The medullary vascular syndromes revisited

There are two major vascular syndromes of the medulla oblongata: the medial and the lateral. The medial medullary syndrome is characterized by the triad of ipsilateral hypoglossal nerve palsy with contralateral hemiparesis and loss of deep sensation. Lateral medullary infarction commonly presents with Homer's syndrome, ataxia, alternating thermoanalgesia, nystagmus, vertigo and hoarseness. Combinations of the two major syndromes occur as bilateral medial medullary, hemi-medullary and bilateral lateral medullary syndromes. Each of these syndromes frequently manifests with incomplete or atypical findings depending on the extent of the lesion. Magnetic resonance imaging has been useful in the clinical diagnosis of medullary infarctions. The site of the lesion may help predict the arteries involved.     

Magnetic resonance imaging of medial medullary infarction

Medial medullary infarction is characterized by ipsilateral hypoglossal nerve palsy, contralateral hemiparesis sparing the face, and contralateral disturbance of deep sensation. Although it is possible to make a clinical diagnosis in typical patients, diagnosis is difficult if hypoglossal nerve palsy is absent. We describe a patient with medial medullary infarction without hypoglossal nerve palsy. The patient suffered from left hemiplegia and homolateral disturbance of deep sensation. Magnetic resonance imaging revealed the site of the lesion to be in the medial portion of the upper medulla oblongata. The result of somatosensory evoked potential testing was compatible with disturbance of the medullary medial lemniscus. In a review of the literature, we examined the relation between clinical features and lesion location in 16 patients with medial medullary infarction and compared these to the present patient. Motor paresis was present in every patient, while disturbance of deep sensation was recorded in nine of 13 patients and hypoglossal nerve palsy in six of 14 patients. In atypical patients with medial medullary infarction (such as the present patient), magnetic resonance imaging is necessary to detect the lesion and to make a clinical diagnosis.     

Sensory symptoms in ipsilateral limbs/body due to lateral medullary infarction

OBJECTIVES: To characterize the incidence, topography, and radiologic and pathophysiologic findings of ipsilateral sensory symptoms in the limbs/body in patients with lateral medullary infarction. METHOD: Between 1994 and 2001, the author identified 12 patients with lateral medullary infarction (6.7% of all lateral medullary infarction patients) who presented with ipsilateral sensory symptoms in the limbs/body in addition to typical lateral medullary syndrome. Brain MRI, nerve conduction velocity, and electromyographic studies were performed. Twenty-four patients without ipsilateral sensory symptoms were included as a control group. Clinical and radiologic findings were compared between the two groups. RESULTS: The ipsilateral sensory symptoms were generally described as numbness or tightness, predominantly affecting the upper extremities, especially distal fingers. Vibration and proprioceptive sensation were occasionally impaired. None showed evidence of peripheral neuropathy or radiculopathy. The patients with ipsilateral sensory symptoms significantly more often had vertigo, nausea/vomiting, severe gait ataxia, hiccup, ipsilateral hemiparesis, and caudally located lesions than those without. The caudal lesions producing ipsilateral sensory symptoms tended to extend dorsomedially. CONCLUSION: Lateral medullary infarction associated with ipsilateral sensory symptoms in the limbs/body is an uncommon but distinct variant caused by caudal lesions extending dorsomedially that probably involve the ipsilateral dorsal column or decussating lemniscal fibers.     

Spectrum of medial medullary infarction: clinical and magnetic resonance imaging findings

