Trigeminal cephalgias and facial pain syndromes associated with autonomic dysfunction.

Trigeminal autonomic cephalgias (TACs) include a spectrum of primary headache syndromes associated with cranial autonomic dysfunction. Other types of headache and facial pain syndromes can be associated with marked localized facial or ear autonomic changes. We report on a group of patients suffering from episodic migraine with cranial autonomic features, patients with different presentations of the 'red ear syndrome' (RES), cluster headache with prominent lower facial involvement and crossover cases. In our experience crossover between TACs and migraine, RES and cluster headache is not uncommon. We propose that all these conditions belong to the same group and a unifying causative mechanism is proposed.

Epidemiology of multiple sclerosis in Arabs in Jordan: a comparative study between Jordanians and Palestinians.

In a 2-year hospital-based study in Jordan 131 Arab multiple sclerosis patients were identified including 84 Palestinians and 36 Jordanians. Based on MS/ALS case ratio, multiple sclerosis was found to be twice as common among Palestinians than Jordanians. Other than the less marked female preponderance among Jordanian patients, the disease had the same clinical and paraclinical characteristics in both groups. It was more likely for Palestinian and Jordanian patients to originate from the northern parts of their countries, to be Rh negative and to be HLA-DR2 positive than their controls. Palestinians (patients and controls) did not show significant differences from Jordanians (patients and controls) in relation to their eye color, ABO and Rh blood groups distribution nor the HLA-DR or HLA-DQ (apart from HLA-DQ3) epitopes frequency, thus not offering any significant difference in the genetic-racial markers studies to explain the difference in the observed disease susceptibility. Previous studies demonstrated that 2 racially different populations sharing the same environment can have different risk of developing multiple sclerosis, but this study has shown that this can also be true for 2 racially similar populations sharing the same environment.

Multiple sclerosis in Arabs in Jordan.

In a 2-year hospital-based study (1992 and 1993), there were 131 multiple sclerosis patients attending 2 large referral hospitals in Jordan. Based on MS/ALS case ratios an overall rate of 32.1 (95% CI 19.7-55.2) was estimated. There were 126 Arabs of whom 84 were Palestinians and 36 indigenous Jordanians. Comparison of these subgroups, which had a similar age distribution revealed that the disease was twice as frequent in Palestinians (estimated 42.0/100,000 (CI 2.8-90.8)) among Jordanians (estimated 20.0/100,000 (CI 9.5-47.2)). Clinical presentation, pattern of disease, disability and HLA association were similar to that in the disease reported in Caucasians in the West. All investigations including neurophysiology and imaging were also very similar to Western reports.

Risk factors of aseptic intracranial venous occlusive disease.

INTRODUCTION: Risk factors for aseptic intracranial venous occlusive disease are varied but only few epidemiologic studies were performed to verify the relative importance of particular factors. PATIENTS AND METHODS: A 2-year hospital-based prospective study was conducted in two hospitals to identify the clinical characteristics and risk factors of patients with confirmed aseptic intracranial venous occlusive disease. RESULTS: 21 patients were identified, representing 0.9% of the total neurological admissions. Men were more commonly affected than women; 81% of the patients presented in a clinical picture indistinguishable from idiopathic intracranial hypertension. Risk factors included Behçet's disease in 4, the puerperium in 3, thrombophelia in 3, familial Mediterranean fever in 2, malignancies in 1, lupus anticoagulant in 1, and the contraceptive pill in 1. CONCLUSION: Aseptic intracranial venous occlusive disease proved to be not rare in Arabs. It should be considered seriously in the differential diagnosis of idiopathic intracranial hypertension, particularly in males. Several risk factors were incriminated.

Autosomal recessive ataxia, slow eye movements and psychomotor retardation.

Two consanguineous Arab families with an autosomal recessive form of hereditary ataxia with slow eye movements and psychomotor retardation are reported. The ataxia presented in the first decade, was severely disabling and was associated with a spectrum of eye movements abnormalities as well as psychomotor retardation and sensory neuropathy. MRI studies of the brain showed a significant degree of cerebellar and brainstem atrophy. These 2 families support a previous report of a similarly affected consanguineous Arab family. The syndrome of autosomal recessive ataxia and slow or even absent saccades is proposed to be related but not identical to the autosomal dominant form known as the Wadia Swami syndrome, both of which seem to be related to the olivopontocerebellar degenerations.

