Prosopagnosia y simultagnosia aisladas debidas a infarto isquémico en territorio occipito-temporal derecho / Isolated prosopagnosia and simultagnosia due to ischemic infarction in right occipitotemporal territory

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[Crazy laughter: first manifestation of a pontine tumour]. / "Risa loca": primera manifestación de un tumor protuberancial.

Crazy laughter (<>) was first described in 1903 as a prodromic symptom of an ischemic stroke and was later associated with brain lesions having a different location and etiology. We describe the case of a patient with a poorly differenciated pulmonary carcinoma who presented a centropontine image consistent with metastasis, whose initial manifestation was involuntary, persistent and unmotivated laughter that preceded other clinical manifestations. We revised, on the one hand, previous cases described in the literature of pathological laughter in relationship to structural lesions, of vascular or neoplastic etiology, and, on the other, the nervous centers and pathways that control the laughter mechanism.

"Risa loca": primera manifestación de un tumor protuberancial / Crazy laughter: first manifestation of a pontine tumour

La "risa loca" (fou rire) fue descrita en 1903 como síntoma prodrómico de un accidente vascular cerebral y posteriormente se ha asociado a lesiones cerebrales de distinta localización y etiología. Describimos el caso de un paciente con un carcinoma pulmonar mal diferenciado que presentaba una imagen a nivel centroprotuberancial compatible con metástasis, cuya manifestación inicial fue una risa involuntaria, persistente e inmotivada que precedió a otras manifestaciones clínicas. Se revisan, por un lado, los casos descritos en la literatura de risa patológica en relación con lesiones estructurales, de etiología vascular o neoplásica y, por otro, los centros y vías nerviosas que controlan el mecanismo de la risa (AU)

Las neuropatías vasculíticas no sistémicas / Nonsystemic vasculitis neuropathies

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[Vesico-sphincter dysfunction in 108 cases of multiple sclerosis]. / Disfunción vésico-esfinteriana en 108 casos de esclerosis múltiple.

OBJECTIVE: To study vesicosphincteric dysfunction in 108 patients with multiple sclerosis. METHODS: We reviewed the clinical records of 108 patients with multiple sclerosis and analyzed those with voiding symptoms ascribable to multiple sclerosis. These patients underwent complete urodynamic assessment and complementary tests according to their symptoms. The Blaivas classification was used for the clinical classification of multiple sclerosis. RESULTS: 64 of the 108 patients presented voiding symptoms ascribable to multiple sclerosis (59.2%). The clinical features presented as episodes in 75% and were progressive in 25% of the cases. In 6% of the patients, the voiding symptoms were the first symptoms of multiple sclerosis. Urodynamic assessment showed detrusor hyperreflexia in 73% of the patients, hyporeflexia in 14%, and 13% showed normal urodynamics. All complications were infective; no patient showed upper urinary tract complications. CONCLUSIONS: Vesicosphincteric dysfunction in multiple sclerosis is frequent. Most of the patients present bladder hyperreflexia. The urological complications are usually infective. Involvement of the upper urinary tract is rare.

[Crossed aphasia: description of a case]. / Afasia cruzada: descripción de un caso.

We describe a new case of crossed aphasia in a right-handed patient with a right hemispheric lesion. A right-handed man, 76 year-old, developed a sudden left hemiparesis with sensitive impairment and mutism. He has neither family history of left handeness or ambidexterity or vascular risk factors. CT cerebral scan showed a large infarct of the middle cerebral artery on the right side, with haemorrhagic suffusion. Cerebral MRI and EEG-cartography confirmed the indemnity of the left hemisphere. Aphasia studies confirmed a mutism with spared verbal comprehension, but alexia was present. A year later, left hemiparesis was recovered but aphasia remained. Crossed aphasia is rarely seen. It is caused by a right hemispheric lesion in right-handed subjects. Fluency is most commonly impaired. At onset, mutism is the common symptom, which evolves to expressive aphasia. Several hypothesis have been raised about the possible mechanisms involved. The few number of PET or SPECT studies performed in these patients have disclosed extensive areas of hypometabolism in the right hemisphere, that exceed the size of the image observed with CT scan or MRI.

