Utility of transcranial sonography in the diagnosis of drug-induced parkinsonism: a prospective study.

BACKGROUND AND PURPOSE: Drug-induced parkinsonism usually resolves after discontinuation of the causative agent. However, it persists in some patients, who actually have subclinical neurodegenerative parkinsonism. Identification of this condition is important because these patients could benefit from therapeutic measures. The objective of this study was to prove whether transcranial sonography, a technique used in the diagnosis of neurodegenerative parkinsonism, can be used for the said identification. METHODS: In this prospective study, patients with drug-induced parkinsonism were followed for at least 6 months after discontinuation of the causative drug and performance of blinded transcranial sonography. Patients were categorized as having iatrogenic parkinsonism if the clinical presentation had resolved or subclinical drug-exacerbated parkinsonism if it persisted. Once the patient was classified into one of the two groups, an expert assessed the transcranial sonography findings and their agreement with the clinical diagnosis. RESULTS: Twenty patients composed the group for analysis of results. Assessing hyperechogenicity in the substantia nigra >20 mm2 and/or hyperechogenic lentiform nucleus, differences were detected between the iatrogenic parkinsonism and the subclinical drug-exacerbated parkinsonism groups, although they did not reach statistical significance (Fisher's exact test 0.09). Joint assessment of sonographic alterations in both structures had a negative predictive value of 85.7% for diagnosis of drug-induced parkinsonism, with a negative likelihood ratio of 0.3. CONCLUSIONS: Although in our study statistically significant differences were not found between the transcranial sonography characteristics of subclinical drug-exacerbated parkinsonism and iatrogenic parkinsonism patients, we believe that transcranial sonography is a valid technique for diagnosis of drug-induced parkinsonism.

Diagnostic accuracy of 123 I-FP-CIT SPECT in diagnosing drug-induced parkinsonism: a prospective study.

INTRODUCTION: Drug-induced parkinsonism is a major type of parkinsonism in our setting. Symptoms usually disappear after discontinuation of the drug. However, they may persist in patients with a variant known as subclinical drug-exacerbated parkinsonism; early identification of this entity has important prognostic and therapeutic implications. The most widely used complementary test in this diagnosis is single-photon emission computed tomography with ioflupane ((123)I), also known as (123)I-FP-CIT SPECT. The aim of our study is to verify its diagnostic accuracy. METHODS: We designed a prospective study of patients with drug-induced parkinsonism in which, after discontinuing the drug and undergoing a (123)I-FP-CIT SPECT scan, patients would be monitored for at least 6 months. Patients were categorised as having iatrogenic parkinsonism if symptoms disappeared, or as having subclinical drug-exacerbated parkinsonism if they persisted. Lastly, we verified concordance between the clinical diagnosis and results from the (123)I- FP-CIT SPECT scan. RESULTS: The sample included 19 patients. The most commonly prescribed drug class was neuroleptic agents. For the diagnosis of both subgroups, (123)I-FP-CIT SPECT showed a sensitivity of 66.7%, specificity and positive predictive value of 100%, a negative predictive value of 86.7%, and a negative likelihood ratio of 0.33. CONCLUSIONS: Although the study needs to be repeated in a larger sample of patients, (123)I-FP-CIT SPECT is useful in the diagnosis of drug-induced parkinsonism since it is a very precise tool for identifying patients with that illness.

Formulation of anomerization and protonation in d-glucosamine, based on 1H NMR.

The major anomer of non-protonated neutral d-glucosamine GlcN0 is the ß-form, while the α-anomer is dominant for protonated cationic glucosamine GlcNH+. The present work confirmed correlation between the anomerization and the protonation by simultaneous determination of signal intensity and chemical shift in pD-variation 1H NMR, and formulated the equilibrium constants between subspecies α-GlcN0, ß-GlcN0, α-GlcND+, and ß-GlcND+ to interpret the correlation. The individual anomerization constants, Kan = [ßGlcN]/[αGlcN] and KanD = [ßGlcND+]/[αGlcND+], are linked to each other through the relation KDα∙KanD = KDß∙Kan with the deuteration constants KDα and KDß of the anomers. The anomer populations are stimulated by OD- and D+ ions in the dose-response form. The acidic deuteron in α-GlcND+ is populated mostly at the nitrogen atom, whereas the population in ß-GlcND+ is comparable at nitrogen and anomeric oxygen; this difference is consistent with the basicity of the nitrogen and the anomerization process of glucosamine.