Among 4200 consecutive patients admitted to three hospitals with acute ischemic stroke, we found only 11 patients in whom magnetic resonance imaging (MRI) had proved that they had medial medullary infarction (MMI). In our centers, patients with MMI were less than 1 % of those with vertebrobasilar stroke. The infarcts documented by MRI were unilateral in 10 patients and bilateral in one. On clinico-topographical analysis there were four clinical patterns: (1) Classical Dejerine's syndrome was the most frequent, consisting of contralateral hemiparesis, lemniscal sensory loss and ipsilateral lingual palsy in 7 of the eleven patients. (2) Pure hemiparesis was present in 2 patients; (3) Sensorimotor stroke was present in 1 patient with contralateral hemiparesis, hypesthesia and mild decrease in pain sensation without lingual palsy; (4) Bilateral MMI syndrome in 1 patient, accompanied by tetraparesis, bilateral loss of deep sensation, dysphagia, dysphonia and anarthria. Presumed causes of MMI were intrinsic branch penetrator artery disease with concomitant vertebral artery stenosis in 6 of the 11 patients, vertebral artery occlusion in 2, dolichoectatic vertebrobasilar arteries in 2, a source of cardiac embolism in 1. Prognosis at 3 months was favorable in 8 patients, but the patient with bilateral MMI syndrome had persisting motor deficit causing limitation of daily activities, and 2 died from systemic causes. The classical triad of acute MMI facilitates the diagnosis, although the recognition of this syndrome in patients with incomplete manifestations can be difficult and occurs more frequently than commonly thought. Moreover, vertebral artery atherosclerosis and branch atheromatous disease of the penetrating arteries are the main causes of medullary infarction. Received: 21 February 2001, Received in revised form: 22 February 2001, Accepted: 17 May 2001     

Medial medullary syndrome with contralateral face hypalgesia: A report of two cases.

Classically, patients with unilateral medial medullary syndromes show contralateral deep sensory loss, contralateral hemiparesis, and ipsilateral tongue paralysis. We encountered two patients with medial medullary syndromes showing hypalgesia of the contralateral face. Both patients had contralateral deep sensory loss and hemiparesis, but no hypoglossal nerve palsy, so it was difficult to establish a medial medullary syndrome from the clinical neurological signs alone. Magnetic resonance images showed that the infarcted areas were located in the ventromedial area of the upper medulla, probably involving the trigeminothalamic tract in the medial lemniscus. We reviewed the reported cases of medial medullary syndromes and summarized their clinical features as well as the topography concerned with the associated sensory disturbances.     

Opalski syndrome detected on DWI MRI: A rare lateral medullary infarction. Case report and review

Introduction

Wallenberg's syndrome and ipsilateral paresis due to combined infarction of the lateral medullary and cervical spinal infarction is known as Opalski syndrome. This rarely described syndrome was reported, to our knowledge, with DWI MRI, only once.

Case report

We report the case of a 43-year-old man with autosomal dominant polycystic kidney disease who, after a brief episode of coma, developed Wallenberg syndrome and ipsilateral hemiparesis. Initial diffusion weighted-imaging MRI showed a high-intensity signal involving the lateral medulla oblongata and the spinal cord; but FLAIR MRI sequences showed bilateral high-intensity signals in the lateral medulla oblongata and spinal cord and high-intensity signals in the right and left cerebellar hemisphere in the PICA territories. MRI performed one year later showed an infarction involving the left medullary area and adjacent spinal segments alone.

Conclusion

This observation illustrates a rare syndrome of lateral medullary infarction, associated with spinal cord infarction related to a possible transient basilar occlusion.     

Medial medullary infarction demonstrated by MRI

A 67-year-old woman with medial medullary infarction is reported, including clinical manifestations, MRI and angiographical findings, and results of evoked potentials. She suffered from contralateral hemiplegia and disturbance of deep sensation. Motor paresis of the tongue was absent. Magnetic resonance imaging revealed a lesion in the medial portion of the medulla oblongata. The 17 cases previously reported with medial medullary infarction are reviewed. Only 3 cases had triad of medial medullary infarction, contralateral hemiparesis, deep sensory disturbance, and ipsilateral hypoglossal paresis. Therefore, lesion detection is necessary to diagnose medial medullary infarction. Most infarctions limited to the upper third of the medulla were caused by occlusions of vertebral arteries or their branches and prognosis was good. In contrast, infarctions in the lower two thirds were caused by occlusions of anterior spinal arteries and their branches and the prognosis was poor. Thus localization of the lesion using MRI plays an important role to predict the prognosis.     

