Asymptomatic Severe Vagal and Sympathetic Cardiac Denervation in Holmes-Adie's Syndrome.

A 40-year-old woman was found to have bilateral Adie's pupils and generalized muscle stretch areflexia. She did not have orthostatic hypotension but, in an ECG strip in the office, she appeared to have an almost fixed heart rate. We thus studied the heart rate variability (HRV) and the systolic blood pressure variability (SBPV) in supine and standing position and also during rhythmic breathing. We found a decreased HRV in the time domain with very low standard deviation in supine and standing position and during rhythmic breathing. HRV in the frequency domain was low with a decrease in the absolute power of HF and LF and a decrease in the sympathovagal balance in supine and standing positions. SBPV in the time and frequency domains was found to be normal. This patient with Holmes-Adie syndrome had an asymptomatic severe loss of HRV and a preserved SBPV. The global decrease in the HRV in the time and frequency domains indicated that she had both vagal and sympathetic cardiac denervation, whereas the preserved SBPV suggested normal innervation of the blood vessels.

Effects of cerebellectomy on eye movements in man.

A patient with a right hemicerebellectomy and recurrence of a tumor on the left cerebellar hemisphere was examined regarding oculomotor and vestibuloocular functions. The findings were as follows: inability to maintain eccentric gaze; defective smooth pursuit system; failure to suppress by visual fixation the nystagmus induced by sinusoidal horizontal rotations; moderate increase of the vestibulo-ocular reflex gain when rotated in the dark; relative preservation of the slow phases of the optokinetic nystagmus, as compared to the slow phases induced by tracking a pendulum; decreased gain of the vestibulo-ocular reflex when rotated with the lights on fixating the environment; square wave jerks; and no saccadic dysmetria.

Neuro-otology of the lateral medullary infarct syndrome.

Nine patients had a lateral medullary infarction. Examination during the acute phase of the lesion to determine oculomotor and oculovestibular functions showed that all patients had hypermetric saccades and increased gain of the vestibulo-ocular reflex (VOR) to the side of the lesion and hypometric saccades and decreased gain of the VOR to the side opposite the lesion. These abnormalities may be the consequence of the isolation of the vestibular nuclei from the flocculus, with loss of saccadic and vestibular gains by the cerebellum.

Oculomotor and oculovestibular functions in a hemispherectomy patient.

Oculomotor and oculovestibular functions in a patient who had undergone a left hemispherectomy were examined under a variety of visual and vestibular stimuli. The main findings were an inability to hold left eccentric gaze, resulting in gaze-evoked nystagmus to that side; inaccurate saccades to the right; defective smooth pursuit to the left; asymmetrical vestibulo-ocular responses (VOR) in the dark due to the interaction of the VOR with the "spasticity of conjugate gaze", decreased gain of the visuo-vestibulo-ocular reflexes to both sides, more marked to the right, when rotated fixating on a stationary wall target or the environment, due to abnormal smooth and optokinetic systems; and failure to inhibit the VOR when rotated to the left with visual fixation.

HLA class II genotypes in Mexican Mestizos with familial and nonfamilial multiple sclerosis.

Predisposition to MS is associated with the HLA-DR2 antigen in white patients. The authors investigated the genetic factors behind the increasing frequency of MS in the Mexican population. HLA-DR and DQ were analyzed in 17 patients with MS, 15 of their first-degree relatives, and 99 healthy ethnically matched controls. DR2 or DR3 was found in 15 of 17 patients. In controls, both alleles had frequencies less than 0.05. MS in Mexican patients was associated with HLA-DR2 and DR3.

Fourth ventricular cysticercosis.

This is a review of the clinical presentation and results of surgical treatment of 25 cases of cysticercosis of the 4th ventricle. All of the patients presented with hydrocephalus and, after shunting, dilatation of the 4th ventricle persisted while the supratentorial ventricular system returned to its normal size. We propose that, after a shunt procedure is performed, early direct surgical excision of the parasite should be done. Good or excellent results were achieved in 81.4% of the patients. Associated arachnoiditis and vasculitis were the major causes of poor outcomes.

Mechanisms of hydrocephalus in cerebral cysticercosis: implications for therapy.

Patients with hydrocephalus secondary to cerebral cysticercosis are a highly heterogeneous group. The mechanisms of hydrocephalus in these patients are multiple. Intraventricular cysts may be found in the 3rd and 4th ventricles, the sylvian aqueduct, and the foramen of Monro. Intraventricular cysts can be suspected when the 3rd and 4th ventricles or aqueduct remain enlarged despite shunting. Intraventricular contrast medium demonstrates the presence of the parasites. The intraventricular cysts should be removed surgically. Hydrocephalus due to cisternal cysticercosis can be diagnosed by isotope cisternography. These patients should receive shunts, but the long term prognosis is probably poor. Guidelines for the management of hydrocephalus due to cysticercosis are suggested.