Pallido-pyramidal degeneration, supranuclear upgaze paresis and dementia: Kufor-Rakeb syndrome.

An unusual neurological syndrome in an Arab family with five affected siblings, is reported. Autosomal recessive inheritance is suggested by having multiple affected siblings born to phenotypically normal consanguineous parents. Similar to Davison's Pallido-pyramidal syndrome, they presented with the clinical signs and symptoms of severe parkinsonism as well as evidence of cortico-spinal tract disease. In addition, they had dementia and supranuclear upgaze paresis. MRI studies showed significant atrophy of the globus pallidus and the pyramids, as well as generalized brain atrophy in later stages. Therapy with levodopa resulted in significant improvement in the extrapyramidal dysfunction. We suggest that this probably represents a new syndrome which is closely related but not identical to the pallido-pyramidal syndrome.

Neuro-ophthalmic manifestations of the syndrome of ophthalmoplegia, ataxia and areflexia. Observations on 20 patients.

The neuro-ophthalmological manifestations of 20 patients with the syndrome of ophthalmoplegia, ataxia and areflexia are described. The symmetrical nature of the ophthalmoplegia and the associated cerebellar ataxia point to centrally placed lesions. Several supranuclear, nuclear and internuclear ophthalmological signs are identified. Some of these, like partial sparing of the levator palpebrae and normal downgaze in the presence of severe ophthalmoplegia are noted too frequently to be just unusual signs of peripheral oculomotor dysfunction. Other identified features included upper lid retraction on attempted upgaze and preserved Bell's phenomenon in the presence of paralysis of the latter, as well as several other central ophthalmological signs. These findings contrast with those seen in the Guillain-Barré syndrome and, thus, the syndrome of ophthalmoplegia, ataxia and areflexia is not a mere variant of it.

Ataxia and areflexia in SOAA.

Fifteen patients with the classical syndrome of ophthalmoplegia, ataxia, and tendon areflexia (SOAA) were studied in an attempt to clarify the mechanisms of ataxia and myotatic hyporeflexia. All showed features of cerebellar rather than sensory ataxia. Peripheral nerve conduction studies, including F-waves, were normal in a majority of the patients, as was needle EMG. Low-amplitude compound sensory nerve potentials were seen in four patients only, and mild slowing of sensory conduction velocity in two. Three had abnormal blink reflex studies, suggestive of a central lesion in two, and another two showed a transient delay of N5 peak of brainstem auditory evoked potentials. Somatosensory evoked potentials were normal. Despite clinically depressed or absent tendon jerks, T-waves were elicited at normal latencies. These findings do not support the prevailing view that the neurological abnormalities in SOAA are due to involvement of sensory fibres in the peripheral nerves and dorsal roots. We suggest that lesions scattered in the brainstem tegmentum and in the cerebellar peduncles are responsible for the ataxia and the depressed tendon jerks.

Macrocytosis in multiple sclerosis. A study in 82 de novo Arab patients.

Macrocytosis, without anaemia, was common in 82 de novo multiple sclerosis patients compared with a similar number of age and sex matched controls. This was an early phenomenon in the course of the disease and was not influenced by the age of the patients nor the duration of the disease. None of the patients proved to have pernicious anaemia, yet the similarity in the geographical and sex distribution as well as the similarity in HLA associations of multiple sclerosis and pernicious anaemia may indicate that both diseases are under similar genetic influence.

Epidemiology of multiple sclerosis in Arabs in Kuwait: a comparative study between Kuwaitis and Palestinians.

On December 31, 1988 there were 201 registered multiple sclerosis patients in Kuwait, an overall prevalence rate (PR) of 10.2 per 100,000; among them were 186 Arabs, of whom 72 were Palestinians and 51 Kuwaitis. Comparison of these two subgroups, who had a similar age distribution revealed that the disease was 2 1/2 times more frequent among Palestinians (PR 23.8/100,000) than among Kuwaitis (PR 9.5/100,000). Palestinians also showed significant differences from Kuwaitis in eye color, blood group distribution and HLA-DR and HLA-DQW epitopes frequency. This suggests that genetic rather than environmental factors might be the underlying cause for the high susceptibility to develop MS among Arabs originating from the Eastern Mediterranean basin.