[Cerebral hemodynamics in the syndrome of pseudomigraine with csf-pleocytosis:a transcranial doppler study]. / Hemodinámica cerebral en el síndrome de pseudomigraña con pleocitosis de LCR: un estudio Doppler transcraneal.

INTRODUCTION: The clinical picture of the pseudomigraine with CSF pleocytosis syndrome is of intermittent headaches, sometimes accompanied by episodes of transient neurological defects and lymphocyte pleocytosis, which occur for a variable length of time, followed by spontaneous resolution. It has been suggested that the cause is immunogenic due to post-infectious activation of the humoral immune system against the leptomeningeal vascular structures, but this hypothesis does not explain the accompanying transient deficit phenomena. MATERIAL AND METHODS: We present a study of cerebral hemodynamics using transcranial Doppler in a patient diagnosed as having pseudomigraine with pleocytosis according to current criteria. RESULTS: Our results suggest the presence of cerebral vascular instability and increased vascular reactivity to induced hypocapnia and hypercapnia, similar to that reported in patients with migraine with aura. At no time was significant increase in the average rate of cerebral blood flow detected, comparable to that usually seen in patients with bacterial or viral meningitis. CONCLUSIONS: Although the cause cannot be established, from our study it would seem that headache in the pseudomigraine with pleocytosis syndrome is related to cerebral vascular instability and therefore probably to activation of the trigeminal-vascular system. It is possible that the deficit phenomena may also have a similar explanation to that of patients with migraine with aura, that is, a propagated cortical depression. Transcranial Doppler studies may be useful in the diagnosis of pseudomigraine with pleocytosis syndrome.

[Epidemiological features of epilepsy in adults requiring hospital admission]. / Caracterización epidemiológica de la epilepsia del adulto subsidiaria de ingreso hospitalario.

INTRODUCTION AND OBJECTIVES: There are few epidemiological studies specifically directed towards finding the features of the adult epileptic population requiring hospital admission. We proposed to analyze these features in a consecutive series of patients admitted to our department with epilepsy and find the prevalence of diagnoses classified as syndromes and epileptic seizures according to the classification of ILAE and their distribution according to age. PATIENTS AND METHODS: We made a retrospective classification of the characteristics of the patients admitted to our department with the diagnosis of epilepsy during 1999. We divided the sample into three age groups: 18 30, 31 60 and >60 years. We calculated the absolute prevalence of the different types of crises and epileptic syndromes, the specific relative prevalence according to age groups and diagnostic category expressed with confidence intervals of 95%. The degree of statistical significance was obtained using the chi squared test of Mantel Haenszel. RESULTS: The sample group contained 96 patients (51% men and 49% women). There were 65.62% partial seizures, 21.87% generalized seizures and 12.5% pseudoseizures. The syndromes found included 60.7% of epilepsies related to site, 5.95% generalized epilepsies, 7% unclassified syndromes and 26.19% special syndromes. Of the symptomatic epilepsies, the commonest etiology was vascular, followed by toxic, tumoral and degenerative. The evolution of the relative prevalence according to age corresponded to three different patterns: ascending (partial crises and special syndromes), descending (pseudocrises and generalized epilepsy) and stable with peaks in middle age (generalized seizures, partial with generalization, unclassified syndromes, symptomatic and cryptogenic partial epileptic syndromes). CONCLUSIONS: The distribution according to seizures is similar to that reported in population studies. Special syndromes are much commoner than that reported in population studies, especially in epilepsies conditioned by the environment. The diagnosis of pseudoseizures are significantly commoner in the group of young adults. Partial seizures and special syndromes tended to be more frequent in older patients, whilst pseudocrises and generalized epilepsies were commoner in younger adults. The remaining seizures and syndromes predominated in the 18 30 year age group.