Coincidence of cerebrovascular accident and silent myocardial infarction.

Although it is well known that a myocardial and a cerebral infarction may be coincident, the nature of this association is not clear. The problem is further complicated because the myocardial infarction may be silent. This is a report of 3 patients with cerebral infarct in whom a silent recent myocardial infarction was found. All patients with cerebrovascular disease should be screened for a possible myocardial lesion.

Diverse clinical manifestations of pheochromocytomas.

Many difficulties are encountered by clinicians in attempting to diagnose pheochromocytomas. We describe several patients with unusual clinical features. These include sudden death, cerebral hemorrhage, refractory congestive heart failure, acute abdominal pain, and hypercalcemia. In 2 patients, the rare association of this tumor and pregnancy was observed. Two subjects had sudden death, 1 during a pneumoencephalogram and another during an epidural block. The clinicians should be aware of these manifestations of pheochromocytomas.

[A case of cortical amusia in a patient with musical ability]. / Un caso de amusia cortical en una paciente con habilidad musical.

CLINICAL CASE: A women who had previously had musical ability presented with bilateral embolic damage to both superior temporal convolutions. For the first few days, she had complete cortical deafness with reception aphasia. This evolved to a slight Wernicke's aphasia and reception amusia. The amusia was characterized by inability to understand lyrics and tunes, and in this patient has persisted a year and a half later. CONCLUSIONS: It is postulated that the inability to understand the words of songs is due to damage to the first left temporal convolution, whilst the amusia of tunes is due to damage to the first right temporal convolution.

[Alexia without agraphia due to the lesion in the right occipital lobe in a right-handed man. Detection of hemispheric lateralization of handedness and language in a right-handed patient]. / Alexia sin agrafia por lesión occipital derecha en varón diestro. Segregación de la lateralización hemisférica manual y del lenguaje en un paciente diestro.

INTRODUCTION AND CLINICAL CASE: A 65 year-old man, right-handed, without any family history of left handiness, suddenly developed a left homonymous hemianopia and incapacity for reading. Neurological and neuropsychological examinations showed the presence of a profound alexia with preservation of writing to dictation and spontaneously. He was unable to read what he had written. He could spell the words letter by letter but he was unable to read the complete word. MRI showed an extensive infarct in the territory of the right posterior cerebral artery. The infarct extended anteriously to the right thalamus and to the medial temporal fifth or fusiform gyrus. The splenius was spared. Brain SPECT disclosed the area of the infarct and an extensive area of decreased cerebral perfusion over the right parietal and temporal areas. CONCLUSION: Alexia without agraphia has been reported in right-handed patients with left occipital lesions and in right occipital regions in left-handed patients but rarely if ever in right occipital lesions in right-handed patients.

[Elevation of total urinary catecholamines in patients with subarachnoid hemorrhage]. / Elevación de catecolaminas urinarias totales en pacientes con hemorragia subaracnoidea.

We designed a prospective study in order to evaluate total urinary catecholamines in patients with subarachnoid hemorrhage (SAH) and correlate them with some prognostic factors and its complications. We studied 24 patients: 19 were normotensive, five had had a previous arterial hypertension that persisted during the SAH, and five developed the hypertension during the SAH. The latter showed cardiac complications with a more severely affected Hunt and Hess scale and a higher level of excretion of urinary catecholamines than the normotensive patients (p < 0.003). The cases with previous arterial hypertension that persisted during the SAH had more complications than the normotensive patients but in a lesser degree compared to chose who developed the hypertension during the SAH.

Horizontal appendicular dysmetria in four patients with a typical Wallenberg's syndrome.

During the acute stage of a Wallenberg's syndrome ipsilateral appendicular dysmetria is frequently seen. The dysmetria is more apparent in the ipsilateral upper extremity. These patients also have a peculiar type of dysmetric eye movements that are characterized by hypermetric saccades toward the side of the lesion and hypometric saccades to the opposite side. We examined four patients with acute Wallenberg's syndrome and found horizontal dysmetria of the affected extremity. Hypermetric arm and hand movements were present to the side of the lesion and hypometric movements toward the opposite side. This type of dysmetria is probably related to the same patophysiological mechanism that underlies dysmetric eye movements in the Wallenberg's syndrome. The dysmetria tends to disappear with time although it continues to be present in some patients six months after the ischemic damage.

[The other genome: the clinical concept of mitochondrial cytopathies or disorders of pxidative phosphorylation]. / El otro genoma: el concepto clínico de las citopatías mitocondriales o enfermedades de la fosforilación oxidativa.

The investigations of mitochondrial DNA abnormalities and its relationship with derangements of oxidative phosphorylation and electron transport system, has yielded description a myriad of syndromes called mitochondrial diseases or cytopathies. The objective of this paper is to review the clinical relevant features of this heterogeneous group of diseases, to understand the board spectrum of signs and symptoms and suggest an algorithm for the diagnosis.