Unilateral periorbital pain, cranial nerve palsies and a dramatic response to corticosteroid therapy are the hallmarks of clinical presentation in Tolosa-Hunt-Syndrome (THS) and Idiopathic Pseudotumor of the Orbit (IIPO). Both are unspecific chronic granulomatous diseases of unknown origin, sharing clinical as well as paraclinical characteristics. We observed two patients suffering from acute granulomatous IIPO, who also fulfilled the criteria of THS. Patient 1 developed leftsided infiltration of the medial ocular muscle with periorbital pain and cranial nerve palsy. After an initial response to corticosteroid therapy, contralateral relapse occurred with a THS-like infiltration of the sinus cavernosus and narrowing of the intracavernous internal carotid artery. Granulomatous infiltration of the right sinus cavernosus with secondary involvement of the ipsilateral nervus opticus and a slight exophthalmos was seen in Case 2. According to the literature, MRI and CT show identical signal intensity with different localisation: IIPO preferentially intra- and THS retroorbital. Apart from neuroradiological findings, almost similar histopathology and clinical presentation makes it difficult to distinguish between these two syndromes. Similarities between these two syndromes have been discussed for more than 20 years. Our two cases show their close relationship and we suggest that both diseases belong to the same pathological process.
Involvement of the PNS is more uncommon than cerebral ischemia and neuroophthalmological complications in patients suffering from GCA. Severe PNS involvement has an affinity to the midcervical nerve roots and the brachial nerve plexus.
Administration of sumatriptan in subarachnoid haemorrhage (SAH) patients, misdiagnosed as migraine patients, may induce symptomatic cerebral vasospasm with potentially dangerous consequences. Over a 5-year period, we observed three patients with a 3-15-year history of migraine, who received sumatriptan for acute headache. Two patients received 6 mg sumatriptan subcutaneously on days 4 and 6, and one patient 3 x 100 mg sumatriptan orally on day 1 after an acute headache episode. In all three cases, an alleviation of headache intensity from severe to moderate was observed. When headache recurred and meningeal signs appeared, SAH was diagnosed by computed tomography in all three cases. No neurological deficits occurred during the further course of the disease. In both patients with a SAH caused by an aneurysm, transcranial Doppler sonography demonstrated vasospasm of the basal cerebral arteries. An antinociceptive effect of sumatriptan can be observed in SAH patients in good clinical condition, which suggests a specific craniovascular antinociceptive action. This may lead to misdiagnosis as migraine and delayed appropriate diagnosis and treatment.
Spinal symptoms in acute bacterial meningitis are rare. In a series of 10 cases of neurolisteriosis, we observed 2 spinal complications, one due to an acute intramedullary abscess, the other caused by chronic spinal arachnoiditis. Therefore, if spinal symptoms develop in acute bacterial meningitis, Listeria monocytogenes infection should be considered and early adequate antibiotic treatment be implemented.
Hearing disorders are a well-described symptom in patients with multiple sclerosis (MS). Unilateral or bilateral hyperacusis or deafness in patients with normal sound audiometry is often attributed to demyelinating lesions in the central auditory pathway. Less known in MS is a central phonophobia, whereby acoustic stimuli provoke unpleasant and painful paresthesia and lead to the corresponding avoidance behaviour. In our comparison collective, patient 1 described acute shooting pain attacks in his right cheek each time set off by the ringing of the telephone. Patient 2 complained of intensified, unbearable noise sensations when hearing nonlanguage acoustic stimuli. Patient 3 noticed hearing unpleasant echoes and disorders of the directional hearing. All patients had a clinical brainstem syndrome. ENT inspection, sound audiometry and stapedius reflex were normal. All three patients had pathologically changed auditory evoked potentials (AEPs) with indications of a brainstem lesion, and in magnetic resonance imaging (MRI) demyelinating lesions in the ipsilateral pons and in the central auditory pathway. The origin we presume in case 1 is an abnormal impulse conduction from the leminiscus lateralis to the central trigeminus pathway and, in the other cases, a disturbance in the central sensory modulation. All patients developed in the further course a clinically definite MS. Having excluded peripheral causes for a hyperacusis, such as, e.g., an idiopathic facial nerve palsy or myasthenia gravis, one should always consider the possibility of MS in a case of central phonophobia. Therapeutic possibilities include the giving of serotonin reuptake inhibitors or acoustic lenses for clearly definable disturbing frequencies.
We found that not only specific neurological disorders, but also earlier symptoms are related to impaired functioning and predict mortality in the elderly. These findings have potential clinical relevance for screening and early detection of individuals at risk.
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