[Parry-Romberg progressive facial hemiatrophy: pathogenic and evolutionary considerations with regards to a case followed up for a long period of time]. / Hemiatrofia facial progresiva de Parry-Romberg: consideraciones patogénicas y evolutivas a propósito de un caso con seguimiento prolongado.

INTRODUCTION: The Parry-Romberg syndrome is a clinical condition in which there is progressive facial hemiatrophy which may be associated with neurological, ocular, cutaneous and auto-immune disorders. It is of unknown aetiology, although many theories have been put forward. CLINICAL CASE: We present a case of left facial hemiatrophy associated with ipsilateral lingual hemiatrophy and signs of cortico-cerebellar dysfunction, the neuroimaging findings (NMR) and clinico-radiological follow-up covered a period of over 10 years. CONCLUSION: The data on the clinical course and neuroimaging, together with our review of the literature, suggest that the Parry-Romberg syndrome may be considered to be a dysgenetic process which may originate during the first stages of CNS embryogenesis.

[Ring chromosome 20: an epileptic channel disorder?]. / Cromosoma 20 en anillo: una canalopatía epiléptica?

INTRODUCTION: The ring-shaped chromosome 20 (r20) syndrome is an infrequent chromosopathy which is associated with epileptic seizures, behaviour disorders and mental retardation. It results from the fusion of the two arms of the chromosome with deletion of the telomeric portions. CLINICAL CASE: We present a case of r20, review published cases and describe the clinical and neurophysiological characteristics. CONCLUSIONS: The r20 syndrome is the third type of epilepsy known to be of genetic basis related to chromosome 20. It has clinical and neurophysiological characteristics which give it a distinctive character and are easily identified. The fact that on locus 20q13 (telomeric portion of the long arm of chromosome 20) two genes related to epileptic channelopathies (CHRNA4 and KCNQ2) have been described, suggest the hypothesis that the subjacent deletion in cases of r20 syndrome affect one of these genes and explains the epileptogenicity. We consider this hypothesis and the possibility that r20 syndrome may be an epileptic channelopathy.

[Inflammatory pseudotumor: differential diagnosis of tumors of the 4th ventricle]. / Pseudotumor inflamatorio: diagnóstico diferencial de los tumores del IV ventrículo.

INTRODUCTION: An inflammatory pseudotumour is a condition of unknown origin and inflammatory nature. It is rarely found in the CNS. We report a case of inflammatory pseudotumour localized to the IV ventricle and review the clinical characteristics of previously reported cases in order to outline the clinical profile of this condition. CLINICAL CASE: A 40 year old man was admitted to hospital complaining of a subacute condition involving difficulty in speaking and in moving his right arm and leg. On examination he had ocular deviation on initial gaze, a complete right Horner's syndrome, right supranuclear facial palsy, dyssynergy-asymmetry on the right finger-nose test and a dissociated sensitivity disorder of the left arm. On MR of the brain there was a space occupying lesion, nodular in form and fixed to the roof of the fourth ventricle. The histopathological report on the specimen removed by surgery stated it to be an inflammatory pseudotumour. On a MEDLINE search for reported cases of inflammatory pseudotumour of the CNS, 27 were found since 1967. Four cases, including ours, involved masses growing into the interior of the fourth ventricle. CONCLUSIONS: Inflammatory pseudotumour of the CNS is a condition affecting young adults, with a slight male predominance and some association with clinical and analytical data suggesting autoimmune dysfunction. The intraventricular site, particularly within the fourth ventricle, is relatively common (4/28) and is usually associated with clinical features of dysfunction of the posterior fossa and/or intracranial hypertension. We consider that inflammatory pseudotumour should be included in the differential diagnosis of tumours of the fourth ventricle.

