibrillar complex becomesdisorganised, whereas Z-band changes are secondaryand manifest differently.4

In 1971, he co-authored a case study of McArdledisease (glycogenesis V). The authors believed that thiscase study was probably the first one published in theworld, and they concluded that in addition to thecharacteristic alterations in glycogen storage, there wasalso an active myopathic process.5

Dr Ricoy has published more than 130 scientific papersin Spanish and international journals. His remarkabledoctoral thesis, defended in 1974, addressesorthochromatic (sudanophilic) leukodystrophies.6 Thisthesis reviews the concept of diffuse sclerosis anddistinguishes three subtypes: myelinoclastic diffusesclerosis (Schilder’s disease), van Bogaert encephalitis,and the leukodystrophies. Within this last category,only the orthochromatic or sudanophilic varieties areconsidered true leukodystrophies. Ricoy studied theseentities using the classification system set out by Peiffer,and proposed 2 types: pure forms and special forms(Pelizaeus-Merzbacher disease and variants).

The pure forms include cases in which the underlyingdisease consists of a change in the endoplasmicreticular system of oligodendroglia. This gives rise toalterations in the makeup or maintenance of myelin. Inspecial forms, however, microscope imaging revealspatchy demyelination, limited axonal lesions, and ascarce or absent astroglial and sudanophilic reaction.Remarkable ultrastructure findings in these forms areabsence of normal myelin, degeneration of myelinsheaths, spiral wrapping in periaxonal membrane,limited axonal degeneration, oligodendrocyte deficit,and swollen astrocytes poor in glial filaments andcontaining lipid bodies.

I should also point out his contributions to the studyof toxic oil syndrome, a neurological disease of greatsocietal concern that struck in Spain in 1981. Itaffected approximately 20 000 people and caused 330deaths. The study published in Brain in 1983distinguishes between lesions in muscle tissue,peripheral nerves, and the central nervous system.7 Italso describes two different and time-dependent typesof muscle system lesions. Patients initially presentedmyalgia and inflammatory infiltration in theperimysium, the muscle spindle capsules, and theintramuscular nerves. Muscle tissue displayedelectron-dense material similar to Z-bands.Observations at later stages of the disease also includedpronounced neurogenic amyotrophy with endomysialfibrosis. The onset of denervation atrophy is secondaryto impairment of peripheral nerves, which is one of thedistinguishing features of this disease.

The most obvious lesion in this syndrome wasimpairment of the peripheral nerves; researchersobserved perineuritis and perineural fibrosis as typicalfeatures in toxic oil syndrome.

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The central nervous system consistently displayedlesions in cells of the anterior horns, and in the nucleiof cranial nerves and reticular neurons. Astrocytes ofthe brainstem showed hypertrophy and abnormalnuclei. The study also found areas of chromatolysis inlocations with microglial proliferation.

The adulterated oil had a high linoleic acid content.This led to an excess of arachidonic acid, which mayhave played a role in lesion pathogenesis.

In addition to his clinical, diagnostic, and researchcontributions, José Ramón Ricoy was outstanding as aprofessor. He trained specialists in general anatomicalpathology and neuropathology during his career as a

neuropathologist at several hospitals (Gran Hospital delEstado [1970-1973], Clínica Puerta de Hierro [1973-1976], and Hospital Universitario 12 de Octubre from1985 until his retirement).

Between 1983 and 1996, he developed his considerableabilities as an administrator and manager whiledirecting Hospital Universitario 12 de Octubre. Heserved as the Deputy Director General of Spain’sNational Institute of Health, and also directed theSpanish health research fund (Fondo de InvestigacionesSanitarias), the research and education managementstrategy (Ordenación de la Investigación y Formación),and Institute of Health Carlos III.

Figure 2. Tuberous sclerosis or Bourneville disease Figure 1. Huntington Disease

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Anatomical pathology collection

José Ramón Ricoy has donated a collection of 56histological slides illustrating different pathologicalfindings in the nervous system to the SEN historicalarchive.

Figure 3. Binswanger disease

This collection is particularly interesting from a didacticpoint of view, since it showcases rare diseases and thetechnique used to prepare the slides is excellent.

Key examples are the slides presenting Huntingtondisease, tuberous sclerosis, and Binswanger diseasebecause the quality of the images that illustrate these rarediseases is especially sharp.

— Huntington disease (Figure 1): the coronal sectionshows diffuse cortical atrophy and pronouncedatrophy of the caudate nucleus of the globus pallidusand putamen. Atrophy of the basal ganglia results inan increase in ventricle size.

— Tuberous sclerosis or Bourneville disease (Figure 2):this coronal section of the brain exhibits glial nodulesarranged in rows. Some invade the ventricular spacesand they also form multiple hardened areas of thecerebral cortex. Nodules are formed by very largesubpial astrocytes and enlarged neurons. Tumoursevolve from these nodules, and some displaycharacteristics of spongioblastomas. The diseaseprofile also includes presence of sebacious adenomas,rhabdomyomas in the myocardium, and multiple renalcysts in the fetus.

— Binswanger disease (Figure 3): coronal sectionshowing foci of myelin destruction in the white matterof the temporal lobe and widening of the horns; bothprocesses are secondary to white matter atrophy. Thecortex and subcortical fibres remain largely intact.

Acknowledgements

My sincere thanks go to José Ramón Ricoy Campo formaking such a generous contribution to the SENhistorical archive.

References

1. Ricoy-Campo JR, Bruens JH. Contribution critique à l’étude dela variante dite myoclonique des idioties amaurotiques fami-liales non gangliosidiques. J Neurol Sci. 1970;10(5):435-56.

2. Vázquez A, Moya G, Ricoy JR. Aspectos neurológicos de loscomas hipoglicémicos; comas hipoglucémicos de origenmédico por uso de hipoglucemiantes orales. Oral commu-nication presented at the XXI Annual Meeting of the SpanishSociety of Neurology; 12-13 Dec 1969; Barcelona, Spain.

3. Ricoy JR. Sobre la individualización de la variante miocló-nica de las idioteces familiares amauróticas. Oral communi-cation presented at the XXI Annual Meeting of the SpanishSociety of Neurology; 12-13 Dec 1969; Barcelona, Spain.

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4. Ricoy JR, Díaz-Flores L. Estudio comparativo de la ultraes-tructura de la fibra muscular estriada en dos enfermedadesde neurona motora: la esclerosis lateral amiotrófica y laenfermedad de Wolfart-Kugelberg-Welander. Oral commu-nication presented at the XXII Annual Meeting of theSpanish Society of Neurology; 11-12 Dec 1970; Barcelona,Spain.

5. Rodríguez de Castro A, Ricoy JR, Díaz-Flores L, CamuñezA, Lafuente G. Un caso de miopatía de McArdle (glucoge-

nosis V). Oral communication presented at the 23rd AnnualMeeting of the Spanish Society of Neurology; 9-11 Dec 1971;Barcelona, Spain.

6. Ricoy JR. Leucodistrofias ortocromáticas o sudanófilasverdaderas [doctoral thesis]. Madrid: Universidad Autó-noma de Madrid, Faculty of Medicine; 1974.

7. Ricoy JR, Cabello A, Rodriguez J, Téllez I. Neuropathologicalstudies on the toxic syndrome related to adulterated rape-seed oil in Spain. Brain. 1983;106(4):817-35.