M. Grasso

Occipital neuralgia as isolated symptom of an upper cervical cavernous angioma

Sirs: Occipital neuralgia (ON) (Arnold’s neuralgia) is characterized by jabbing pain attacks in the territory of occipital nerves associated to hypesthesia/dysesthesia in the same area [1, 2]. Pain frequently radiates to the frontal region and may be associated with tenderness over the affected nerve at the superior nucal line. ON can be caused by trauma, injury, inflammation, or compression of the greater occipital nerve somewhere along its course from the C2 dorsal root to the peripheral territory. A 59-year-old woman was referred to us in May 1999 because of a five-year history of pain involving the right occipito-parietal scalp, often radiating to frontal and retroauricular regions. The pain was sharp and burning with paroxysms triggered by pressure at the emergence of the greater occipital nerve and hyperalgesia for brush, touch and pinprick stimuli in the right occipital as well as in the auricular and retroauricular region. Skin rubbing at those levels caused paroxysmal attacks (for example when she held the telephone receiver over the right ear or put the right side of her head on a cushion). She complained of almost continuous uncomfortable paresthesias in the occipito-nucal area. Head and neck trauma were not reported. No clinical or laboratory evidence of rheumatologic disorders were present. Neurologic examination revealed only a slight hyperreflexia in the right limbs, without other signs of myelopathy. MRI showed a right intramedullary multilobated lesion at C1-C2 level (Figs. 1, 2) with a mild contrast enhancement. The lesion was slightly hyperdense on CT. Angiography of vertebral, common carotid and cervical branches from the subclavian arteries was normal . Therefore a diagnosis of intramedullary cavernous angioma was hypothesized. Amitryptiline and carbamazepine but not NSAIDs and greater occipital nerve anesthetic blockade were useful in reducing the pain. No change was evident on MRI performed 6 months later and the clinical picture was unchanged at the last visit in July 2001. The present case broadens the spectrum of causes of ON and suggests that spinal cord imaging may be useful also without clinical evidence of myelopathy. Indeed, in our patient ON was the unique symptom of an intramedullary cavernous angioma. About 5–10 % of central nervous system cavernous angiomas arise within the spinal cord and the cervical level is the most frequent after the thoracic [3]. They usually cause a progressive myelopathy through gradual enlargement and repeated microhemorrhages [3]. In ON pain and sensory symptoms involve the distribution of the branches derived from C2 spinal nerve. Just immediately after the origin the C2 spinal nerve gives rise to a ventral and a dorsal ramus. The greater occipital nerve raises from the dorsal ramus, enters the scalp through the trapezius aponeurosis at the superior nucal line and innervates the skin of the occipital area up to the coronal suture [1–5]. The lesser occipital nerve emerges from the C2 ventral ramus and supplies the scalp over the mastoid and the posterior surface of the ear. The C2 spinal nerve has several peculiarities [5]. It does not pass through an intervertebral foramen and therefore is relatively LETTER TO THE EDITORS

Atherosclerotic adult Moya-Moya disease in a patient with hyperhomocysteinaemia

Moya-Moya is a rare cerebrovascular occlusive disease characterized by bilateral stenosis or occlusion at the terminal portion of the internal carotid artery and abnormal vascular network at the base of the brain, named “moya-moya”. In children, Moya-Moya disease usually presents with ischemic cerebrovascular events, mainly TIA or lacunar stroke, leading to mental deterioration. In adults, especially in females, it presents with intracranial haemorrhages. We describe the case of an adult patient with an atherosclerotic Moya-Moya disease which presented with a cerebral borderzone infarction.

Restricted Dissociated Sensory Loss in a Patient With a Lateral Medullary Syndrome: A Clinical-MRI Study

Background Various sensory syndromes in lateral medullary infarctions are described. A small variation in the location of a lesion may lead to very different clinical features, owing to the complex anatomy of the medulla oblongata. MRI may identify the location and extent of the ischemic lesions, allowing a clear clinical-anatomical correlation. Case Description We describe a man with an ischemic lesion in the right portion of the lower medulla that presented a contralateral impairment of spinothalamic sensory modalities and an ipsilateral impairment of lemniscal modalities with a restricted distribution (left forearm and hand, right hand and fingers, respectively). The restricted and dissociated sensory abnormalities represent the only permanent neurological consequence of that lesion. Conclusions The atypical sensory syndrome may be explained by the involvement of the medial portion of spinothalamic tract and the lateral portion of archiform fibers at the level of the lemniscal decussation.