[Scenes in movement. Movement disorders on film]. / Escenas en movimiento. Los trastornos del movimiento en el cine.

INTRODUCTION: There are publications in which various neurological diseases are analysed on film. However, no references have been found on movement disorders in this medium. METHODOLOGY AND RESULTS: A total of 104 documents were collected and reviewed using the internet movie data base (IMDb). The majority were associated with dystonia, Parkinson's and tics, were American commercial productions, and the most common genre was drama. DISCUSSION: The cinema usually depicts old men with developed Parkinson's disease. However, motor complications only appear in 19% and non-motor symptoms in 14%. The image of dystonia is generally that of a young man, with disabling dystonia secondary to childhood cerebral palsy. Tics appear associated with Tourette's syndrome, with the excessive use of obscene expressions and with very few references to other important aspects of this syndrome, such as mood and behavioural changes. The majority of tremors portrayed on film are associated with Parkinsonism and are not pathological. Myoclonus appears anecdotically and is normally symptomatic. CONCLUSIONS: Parkinson's disease is the type of movement disorder that the cinema portrays with greater neurological honesty and in a more dignified manner.

[Elucidation on a dystonic emperor]. / Elucubraciones sobre un emperador distónico.

Tiberius Claudius Caesar Augustus (10 BC.-54 DC.) governed the Roman empire for 14 years, being popularly known as Claudium, the <> and <> emperor. Most of the works published on his health coincide that the cause of his sufferings was an infant Athetoid cerebral palsy. However, the reading of the classics (Suetonius, Dion Cassius, Seneca and Tacitus) manifests the existence of some symptoms such as hypoacusis, recurrent abdominal pain, sleep disorders and probable myopathy that could not only be explained by this clinical picture. The analysis of all the vegetative symptoms, presence of pathological family history and the possibility of a progressive course of his cognitive disorder makes it possible to suggest the hypothesis of a mitochondrial cytopathic type multisystemic disease as an etiological alternative.

Possible case of peripheral osmotic demyelination syndrome.

Central pontine myelinolysis (CPM) is an uncommon neurological syndrome that is usually related to the rapid restoration of a previous hyponatraemia. Although the most frequent location of CPM injury is the pons, it is now designated osmotic demyelination syndrome (ODS) because, as well as in the brainstem, these injuries can be observed in other parts of the central nervous system (CNS)-for example, the thalamus, subthalamic nucleus, external geniculate body, putamen, globus pallidum, internal capsule, white matter of cerebellum and the deep layers of the brain cortex. However, an exhaustive search of the literature (MEDLINE 1967-2007) has revealed no case report of peripheral nervous system (PNS) demyelination secondary to severe hyponatraemia.

[Trigeminal-autonomic cephalalgias: International Headache Society diagnostic criteria does not allow for a correct classification]. / Cefaleas trigeminoautonómicas: los criterios diagnósticos de la International Headache Society no permiten su clasificación correctamente.

INTRODUCTION: The International Classification of Headache Disorders only recognizes the following as trigeminal- autonomic cephalalgias (TAC): cluster headache, paroxysmal hemicrania and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome. Nevertheless, there are other types of TAC that still have not been incorporated into the International Classification of Headache Disorders although they have been before reported in the literature. We present the results of the analysis of a series of 94 cases of TAC. METHODS: We performed a retrospective study of 2,132 patient who attended a general neurology consultation due to headache between January 1997 and June 2006. Those patients with unilateral headache (orbital, supraorbital y/o temporal) accompanied of some ipsilateral autonomic sign were selected. We investigated the etiology of all cases and made a nosology classification according with two types of criteria: IHS (International Headache Society) strict criteria as well as other criteria (IHS plus) including migraine with ipsilateral autonomic signs and hemicrania continua, thus following Goabsby. RESULTS: A total of 94 cases of TAC (4.4% of all the headaches) were found, 89 of which had a primary etiology and 5 secondary etiology. Cluster headache was the most frequent TAC in our series (29%), independently of the criteria used. However, hemimigraine with ipsilateral autonomic signs had a similar frequency (28%) according to IHS plus criteria. CONCLUSIONS: a) In our series TAC have constituted an not very frequent entity, there being a subgroup of secondary cases among them, and b) the current International Classification of the Headache Disorders has some limitations as an instrument for the nosology classification of TAC.