A case report of central respiratory failure due to hemimedullary syndrome]

A hemimedullary infarction, in which both medial and lateral medullary infarctions occur simultaneously, is a rare cerebrovascular disease. Pontomedullary lesions often cause central respiratory failure, and the majority of central respiratory failures are due to bilateral pontomedullary lesions. We report a 66-year-old man with central respiratory failure due to a hemimedullary infarction detected by magnetic resonance imaging. He was admitted to our hospital on March 7, 1998, because of a sudden onset of dysarthria, and both numbness and weakness on his left side. Soon after arriving at the hospital, his spontaneous respiration ceased. Therefore, he was intubated and artificial ventilation was started. Pertinent neurological abnormalities on admission consisted of dysarthria, dysphagia, right Horner's sign, right gaze evoked horizontal nystagmus, right soft palate palsy, and tongue deviation to the right. In addition, left hemiparesis, left Babinski's sign, sensory impairment on the left side including the face, and central respiratory failure were noted. Although voluntary respiration recovered in 12 days, sleep apnea continued for 5 months, which was considered to be due to the automatic respiratory failure. An important feature of this patient was that the hemimedullary infarction caused the central respiratory failure. To our knowledge, this is the third patient whose central respiratory failure occurred because of a hemimedullary infarction.     

The hemimedullary syndrome: case report and review of the literature

Summary Hemi-infarction of the medulla causes the clinical constellation of symptoms and signs of both the lateral and medial medullary syndromes and nearly always results from occlusion of an intracranial vertebral artery. In the case reported here, with a clinical diagnosis of hemimedullary syndrome, the expected infarction was imaged by magnetic resonance. A review of the literature confirms that the hemimedullary syndrome, in which both medial and lateral syndromes occur simultaneously, is extremely rare, since it yielded only two previous cases with adequate anatomical confirmation.     

Contralateral pharyngeal paralysis caused by medial medullary infarction

A patient with unilateral upper medial medullary infarction presented with contralateral paralysis of the pharyngeal constrictor muscle in association with lemniscal sensory loss, pyramidal insufficiency, and central facial palsy on the same side. Individual differences in supranuclear control of the pharyngeal muscles may explain this rare occurrence. The combination of these signs is described as a syndrome of upper medial medullary lesion.     

Sensory dermatomal representation in the medial lemniscus

BACKGROUND: Restricted sensory deficits along the somatotopic topography of the medial lemniscus rarely develop in medial medullary infarction. We describe a patient with medial medullary infarction who presented with dermatomal sensory deficits caused by a medial lemniscal lesion. CASE DESCRIPTION: A 58-year-old man presented with sudden right-sided hemiparesis and paresthesia. He had noticed the paresthesia below the level of the right L5 dermatome, where his vibration and position senses were mildly diminished. His paresthesia was more severe over the right calf and foot. Magnetic resonance images of the brain showed an acute small infarct in the medial-ventral portion of the left rostral medulla oblongata. A nerve conduction study and electromyography showed no abnormalities. At follow-up, the patient's motor and sensory deficits had improved considerably. CONCLUSIONS: The patient showed lemniscal sensory deficits below the right L5 dermatome that were caused by the partial involvement of the medial lemniscus. These findings suggest that lemniscal sensory dermatomal representation is preserved at least up to the level of the medulla oblongata.     

Pupil-sparing third nerve palsies and hemiataxia: Claude’s and reverse Claude’s syndrome

We report two patients with midbrain infarction with pupil-sparing third nerve palsies and hemiataxia: one with contralateral ataxia (Claude’s syndrome) and one with ipsilateral ataxia (which we refer to as reverse Claude’s syndrome). We highlight the importance of a thorough neurologic evaluation with partial oculomotor palsies and describe, to our knowledge, the fourth account in the literature of a pupil-sparing third nerve palsy with ipsilateral cerebellar ataxia.     

Déjérine's syndrome or Spiller's syndrome?