Pseudo-ulnar sensory loss in a patient from a small cortical infarct of the postcentral knob.

A wide spectrum of sensory syndromes with different topographic patterns have been described in patients with cerebral infarction.1–4 The sensory abnormalities may be limited to small areas, mimicking a radicular or trunk distribution, mainly for lesions in sensory cortex and thalamus.nnA 31-year-old man suddenly had a right arm pain, numbness, and burning sensation on his right forearm and hand, predominant on the ulnar side. He also reported an expressive aphasia that resolved in a few minutes. There was no history of previous strokes. Two hours later, at admission, neurologic examination revealed a full sensory loss for pinprick and temperature modalities restricted to the V digit, the lateral side of the IV digit, the lateral palm, and the dorsal area of his right hand (figure, A and B). Lemniscal sensory modalities were normal. The hypoesthesia strictly coincided with the sensory distribution of the ulnar nerve. Moreover, the patient had a fastidious hyperalgesia for brush, touch, and pinprick stimuli. There was no evidence of motor impairment; specifically, the ulnar-dependent movements were normal. The rest of the …

Palatal myoclonus in a patient with a lateral thalamic infarction

Palatal myoclonus (PM) is characterized by rhythmic involuntary jerking movement of the soft palate and the pharyngopalatine arch, often involving the diaphragm and laryngeal muscles.1nnThe two distinct clinical entities, symptomatic PM and essential PM, arise from different pathophysiologic mechanisms.2nnSymptomatic PM is usually associated with lesions located in the myoclonic triangle (the Guillain–Mollaret triangle). It consists of the red nucleus, inferior olive, contralateral cerebellar dentate nucleus, and the connecting pathways, namely the central tegmental tract, the inferior cerebellar peduncle, and superior cerebellar peduncle.3 Symptomatic PM may be isolated or more rarely associated with movements of the extremities such as tremor, choreoathetosis, and myoclonus.4,5nnA 64-year-old man was referred for acute onset of motor impairment of the right arm. On admission neurologic examination revealed clumsiness of the right arm and right dysmetria on finger-to-nose test. On the fifth day the patient developed myoclonic movements …

Inherited Thrombophilic Conditions, Patent Foramen ovale and Juvenile Ischaemic Stroke

Ischaemic stroke of ‘undetermined aetiology’ is not rare in young people [1, 2]. In these patients, transoesophageal echocardiography (TEE) often detects atrial septal abnormalities, such as patent foraFig. 1. Axial T2-weighted brain MRI scan. Areas of altered signal intensity consistent with ischaemic lesions are bilaterally evident in frontal and parietal lobes at the corticalsubcortical level.