Utilidad de la tomografía por emisión de fotón simple con 123Ioflupano en el diagnóstico del parkinsonismo relacionado con fármacos. Estudio prospectivo / Diagnostic accuracy of 123I-FP-CIT SPECT in diagnosing drug-induced parkinsonism: a prospective study

Introducción: El parkinsonismo relacionado con fármacos es una de las principales causas de parkinsonismo en nuestro entorno. La clínica suele desaparecer tras la retirada del fármaco, sin embargo puede persistir en un grupo de pacientes que presentan lo que se conoce como parkinsonismo subclínico exacerbado por fármacos y cuya identificación precoz es importante por las implicaciones pronósticas y terapéuticas. La prueba complementaria más utilizada para ello es la tomografía por emisión de fotón simple con 123Ioflupano, también conocida como SPECT [123I]FP-CIT. El objetivo de nuestro estudio es corroborar su utilidad para tal fin. Métodos: Se diseñó˜ un estudio prospectivo de pacientes con parkinsonismo relacionado con fármacos a los que, tras retirada del mismo y realización de SPECT [123I]FP-CIT, se les siguió durante un mínimo de 6 meses. Los pacientes se catalogaron como parkinsonismo iatrogénico si había desaparecido la clínica o parkinsonismo subclínico exacerbado por fármacos si la misma persistía. Finalmente se comprobó la concordancia entre el diagnóstico clínico y los resultados del SPECT [ 123I]FP-CIT. Resultados: La muestra quedó constituida por 19 pacientes. El grupo terapéutico mayoritario fueron los neurolépticos. Para el diagnóstico de ambos subgrupos el SPECT [ 123I]FP-CIT mostró una sensibilidad del 66,7%, una especificidad y un valor predictivo positivo del 100%, un valor predictivo negativo del 86,7% y una razón de verosimilitud negativa de 0,33. Conclusiones: Aunque es necesario comprobarlo con un número mayor de pacientes, el SPECT [123I]FP-CIT es una técnica útil en el diagnóstico de parkinsonismo relacionado con fármacos ya que identifica con gran precisión a los enfermos (AU)

Introduction: Drug-induced parkinsonism is a majortype of parkinsonism in our setting. Symptoms usually disappear after discontinuation ofthe drug. However,they may persistin patients with a variant known as subclinical drug-exacerbated parkinsonism; early identification of this entity has important prognostic and therapeutic implications. The most widely used complementary test in this diagnosis is single-photon emission computed tomography with ioflupane (123I), also known as 123I-FP-CIT SPECT. The aim of our study is to verify its diagnostic accuracy. Methods: We designed a prospective study of patients with drug-induced parkinsonism in which, after discontinuing the drug and undergoing a 123I-FP-CIT SPECT scan, patients would be monitored for at least 6 months. Patients were categorised as having iatrogenic parkinsonism if symptoms disappeared, or as having subclinical drug-exacerbated parkinsonism if they persisted. Lastly, we verified concordance between the clinical diagnosis and results from the 123IFP-CIT SPECT scan. Results: The sample included 19 patients. The most commonly prescribed drug class was neuroleptic agents. For the diagnosis of both subgroups, 123I-FP-CIT SPECT showed a sensitivity of 66.7%, specificity and positive predictive value of 100%, a negative predictive value of 86.7%, and a negative likelihood ratio of 0.33. Conclusions: Although the study needs to be repeated in a larger sample of patients, 123I-FPCIT SPECT is useful in the diagnosis of drug-induced parkinsonism since itis a very precise tool for identifying patients with that illness (AU)

Escenas en movimiento. Los trastornos del movimiento en el cine / Scenes in movement. Movement disorders on film

