[Sleep-related movement and behavioural disorders in adults]. / Trastornos del movimiento y de la conducta durante el sueño en el adulto.

Sleep-related movement and behaviour disorders may have an impact on sleep quality and lead to daytime symptoms. These groups of conditions include diseases such as restless legs syndrome, periodic leg movements, and REM and NREM parasomnias. The knowledge of their clinical features and management is of utmost importance for the neurologist and sleep specialist. Frequently, these patients are referred to such specialists and it is relevant to know that certain sleep disorders may be associated with other neurological conditions.

TITLE: Trastornos del movimiento y de la conducta durante el sueño en el adulto.Los trastornos del movimiento y de la conducta durante el sueño pueden tener un impacto en la calidad del sueño del paciente y dar lugar a síntomas diurnos. En estos grupos de enfermedades se incluyen entidades como el síndrome de piernas inquietas, los movimientos periódicos de las piernas y las parasomnias del sueño de movimientos oculares rápidos (REM) y no REM. El conocimiento de sus características clínicas y nociones sobre su manejo es de gran importancia para el neurólogo y especialista en sueño por su frecuencia e impacto en la calidad del sujeto. Con frecuencia, estos pacientes son referidos a dichos especialistas, y es relevante conocer que ciertos trastornos del sueño pueden asociarse a otras enfermedades neurológicas.

[Zolpidem-induced sleep-related behavioural disorders]. / Conductas complejas durante el sueño inducidas por zolpidem.

OBJECTIVE: To present five patients with zolpidem-induced sleep-related behavioural disorders. METHODS: Evaluation using a questionnaire designed to study sleep behaviours and past medical history in all patients. RESULTS: The patients performed complex actions while sleep-walking (telephoning, house-cleaning, feeding the dog or waxing their legs). Inappropriate feeding behaviour with excessive food intake during the night were reported by all patients. All had weight gain, which in one patient led to extreme obesity. Two patients suffered injuries (knife cuts and burns) related to attempting to prepare food. One patient took a laxative. CONCLUSION: Withdrawal of zolpidem resolved the behaviours in all cases, highlighting the importance of an adequate diagnosis of this side effect.

Conductas complejas durante el sueño inducidas por zolpidem / Zolpidem-induced sleep-related behavioural disorders

Objetivo: Presentar cinco pacientes con trastornos conductuales durante el sueño, sin conciencia posterior de los mismos, inducidos por zolpidem.Métodos: Evaluación con un cuestionario diseñado para estudiar las características y las conductas realizadas durante el sueño por todos los pacientes. Resultados:Las conductas realizadas por los pacientes fueron muy variadas (telefonear, limpieza y labores del hogar, alimentar al perro o depilarse), aunque en todos los pacientes se recogieron conductas alimentarias inapropiadas. Todos sufrieron una ganancia ponderal importante, debido a su preferencia por alimentos hipercalóricos durante los episodios, que en una de las pacientes desembocó en una obesidad extrema. Dos de los pacientes sufrieron lesiones (cortes con cuchillo y quemaduras) en relación con el intento de elaboración de platos. Un paciente llegó a ingerir un laxante.Conclusiones: La retirada de zolpidem resolvió de forma inmediata las conductas anormales durante el sueño en todos los casos. Esto subraya la importancia de diagnosticar adecuadamente este efecto secundario (AU)

Objective: To present five patients with zolpidem-induced sleep-related behavioural disorders. Methods:Evaluation using a questionnaire designed to study sleep behaviours and past medical history in all patients. Results: The patients performed complex actions while sleep-walking (telephoning, house-cleaning, feeding the dog or waxing their legs). Inappropriate feeding behaviour with excessive food intake during the night were reported by all patients. All had weight gain, which in one patient led to extreme obesity. Two patients suffered injuries (knife cuts and burns) related to attempting to prepare food. One patient took a laxative. Conclusion: Withdrawal of zolpidem resolved the behaviours in all cases, highlighting the importance of an adequate diagnosis of this side effect (AU)

Parálisis del hipogloso como forma de presentación de una disección espontánea de múltiples arterias cervicales / Hypoglossal paralysis as a form of presentation of a spontaneous multiple cervical artery dissection

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Apolipoproteína e4 en la demencia con cuerpos de Lewy / Apolipoprotein e4 in dementia with Lewy bodies