Internal carotid artery dissection after mandibular third molar extraction

Sirs: Cervicocephalic arterial dissection (CAD) is an important cause of cerebrovascular accidents in young and middle-aged patients [1]. CAD may be caused by major non-penetrating trauma of the cervical carotid and vertebral arteries; it more frequently occurs spontaneously or in association with trivial trauma causing hyperextension or rotation of the neck [1–4] . The activities include practicing yoga, sport activity, violent coughing and vomiting, sneezing, vigorous noseblowing, sexual activity, chiropractic manipulations, anaesthesia administration, car pushing, neck-turning while leading a parade, rhythmical flexion and extension of the neck to music, prolonged telephone usage with flexion in the neck and childbirth [1–4]. We describe a patient with a left parietal infarction caused by extracranial carotid artery dissection occurred after a removal of the left mandibular third molar. A 47-year-old woman had a sudden onset of a right visual field defect and slight sensorimotor impairment involving her right arm. For two weeks she had been affected by a periodontal infection, unsuccessfully treated with antibiotics. Six days before the stroke onset she underwent a difficult left lower third molar extraction. No direct trauma related to the dentist’s instruments was reported. After surgery the dental pain spread to the entire head on the left side. Neurological examination on admission revealed a right homonymous hemianopia and a mild sensory impairment in the right hand. Brain MRI revealed a parieto-occipital infarction in the territory of a parietal branch of the middle cerebral artery and an intramural haemorrhage in the intrapetrous portion of the left internal carotid artery (Fig. 1A). Duplex ultrasonography of the carotid arteries was normal. Conventional angiography revealed an irregular stenosis of the left internal carotid artery extending from the carotid bulb to the base of the skull (Fig. 1B); the contralateral carotid artery was normal, while both the vertebral arteries appeared tortuous. Conventional vascular risk factors as well as clinical conditions predisposing to arterial dissection were absent. In particular, features of type IV Ehlers-Danlos syndrome, Marfan’s syndrome, pseudoxanthoma elasticum, osteogenesis imperfecta type I and systemic lupus erythematosus were absent. A wide variety of physical stresses causing CAD has been described [1–4] but until now no case has been reported as a result of dental extraction. Hyperextension of the neck associated with sudden neck movement due to a difficult third molar extraction is the proposed mechanism causing the carotid dissection in our patient. Cervical extension and sudden neck movement may compress the cervical carotid artery against the transverse processes or the bony mass of the upper cervical vertebrae [4]. During dental extraction the opening of the mouth reduces the distance between mandibular angle and upper cervical vertebrae thus favouring the compression of the cervical carotid artery. The mechanical stretching of the soft neck tissue may cause the rupture of the intima and media tunica with LETTER TO THE EDITORS

Emotional facial paresis in a patient with a lateral medullary infarction.

Emotional facial paresis (EFP) is characterized by a weakness of inferior facial muscles evident during emotionally evoked movements but not during voluntary contraction. EFP is described in patients with lesions of the supplementary motor area, basal ganglia, temporal lobes, frontal white matter, anterolateral thalamus, anterior limb of the internal capsula, and brainstem tegmentum.1,2⇓ Voluntary facial paresis (VFP) is caused by lesions of the motor cortex or descending pyramidal tract at the level of the corona radiata, posterior limb of the internal capsula, ventral mesencephalon, and ventral pons.1 We describe a patient with EFP due to a small lesion in the upper medulla oblongata.nnA 60-year-old man presented with sudden onset of vertigo, dizziness, dysphagia, hoarseness, and facial paresis. He had hypercholesterolemia and a family history of stroke. Admission neurologic examination found mild gait ataxia, left Horner’s syndrome, hiccups, and hypesthesia for tactile and thermic sensation involving arm and trunk on …

Hypogeusia and hearing loss in a patient with an inferior collicular infarction

Various vascular syndromes have been described in patients with midbrain infarction.1,2nnThe clinical picture is dominated by third-nerve palsy, conjugate or disconjugate gaze impairment, contralateral motor and sensory deficit and homolateral or contralateral limb ataxia. We describe a patient with a pontomesencephalic tegmental infarction with hearing loss and hypogeusia as dominant features.nnA 52-year-old man had dizziness and loss of taste while eating dinner. The next morning upon awakening, he developed sensory changes of the left half of his face, clumsiness of the right arm and leg, and sensation of bilateral ear wadding. At admission, neurologic examination revealed right limb ataxia, decreased pinprick and touch sensation over the left half of his face, and bilateral hypoacusia, more marked on the left side. There was a marked diminution of taste on the left side of the tongue. His taste was examined by application of 0.5 M NaCl, 10% glucose, 0.2 M acetic acid, and 0.02 M quinine. The rest of the neurologic examination was normal. Limb ataxia and left face hypoesthesia gradually disappeared, and 10 days later only left tongue hypogeusia …

Restricted pain and thermal sensory loss in a patient with pontine lacunar infarction : a clinical MRI study

Pure sensory syndrome (PSS) is characterized by hemisensory symptoms without other major neurological signs. It was initially attributed to thalamic lacunar infarction, but several reports have shown the PSS can be due to small infarcts involving the posterior part of the internal capsula, the cerebral cortex and the brainstem. Paramedian and lateral pontine infarctions are associated respectively with lemniscal and spinothalmic (ST) sensory impairment. We describe a patient with an isolated impairment of the ST modalities caused by a segmental paramedian pontine infarction.