Autosomal recessive ataxia, slow eye movements, dementia and extrapyramidal disturbances.

An Arab family with an autosomal recessive form of spinocerebellar degeneration with slow eye movements is reported. Hitherto all the reported cases were either sporadic or of autosomal dominant inheritance. Associated are progressive intellectual impairment and extrapyramidal dysfunction as well as peripheral neuropathy and skeletal abnormalities. Muscle biopsy revealed non-specific mitochondrial abnormalities. The spectrum of eye movement abnormalities is discussed and the literature is reviewed. It is concluded that the hallmark of this syndrome (slow or even absent saccades) is one of a group of oculomotor abnormalities, all being characterized by delayed initiation and slow velocity. The syndrome seems to be related to the olivopontocerebellar degenerations, but differs in that there is in addition selective degeneration of certain tracts and nuclei in the mesencephalon and probably more rostral structures.

Late onset cerebello-pontomesencephalic degeneration.

Two siblings are presented with late onset, rapidly progressive truncal ataxia, paralysis of down-gaze and loss of up-gaze saccades in association with other oculomotor dysfunctions as well as dementia. Electron microscopic muscle studies revealed abnormal distribution and form of the mitochondria, probably being the ultrastructural basis of the pathologic changes. A neurological syndrome as that described here has not been reported before.

Brainstem auditory evoked potential (BAEP) abnormalities in brucellosis.

Twelve patients with neurobrucellosis and 17 patients with systemic brucellosis without neurological involvement underwent a brainstem auditory evoked potentials (BAEP) study. All neurobrucellosis patients (100%) showed abnormalities in their BAEP recordings, suggestive of brainstem lesions at various levels. On the other hand, only 5 (29%) of the 17 patients with systemic brucellosis had mild unilateral BAEP abnormalities, while the remaining 12 had normal responses. Comparison of pooled data between the systemic brucellosis and neurobrucellosis groups showed highly significant differences in all BAEP parameters. The recording of BAEP is thus considered a sensitive supplementary method to reveal CNS lesions in patients with neurobrucellosis.

The nosological position of the ophthalmoplegia, ataxia and areflexia syndrome: "the spectrum hypothesis".

Two cases of ophthalmoplegia ataxia and areflexia are described, each with undoubted central and peripheral neural affection. It is concluded that the cardinal features are due to brainstem pathology, and support the hypothesis that this syndrome and the post-infectious polyradiculoneuritides represent differing ends of a spectrum which reflects two modes of nervous system reaction to a presumed infective challenge.

Clinical categories of neurobrucellosis. A report on 19 cases.

Brucellosis rarely can present with involvement restricted to the nervous system. We describe a total of 19 cases of neurobrucellosis in whom the clinical presentation lay in three distinct categories. The first was an acute presentation with meningoencephalitis. The disease also presented in a chronic form where the brunt of the illness can either be in the peripheral or the central nervous system (CNS). The chronic peripheral form is that of a proximal polyradiculoneuropathy. The central form is that of diffuse CNS involvement, predominantly with myelitis or cerebellar involvement with or without cranial nerve palsies. Although the two chronic forms, 'peripheral' and 'central', are distinct, some overlap is possible. This was not observed for the acute form. The pathology of the three presentations may be different, being a direct effect of infection in the acute form, and an immune-related process, possibly demyelinating in nature, in the chronic forms. The response to treatment in the acute and chronic forms is also different, being much better in the acute form. Awareness of the condition and performance of the appropriate serological tests will differentiate neurobrucellosis from other chronic CNS infections, especially tuberculosis and neurosyphilis.

Autosomally inherited recessive spastic ataxia, macular corneal dystrophy, congenital cataracts, myopia and vertically oval temporally tilted discs. Report of a Bedouin family--a new syndrome.

A Bedouin family is described with an unusual form of spinocerebellar degeneration. Spastic ataxia was found to be associated with congenital cataracts, macular corneal dystrophy and non-axial myopia, in the absence of retardation of somatic or mental maturation. Immunological abnormalities were common. Genetic analysis revealed that the pedigree is expressing the transmission and segregation of a single mutant autosomal recessive gene.