Afasia cruzada: descripción de un caso / Crossed aphasia: description of one case

El objetivo del trabajo es la descripción de un nuevo caso de afasia cruzada en un paciente diestro con lesiones en el hemisferio derecho. Se trata de un varón de 76 años, diestro, sin antecedentes familiares de zurdera y sin factores de riesgo vascular, que presentó de forma brusca hemiparesia izquierda con compromiso sensitivo y cuadro de mutismo. La TC craneal confirmó la existencia de un extenso infarto isquémico con componente hemorrágico en el territorio córtico-subcortical de la arteria cerebral media derecha. El estudio mediante RM y la cartografía-EEG confirmaron la indemnidad del hemisferio izquierdo. Los tests de afasia pusieron de manifiesto un cuadro de mutismo con comprensión preservada para el lenguaje oral, pero alterada para el escrito. El paciente recuperó progresivamente el déficit motor, persistiendo una grave afasia de expresión. La afasia cruzada es una alteración del habla poco frecuente. Se produce por una lesión hemisférica derecha en personas diestras y suele manifestarse por trastornos de la fluencia, con mutismo inicial que evoluciona hacia una afasia motora. Se han planteado diversas hipótesis referentes a los mecanismos responsables. Los estudios con PET o SPECT han demostrado zonas de hipometabolismo en el hemisferio derecho, que sobrepasan la lesión demostrada por TC o RNM (AU)

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[Bilateral intra-axial involvement of the common oculomotor nerve (III): 2 cases]. / Atteinte bilatérale intra-axiale du nerf moteur oculaire commun (III): deux cas.

Bilateral palsy of the common ocular motornerve (III) was observed in two patients with an intra-axial lesion due ti hemorrhage in one and ischemia in the other. The lesions involved the cerebral peduncle in the periaqueductal region and the nuclear complex of the III in the first case. Bilateral infarct of the thalamus was seen in the second. Clinical manifestations were transitory except for the oculomotor impairment. In the first patient, oculomotricity was dissociated as intrinsic mortricity was spared. These exceptional cases demonstrate a syndrome with unique oculomotor expression resulting from intra-axial oculomotor lesions. Prognosis varies and is related to the ischemic or hemorrhagic nature of the causal lesion and its localization.

[Disappearance of migraine crises in two patients with male infertility treated with human chorionic gonadotropin/human menopausal gonadotrophin]. / Desaparición de crisis migrañosas en dos pacientes tratados con hCG/hMG por infertilidad masculina.

Two patients aged 52 and 31 respectively, treated for male infertility with gonadotrophins (LCG/LMG), showed marked improvement of their migraine crises associated with a typical aura which had been present since puberty. Changes in the number, motility and morphology of the spermatozoids were seen in the seminogram. The plasma concentrations of FSH, LH, testosterone an 17-beta oestrodiol were within normal limits. After three months of empirical treatment with LCG/ LMG (to stimulate spermatogenesis) the migraine crises ceased and the patients are still free of migraine after 32 and 26 months respectively. The relationship between migraine and the sex hormones is discussed, in the context of current knowledge of the psysiopathology of migraine and the beneficial effects obtained after treatment with LCG/LMG. We have not found any reference in the literature to the use of gonadotrophins in the treatment of migraine with a typical aura.

[Spontaneous remission of cervical epidural hematoma with pathological confirmation]. / Hematoma epidural cervical con remisión espontánea y verificación neuropatológica.