Introducci¨®n. Existe una fuerte asociaci¨®n entre el alelo¦Å4 de la apolipoprote¨ªna E (APOE) y enfermedad de Alzheimer(EA), constituyendo este alelo un factor de riesgopara padecer esta enfermedad. Su asociaci¨®n con la demenciacon cuerpos de Lewy (DCL) es objeto de controversia. Enla DCL la presencia de APOE4 se ha relacionado con unamayor carga de placas seniles y degeneraci¨®n neurofibrilar.M¨¦todo. Estudio de casos y controles en el que se determin¨®el genotipo APOE mediante la t¨¦cnica de reacci¨®nen cadena de la polimerasa (PCR) modificada de Wenhamen 306 pacientes diagnosticados de EA probable, criteriosNINCDS-ADRDA, 58 casos de DCL probable, criterios de consensode McKeith et al. (1996), todos ellos con SPECT con 123IFP-CIT patol¨®gico, y 80 controles normales (CN) de edad y distribuci¨®npor sexos similares.Resultados. La frecuencia de alelos fue la siguiente:DCL ¦Å4: 16%; ¦Å3: 75%; ¦Å2: 9%; EA ¦Å4: 32%; ¦Å3: 67%; ¦Å2:1%; CN ¦Å4: 12%; ¦Å3: 83%; ¦Å2: 5%. En los tres grupos la distribuci¨®nde alelos en ambos sexos fue similar.Conclusiones. En la DCL la frecuencia de ¦Å4 (16%) esmuy inferior a la de la EA (32%) y muy pr¨®xima a la cifra delos CN (12%). Teniendo en cuenta que la presencia de alteracionesmorfopatol¨®gicas tipo Alzheimer en la DCL, fundamentalmentedegeneraci¨®n neurofibrilar, se correlacionainversamente con la presencia de signos parkinsonianos, esposible que este grupo represente a las formas puras de laenfermedad, aunque la falta de comprobaci¨®n neuropatol¨®gicano permite confirmar esta hip¨®tesis (AU)

Introduction. There is a strong association betweenthe ¦Å4 allele of apolipoprotein E (APOE) and Alzheimer¡¯sdisease (AD). This converts this allele into a risk factorfor the development of AD. The association between APOE4and dementia with Lewy bodies (DLB) is under discussion.In DLB, the presence of APOE4 has been related with a greateramount of senile plaques and neurofibrillary tangles.Method. This is a case-control study in which the APOEgenotype was determined using the modified PCR techniqueof Wenham in 306 patients with diagnosis of probably AD,NINCDS-ADRDA criteria, 58 cases of probably DLB, McKeithet al. consensus criteria (1996), all of them with SPECT withpathological 123I-FP-CIT and 80 normal controls (NC) havingsimilar age and gender distribution.Results. The frequency of alleles was: DLB group ¦Å4:16%; ¦Å3: 75%; ¦Å2: 9%; AD: ¦Å4: 32%; ¦Å3: 67%; ¦Å2: 1%;and in the normal control group: ¦Å4: 12%; ¦Å3: 83%; ¦Å2:5%. The percentage of alleles in both genders was similarin the three groups.Conclusions. APOE4 percentage in DLB group (16%)was lower than in AD group (32 %), and similar to thecontrol group (12 %). Considering that the presence ofmorphopathological Alzheimer type alterations in DBL,essentially neurofibrillary tangles, is inversely correlatedwith the presence of Parkinsonian signs, this group mayrepresent pure forms of the disease, although the lack ofneuropathological demonstration does not make it possibleto confirm this hypothesis (AU)

[Apolipoprotein E4 in dementia with Lewy bodies]. / Apolipoproteína e4 en la demencia con cuerpos de Lewy.

INTRODUCTION: There is a strong association between the e4 allele of apolipoprotein E (APOE) and Alzheimer's disease (AD). This converts this allele into a risk factor for the development of AD. The association between APOE4 and dementia with Lewy bodies (DLB) is under discussion. In DLB, the presence of APOE4 has been related with a greater amount of senile plaques and neurofibrillary tangles. METHOD: This is a case-control study in which the APOE genotype was determined using the modified PCR technique of Wenham in 306 patients with diagnosis of probably AD, NINCDS-ADRDA criteria, 58 cases of probably DLB, McKeith et al. consensus criteria (1996), all of them with SPECT with pathological 123I-FP-CIT and 80 normal controls (NC) having similar age and gender distribution. RESULTS: The frequency of alleles was: DLB group epsilon4: 16%; epsilon3: 75%; epsilon2: 9%; AD: epsilon4: 32%; epsilon3: 67%; epsilon2: 1%; and in the normal control group: epsilon4: 12%; epsilon3: 83%; epsilon2: 5%. The percentage of alleles in both genders was similar in the three groups. CONCLUSIONS: APOE4 percentage in DLB group (16%) was lower than in AD group (32%), and similar to the control group (12%). Considering that the presence of morphopathological Alzheimer type alterations in DBL, essentially neurofibrillary tangles, is inversely correlated with the presence of Parkinsonian signs, this group may represent pure forms of the disease, although the lack of neuropathological demonstration does not make it possible to confirm this hypothesis.

[Chronic relapsing inflammatory optic neuropathy (CRION) without detection of IgG-NMO antibodies]. / Neuropatía óptica inflamatoria recurrente crónica (CRION) sin detección de anticuerpos IgG-NMO.

Chronic relapsing inflammatory optic neuropathy is a disease that should be distinguished from demyelinating optic neuropathy (ON). Long-term immunosuppressive therapy is usually necessary and either an inflammatory granulomatous origin or a relationship with neuromyelitis optica (NMO) is hypothesized. We report a 43-year-old man who suffered from several ON episodes affecting one eye or the other. These ON attacks completely disappeared with steroid therapy but relapsed after its withdrawal. IgG-NMO antibodies were negative.

