Paroxysmal neuralgic upper cervical pain attacks: the lower syndrome of cluster headache.

We present a group of seven patients with a lower syndrome (LS) of cluster headache (CH). Seventy-three newly diagnosed patients with CH were subjected to a 9 years follow-up study; 66 patients were classified as upper syndrome (US) and only seven patients (9.5%) as LS. We focus on the characteristics of this group of seven patients with LS and compare them with existing literature. The seven cases with LS illustrate the wider spectrum of clinical manifestations that can occur in CH, namely infraortibal symptoms or these outside the territory of the trigeminal branches such as the upper cervical region or the craniocervical margin. The duration of the attacks can last more than 180 min. The attacks show a circadian/circannual regularity and a stereotypic pattern of symptoms in most of the patients. The severity and duration of the attacks may increase over the years. The neck pain can overshadow all the other symptoms of CH. Because of the regularity of the attacks at particular times of day or night in some patients, even abortive therapy can be used as prevention when taken some hours before the suspected attack. We hypothesize that some patients with LS may represent an anatomical-functional variant of a primary chronic neurovascular pain disorder originating from the central nervous system, with possible involvement of the hypothalamus and the trigeminovascular (TV) system, with inputs from the cervical roots C(0)-C(2). Clinicians should consider CH when pain attacks are located outside the orbitotemporal regions, but fulfil the other diagnostic criteria for CH and should try ergotamine, oxygen, sumatriptan, verapamil, steroids or even combinations in these patients.

Some controversies about type and nature of aphasic symptomatology in Landau-Kleffner's syndrome: a case study.

The syndrome of acquired aphasia and convulsive disorders in childhood has received ample discussion since the initial description by Landau and Kleffner in 1957. Despite numerous investigations considering the linguistic and epileptic phenomena of the syndrome, the symptomatology still remains of a puzzling nature. Two aspects of the syndrome, the acquired nature of the aphasia and the type of linguistic symptomatology, have not been discussed thoroughly. In our case not only a history of developmental language pathology was present but language symptomatology also revealed a predominantly motor aphasia during a language deterioration phase. A careful review of the literature as presented here yields data in agreement with our findings. First we found further evidence in the literature that Landau-Kleffner's syndrome is not exclusively a primarily receptive language disturbance. Secondly, evidence in favour of a developmental type of Landau-Kleffner's syndrome was found in 25 additional cases. In this group of children with developmental language disturbances at least two distinct types seem to emerge. A group in which the already existing developmental language disturbances did not fluctuate during the course of the illness and a group in which a considerable deterioration of the developmentally disturbed language functions took place. The different patterns of language evolution not only seem to suggest the possibility of an acquired language pathology within a context of developmental language disturbances but also a congenital onset of the syndrome of Landau-Kleffner as the probable cause of developmental language pathology.

Single-dose and steady-state pharmacokinetics of sabeluzole in senile dementia of Alzheimer type patients.

The single- and repeated-dose pharmacokinetics of sabeluzole have been determined in six elderly patients with [senile] dementia of the Alzheimer type. After a single oral dose of 10 mg sabeluzole, the peak plasma concentration was attained at 1 to 4 h; it averaged 42 ng.ml-1. On repeated dosing (10 mg b.d.), steady-state was virtually attained after 3 days of treatment. Steady-state mean trough and peak plasma concentrations fluctuated between 53 and 94 ng.ml-1. The mean terminal half-life after a single dose and at steady-state was of the order of 33 h. Sabeluzole was well tolerated and at the end of treatment, no systematic changes in blood haematology, biochemistry or urinalysis were seen.

Evolution of technetium-99m-HMPAO SPECT and brain mapping in a patient presenting with echolalia and palilalia.

A 78-yr-old woman presented with transient echolalia and palilalia. She had suffered from Parkinson's disease for 2 yr. Routine laboratory examination showed hypotonic hyponatremia, but was otherwise unremarkable. Brain mapping revealed a bifrontal delta focus, more pronounced on the right. Single photon emission computed tomography (SPECT) of the brain with technetium-99m labeled d,l hexamethylpropylene-amine oxime (99mTc-HMPAO), performed during the acute episode showed relative frontoparietal hypoactivity. Brain mapping performed after disappearance of the echolalia and palilalia, which persisted only for 1 day, was normal. By contrast, SPECT findings persisted for more than 3 wk. Features of particular interest in the presented patient are the extensive defects seen on brain SPECT despite the absence of morphologic lesions, the congruent electrophysiologic changes and their temporal relationship with the clinical evolution.

Slow progressive bilateral posterior artery infarction presenting as agitated delirium, complicated with Anton's syndrome.

Three patients presented with an acute agitated delirium as the earliest sign of bilateral posterior cerebral artery infarction. All patients showed a unique slow progressive deterioration with a remarkably long interval between the first neuropsychological and subsequent visual and neurological symptoms, ranging from 3 to 30 days. Repeated CT scans demonstrated hypodensities in the posterior artery territory only after a long interval of 9-12 days, in case 3, and between 33 and 48 days in case 2. In the latter case MRI was still negative 33 days after onset. In 2 patients the cortical blindness was complicated with anosognosia for blindness. Clinical condition worsened progressively in all patients, leading to death, probably due to brainstem infarction. In all 3, the combination of clinical and radiological findings indicated a 'top of the basilar' distribution, which could be confirmed in two by autopsy.

Methanol intoxication. Parkinsonism and decreased Met-enkephalin levels due to putaminal necrosis.

Due to methanol intoxication, a 38-year-old male developed permanent parkinsonism with visual impairment. Brain computerized tomography (CT) and magnetic resonance imaging (MRI) revealed cystic resorption of the putamen. An alteration of the central noradrenergic activity and the opioid system can be assumed because of increased dopamine beta-hydroxylase (D beta H) activity and decreased methionine-enkephalin (Met-Enk) levels in the cerebrospinal fluid (CSF).