Introducción: Hay publicaciones que analizan distintas afecciones neurológicas en el cine; sin embargo, no se han encontrado referencias sobre los trastornos del movimiento en dicho medio. Metodología y resultados: Se han revisado 104 documentos, recopilados a través de la base de datos cinematográfica de internet (IMDb), entre los que predominan los relacionados con la distonía, el parkinsonismo y los tics. En su mayoría son producciones comerciales estadounidenses y el género más frecuente es el drama. Discusión: El cine suele mostrar varones ancianos con enfermedad de Parkinson evolucionada; sin embargo, sólo en el 19% aparecen complicaciones motoras y en el 14%, sintomatología no motora. La imagen del distónico generalmente es la de un varón joven, con distonía discapacitante secundaria a parálisis cerebral infantil. Los tics aparecen asociados al síndrome de Tourette, con uso abusivo de expresiones coprolálicas y con escasas referencias a otros aspectos relevantes de este síndrome, como las alteraciones anímicas o conductuales. La mayor parte del temblor cinematográfico está relacionado con el parkinsonismo o no es patológico. Las mioclonías aparecen anecdóticamente y suelen ser sintomáticas. Conclusiones: La enfermedad de Parkinson es el tipo de trastorno del movimiento que el cine aborda con mayor rigor neurológico y de una manera más digna (AU

Introduction: There are publications in which various neurological diseases are analysed on film. However, no references have been found on movement disorders in this medium. Methodology and results: A total of 104 documents were collected and reviewed using the internet movie data base (IMDb). The majority were associated with dystonia, Parkinson’s and tics, were American commercial productions, and the most common genre was drama. Discussion: The cinema usually depicts old men with developed Parkinson’s disease. However, motor complications only appear in 19% and non-motor symptoms in 14%. The image of dystonia is generally that of a young man, with disabling dystonia secondary to childhood cerebral palsy. Tics appear associated with Tourette’s syndrome, with the excessive use of obscene expressions and with very few references to other important aspects of this syndrome, such as mood and behavioural changes. The majority of tremors portrayed on film are associated with Parkinsonism and are not pathological. Myoclonus appears anecdotically and is normally symptomatic. Conclusions: Parkinson’s disease is the type of movement disorder that the cinema portrays with greater neurological honesty and in a more dignified manner (AU)

Elucubraciones sobre un emperador distónico / Elucidation on a dystonic emperor

Tiberio Claudio César Augusto Germánico (10 a.C.-54 d.C.) gobernó el imperio romano durante 14 años, siendo popularmente conocido como Claudio, el emperador «cojo» y «tartamudo». La mayoría de los trabajos publicados sobre su salud coinciden en que la causa de sus padecimientos fue una parálisis cerebral infantil de tipo atetoide. Sin embargo, la lectura de los clásicos (Suetonio, Dion Casio, Séneca y Tácito) pone de manifiesto la existencia de unos síntomas, como hipoacusia, abdominalgias recurrentes, trastornos del sueño y probable miopatía, que no podrían explicarse sólo por este cuadro clínico. El análisis de todo el cortejo sintomático, la presencia de historia familiar patológica y la posibilidad de un curso progresivo de su trastorno cognitivo permiten plantear la hipótesis de una enfermedad multisistémica, del tipo citopatía mitocondrial, como alternativa etiológica (AU)

Tiberius Claudius Caesar Augustus (10 BC.-54 DC.) governed the Roman empire for 14 years, being popularly known as Claudium, the <> and <> emperor. Most of the works published on his health coincide that the cause of his sufferings was an infant Athetoid cerebral palsy. However, the reading of the classics (Suetonius, Dion Cassius, Seneca and Tacitus) manifests the existence of some symptoms such as hypoacusis, recurrent abdominal pain, sleep disorders and probable myopathy that could not only be explained by this clinical picture. The analysis of all the vegetative symptoms, presence of pathological family history and the possibility of a progressive course of his cognitive disorder makes it possible to suggest the hypothesis of a mitochondrial cytopathic type multisystemic disease as an etiological alternative (AU)