Neuropatía óptica inflamatoria recurrente crónica (CRION) sin detección de anticuerpos IgG-NMO / Chronic relapsing inflammatory optic neuropathy (CRION) without detection of IgG-NMO antibodies

La neuropatía óptica inflamatoria recurrente crónica (CRION) es una entidad que debe ser diferenciada de la neuropatía óptica (NO) desmielinizante dada la necesidad de mantener un tratamiento inmunodepresor prolongado y de la que se especula un origen inflamatorio granulomatoso o relacionado con el espectro clínico de la neuromielitis óptica (NMO). Presentamos el caso de un hombre de 43 años con varios episodios de NO, afectando a uno u otro ojo, que remitían de forma rápida al instaurar tratamiento esteroideo y recurrían tras la retirada del mismo, en el que no se detectaron anticuerpos IgG-NMO

Chronic relapsing inflammatory optic neuropathy is a disease that should be distinguished from demyelinating optic neuropathy (ON). Long-term immunosuppressive therapy is usually necessary and either an inflammatory granulomatous origin or a relationship with neuromyelitis optica (NMO) is hypothesized. We report a 43-year-old man who suffered from several ON episodes affecting one eye or the other. These ON attacks completely disappeared with steroid therapy but relapsed after its withdrawal. IgG-NMO antibodies were negative

[Multiple myeloma as a treatable cause of stroke: clinical case and review of the literature]. / El mieloma múltiple como causa tratable de ictus. Caso clínico y revisión de la literatura.

Monoclonal and polyclonal immunoglobulinemia, including lymphoma, Waldenström's macroglobulinemia and less commonly multiple myeloma (MM), are considered as infrequent causes of ischemic stroke. Hyperviscosity states, as well as procoagulant disturbances, both potentially treatable, have been implicated in its etiopathogenesis. The so-called "hyperviscosity syndrome" is a clinical syndrome consisting of headache, visual, auditory and vestibular disturbances, confusion and decreased level of consciousness, and is caused by extreme hypervolemia with a high degree of erythrocyte aggregation caused by paraproteinemia. However, in addition to this global cerebral ischemia syndrome, increase in blood viscosity (BV) can also be a cause of focal ischemia. We report a case of a patient diagnosed with IgG type MM, who suffered multiple vertebrobasilar transient ischemic attacks and minor ischemic strokes concurrent with a reactivation of his hematological disease. He became completely asymptomatic after specific treatment with dexamethasone of the paraproteinemia associated with MM. We discuss its pathophysiology in this report.

El mieloma múltiple como causa tratable de ictus. Caso clínico y revisión de la literatura / Multiple myeloma as a treatable cause of stroke. Clinical case and review of the literature

Las inmunoglobulinemias mono y policlonales, incluyendo el linfoma, la macroglobulinemia de Waldenström y, menos comúnmente, el mieloma múltiple (MM), se consideran causas inhabituales de ictus isquémicos. En su etiopatogenia se han implicado tanto estados de hiperviscosidad como alteraciones procoagulantes, ambas potencialmente tratables. El llamado «síndrome de hiperviscosidad» es un cuadro clínico formado por cefalea, alteraciones visuales, auditivas y vestibulares, confusión y disminución del nivel de conciencia y causado por una hipervolemia extrema con una gran agregación eritrocitaria producida por la paraproteinemia. Pero junto a este síndrome de isquemia cerebral global el aumento de la viscosidad sanguínea (VS) puede también ser causa de isquemia focal. Se presenta el caso de un paciente diagnosticado de un MM tipo IgG que sufrió múltiples ataques isquémicos transitorios e ictus isquémicos menores vertebrobasilares coincidiendo con una reactivación de su enfermedad de base y quedó completamente asintomático tras la instauración del tratamiento específico con dexametasona frente a la paraproteinemia asociada al MM. En el presente trabajo se discute su fisiopatología

Monoclonal and polyclonal immunoglobulinemia, including lymphoma, Waldenström's macroglobulinemia and less commonly multiple myeloma (MM), are considered as infrequent causes of ischemic stroke. Hyperviscosity states, as well as procoagulant disturbances, both potentially treatable, have been implicated in its etiopathogenesis. The so-called «hyperviscosity syndrome» is a clinical syndrome consisting of headache, visual, auditory and vestibular disturbances, confusion and decreased level of consciousness, and is caused by extreme hypervolemia with a high degree of erythrocyte aggregation caused by paraproteinemia. However, in addition to this global cerebral ischemia syndrome, increase in blood viscosity (BV) can also be a cause of focal ischemia. We report a case of a patient diagnosed with IgG type MM, who suffered multiple vertebrobasilar transient ischemic attacks and minor ischemic strokes concurrent with a reactivation of his hematological disease. He became completely asymptomatic after specific treatment with dexamethasone of the paraproteinemia associated with MM. We discuss its pathophysiology in this report