Spontaneous cervical epidural hematoma (CEH) is a rare entity but one that can cause permanent neurologic deficit unless, it is widely believed, surgical evacuation is prompt. A diagnosis of CEH is confirmed by computed tomography and/or magnetic resonance imaging. Spontaneous remission is unusual, with only 7 such cases reported in the literature as of 1993. We present a case of CEH with clinical remission and confirmation by neuroimaging in a patient who died of an unrelated cause. We conclude that when neurologic deficit is incomplete and there are no signs of clinical progression after 48 h, surgical treatment should not be applied routinely.

[A descriptive study of 105 patients with cerebral metastasis]. / Estudio descriptivo de 105 pacientes con metástasis cerebrales.

BACKGROUND: To investigate the hospital frequency and natural history of patients with cerebral metastases (CM). METHODS: A retrospective study of patients seen because of CM from 1984 to 1989. They were identified from the discharge reports and the cancer registry (CR). Data of interest were taken from the clinical record and the CR. RESULTS: 105 patients were identified. Mean age was 60 years. There were 77 males. In 44 patients cancer past history was present, in 33 lung cancer was simultaneously diagnosed and in 28 there was no past cancer history and the primary neoplasm was not identified. CM were multiple in 49 patients. In 18 patients CM was single, with no extracerebral neoplasia. Craniotomy was carried out in 22 patients and 11 received postoperative radiotherapy. The probability of one-year survival in the operated and nonoperated group was 27% and 1.5%, respectively (27 +/- 20% and 1.5% +/- 1.5%; 95% confidence intervals). CONCLUSIONS: The frequency of the diagnosis of CM is not negligible and its occurrence virtually always represents a fatal prognosis. About one half are caused by a lung cancer that may have clinically presented with CM. Poor general condition, multiple CM or extracerebral neoplastic disease prevent radical therapeutic intervention in nearly 80% of these patients. Survival with palliative therapy is shorter than that with surgical treatment.

[Non-infective neurologic complications associated to heroin use]. / Complicaciones neurológicas no infecciosas asociadas al consumo de heroína.

The spectrum of neurological complications associated with heroin addiction has changed in the past six years because of the progressive knowledge of the neurological complications related to HIV infection. We reviewed 48 heroin addicts with neurological complications and 452 heroin overdose who were seen in the Emergency Unit of our hospital during 1988 and the publications since 1967. Regarding the overdose we present the results of a prospective study leading to determine the causes. We emphasize the relationship with the level of total morphine in serum, instead of conjugate morphine, and with the presence of high levels of benzodiazepines found in the plasma rather than an hypothetic hypersensitivity phenomenon. We resume the neurological complications related with heroin addiction: spongiform leukoencephalopathy, epileptic seizures, stroke, transverse myelopathy and neuromuscular complications such mononeuropathy, plexopathy, acute inflammatory demyelinating polyradiculoneuropathy, rhabdomyolysis, fibrosing myopathy, musculoskeletal syndrome and acute bacterial myopathy. Some of such complications (i.e. transverse myelitis, polyradiculoneuropathy, leucoencephalopathy) must rise the suspicion of an HIV infection. Likewise, in patients assisted for overdosage we believe it's necessary rule out myoglobinuria by means of CPK serum levels and detection of urine hematic pigments without red blood cels in the urine sediment, in order to prevent and treat the renal failure. We report the results of muscular biopsy found in the musculoskeletal syndrome, which are similar to those found in alcoholic myopathy. Finally, we describe the clinical and diagnostic aspects in an unusually neuromuscular complication: the acute bacterial myopathy.

[Primary meningeal and cerebral lymphoma. Study of a case with 8 years' follow-up]. / Lymphome primitif méningé et cérébral. Etude d'un cas suivi pendant 8 ans.

A primary lymphoma involving the meninges of the frontobasal region, with focal cortical infiltration and prominent cerebral edema, was treated by surgery and radiotherapy. Eight years later the patient died of a recurrence. Morphological and immunohistochemical studies comparing the initial tumor with the recurrent lesion found similar histopathological features.