We report the difference existing between two clinical syndromes: Spiller's syndrome is caused by a complete involvement of the medial hemimedulla, while Déjérine's syndrome is determined by lesions restricted to the anterior portion of the medial hemimedulla and is characterized by hypoglossal nerve palsy and contralateral hemiparesis. Received: 28 January 2001 / Accepted in revised form: 3 September 2001     

Clinical study of twenty-one patients with pure midbrain infarction

We investigated the neurological and MRI findings of 21 patients with pure midbrain infarction. The locations of the infarcts were classified into paramedian (PM) area (13 patients), lateral area (6 patients), and PM and lateral areas (1 patient), based on the distribution of each penetrating artery of the midbrain. The infarction of 1 patient showed bilateral multiple lesions. In the PM area group, 12 patients showed eye movement disorders and truncal and gait ataxia. Eye movement disorders consisted of medial longitudinal fasciculus syndrome, oculomotor nerve palsy, and bilateral vertical gaze palsy. The infarcts of the 2 patients with bilateral vertical gaze palsy were located in the PM area on both sides. Eye movement disorders and truncal and gait ataxia were prominent in the PM area group. Ataxic hemiparesis was shown in 2 patients of the PM area group and 4 patients of the lateral area group. We showed that ataxic hemiparesis also occurred in PM area infarction, although ataxic hemiparesis was a prominent feature of lateral area infarction.     

Babinski-Nageotte syndrome on magnetic resonance imaging

A 70-year-old woman developed left hypoglossal nerve palsy, a right hemiparesis sparing the face, and a typical left Wallenberg's syndrome. These symptoms resulted from a lesion in the left half of the medulla oblongata, suggesting Babinski-Nageotte syndrome, a rare cerebrovascular disease. This is the first case of ischemic infarction in the territory of the left vertebral artery and posterior inferior cerebellar artery demonstrated on magnetic resonance imaging. Severe bilateral lesions of the distal vertebral arteries demonstrated on digital subtraction angiography may have contributed to the development of this syndrome.     

Severe Unilateral Proprioceptive Loss in Medullary- Rostral Spinal Cord Infarction. A Posterior Spinal Artery Syndrome

We draw attention to a unique presentation, severe unilateral loss of limb proprioception, in patients with medullary and rostral spinal cord infarction. Two patients developed acute severe proprioceptive loss in the limbs ipsilateral to infarcts that involved the caudal medulla and rostral spinal cord. They also had symptoms and signs often found in lateral medullary infarction. The proprioceptive loss is attributable to injury to the gracile and cuneate nuclei and/or their projections to the medial lemniscus. The infarct territory is supplied by the posterior spinal branches of the vertebral artery near its penetration into the posterior fossa. The presence of severe ipsilateral proprioceptive loss in a patient with features of lateral medullary infarction indicates involvement of the rostral spinal cord.     

Case of pontine infarction causing alternating hemiplegia with ipsilateral abducens nerve palsy and contralateral supranuclear facial nerve palsy]

We report a 73-year-old man with alternating abducent hemiplegia (Raymond syndrome) and contralateral supranuclear facial nerve palsy. On admission, he showed lateral gaze palsy of the right eye, left supranuclear facial nerve palsy, dysarthria and left hemiparesis. Brain MRI showed an infarct that was located in the paramedian and lateral area in the base of the caudal pons on the right side. MRA showed a mild stenosis of the basilar artery. Hemiplegia and supranuclear facial nerve palsy were considered to be caused by the involvement of corticospinal tract and corticobulbar tract that run at the ventromedial area of the pons. Abducens nerve palsy was considered to be caused by the involvement of infranuclear abducens nerve fibers. There has been one previously reported case of Raymond syndrome in which MRI determined the precise location of the lesion. In this case, a small hematoma was found at the ventral and medial pontomedullary junction, whereas the infarct in our case was located in the pontine base. We considered that documentation of our case was an important contribution to determine the pathogenesis of supranuclear facial nerve palsy due to caudal pontine lesions.     

Case of Fisher syndrome with impairment of taste]

A 38-year-old man developed dysesthesia, diplopia, and an unsteady gait following an upper respiratory infection. IgG anti-GQ1b antibody was detected in his serum and he was diagnosed as Fisher syndrome. The patient also complained of loss of taste sensation, and it resolved along with improvement of other neurological manifestations. In Guillain-Barré syndrome, cranial nerve involvement is very common, though taste disturbance is a rare complaint. Impairment of taste has been reported in association with severe facial nerve involvement, but taste disturbance developed without facial nerve palsy in the present case and taste sensation was diminished in the area of all four nerves involved in taste sensation. These findings suggest that the impaired taste sensation in the present patient was not a complication of facial nerve palsy as in previous cases, but rather due to taste sensory specific involvement.