Ipsilateral central-type facial palsy and contralateral hemiparesis associated with unilateral medial medullary infarction: a case report

Clinical pictures of medial medullary syndrome are variable, depending upon the extent of the lesion. Facial palsy has rarely been observed even in medullary infarction. However, central-type facial palsy is usually found contralaterally to the infarct area at the level of the rostral medulla. In the present report, we discuss the pathogenesis of the neurological manifestations in a 57-year-old man with hypertension. The patient presented with mild left facial palsy of central type, right hemiparesis, paresthesia, with deep sensory disturbance of the right extremities. An MRI of the brain showed an infarction localized in the medial region of the left upper medulla. Although the exact course of the supranuclear facial pathways remains controversial, the ipsilateral central facial palsy in this patient is considered to have two possible causes: the interruption of aberrant fibers of the corticobulbar tract, which branch off and swing back at the level of the upper-middle medulla, or the disruption of recurrent ascending fibers from the contralateral pyramidal tract, through decussation.     

Medial medullary infarct

Medial medullary infarct is a rare type of brain stem infarction first described in 1908. It was only since the broad use of MRI that an accurate clinical topographical correlation could be documented in survivors. We observed two patients; one of them had an anteromedial unilateral infarction in the upper medulla, and the clinical picture was characterized by contralateral hemiparesis, facial weakness, dysarthria and palatal weakness. The outcome was good. The second patient however showed a severe tetraparesis with only minor brain stem signs, secondary to bilateral anteromedian infarction with a large craniocaudal extension. He survived with severe residual impairment. The most likely cause of infarction was arteriosclerosis of the A. vertebralis and A. spinalis anterior, respectively. These two different cases suggest that the medial medullary syndrome is heterogeneous comprising small unilateral infarctions with mild clinical signs as well as large bilateral infarctions with a poor outcome.     

A case of spastic tetraplegia with medullo-cervical atrophy

A 48-year-old man, who had spastic tetraplegia and a marked atrophy of the medulla oblongata and upper cervical cord, was reported. He began to walk in spastic fashion at the age of 12 years, and was diagnosed as spastic paraplegia. His father, whose onset of the disorder was 35 years old and died of pneumonia at the age of 69 years, manifested symptoms and a course, both resembling those of the present patient. Generalized weakness progressed gradually, and the patient was confined to bed in his forties. On admission, he showed markedly increased tendon reflexes and pathological reflexes in all the extremities. Muscular weakness was severe in the neck, trunk and extremities, and mild in the facial muscles. There were neither bulbar sign, significant respiratory failure nor fasciculation of muscles. A needle EMG examination revealed no apparent neuropathic findings. MR imaging showed a marked atrophy of the medulla oblongata and upper cervical cord, which was not considered to be secondary to vascular or infectious diseases. Though sensory evoked potentials showed no response at the latencies of central nervous components, visual evoked potentials, brainstem auditory evoked response and blink reflex were normal. This case might represent an unknown hereditary degenerative disease with autosomal dominant inheritance.     

Vertebral artery injury following mild neck trauma: report of two cases

Two cases of vertebral artery injury following mild neck trauma are reported. A 52-year-old man was hospitalized with gait disturbance 7 days after mild traffic accident. Right vertebral angiogram revealed complete occlusion of the rt. vertebral artery (VA) and MR images revealed infarction in the rt. cerebellar hemisphere and rt. dorsolateral part of the medulla oblongata and revealed the thrombus in the rt. VA. He underwent anticoagulation and became asymptomatic. Angiogram 6 months later revealed the vessel to be normal. A 23-year-old man who has a habit of self-manipulation of his neck was hospitalized on the day when he experienced dysesthesia in the left part of his face and left upper and lower extremities and unsteady gait. MR images revealed multiple infarction in bilateral cerebellar hemispheres and thrombus in bilateral VAs. Bilateral vertebral angiogram revealed severe stenoses of bilateral VAs. He underwent anticoagulation and wore soft collar. Angiogram 20 days after onset revealed improvement of bilateral VA stenoses. He was discharged with no neurological deficit. It is said that vertebral artery injuries in association with head and neck trauma are relatively rare, but this condition is possible to be more common than realized, considering that the case of unilateral VA occlusion or the case with well developed collateral circulation is sometimes well tolerated for ischemia and that this condition can occur even after mild head and neck injury. The diagnosis must be established by vertebral angiogram, but MRI and MRA are very useful as ancillary methods. The therapeutic point is to prevent propagation of the thrombus and distal embolism, accordingly wearing a collar and anticoagulation are important.     

Bilateral vertebral artery dissection in a patient with afibrinogenemia

BACKGROUND: Afibrinogenemia, a rare coagulation disorder, has not been associated with vertebral artery dissections. CASE DESCRIPTION: A 28-year-old woman with afibrinogenemia developed spontaneous neck pain followed by a right medullary infarction, and MR angiography showed extensive bilateral vertebral artery dissection. She was treated with fibrinogen replacement and anticoagulants and showed a favorable evolution, with only mild residual right upper arm incoordination. CONCLUSIONS: In this patient spontaneous bilateral vertebral artery dissection complicated afibrinogenemia. Since anticoagulant therapy is usually indicated for arterial dissection, this association created a therapeutic problem. This patient received anticoagulants with fibrinogen replacement, which resulted in a favorable evolution.     

Paradoxical cerebral embolism during fibrinolysis therapy in deep vein thrombosis and pulmonary embolism

A 37-year-old woman with increasing dyspnoea over several months suddenly developed severe ortho- and tachypnoea as well as cyanosis of the lips and acrocyanosis. Pulmonary angiography revealed massive bilateral pulmonary emboli with a systolic pulmonary artery pressure of 75 mm Hg. Phlebography demonstrated a thrombotic occlusion of the deep veins of the left leg extending to the distal femoral vein. Thrombolysis treatment was started via an indwelling pulmonary artery catheter (500,000 IU urokinase and 10,000 IU heparin as bolus, then 1 mill. IU urokinase and 1,000 IU heparin per hour). After two hours an incomplete left-sided paresis occurred (involving ocular and facial muscles, dysarthria, left arm and left leg) and the thrombolytic infusion was stopped. But cerebral computed tomography (CT) did not demonstrate any intracerebral haemorrhage. The heparin infusion was restarted (partial thromboplastin time between 70 and 90 s). CT examinations during the next few days showed the development of an ischaemic infarction in the distribution of the right medial cerebral artery. Angiography demonstrated occlusion of the right internal carotid artery. The diagnosis of a paradoxical embolus was supported by easy cardiac catheter passage through a patent foramen ovale. Subsequent pulmonary angiography demonstrated a thrombus-free pulmonary arterial circulation with a normal pulmonary arterial pressure. There was gradual and extensive regression of the incomplete hemiparesis.     

Bilateral medial bulbar infarct related to dolichoectasia of the vertebral artery

INTRODUCTION AND CLINICAL CASE: A 58-year-old hypertensive man presented with the sudden onset of quadriplegia and inability to protrude the tongue. Magnetic resonance imaging showed a bilateral medial medullary infarction associated with an abnormally dilated and partially occluded vertebral artery. CONCLUSION: This is the first clinicoradiological report of a bilateral medial medullary infarction related to vertebral artery dolichoectasia.     

Peripheral motor and sensory nerve conduction studies in haemodialysis patients. A study of 54 patients

Peripheral motor and sensory nerve conduction velocities were studied prospectively in 54 chronic haemodialysis patients. The most sensitive parameters for the detection of polyneuropathy were the deep peroneal nerve motor conduction velocity, the sural nerve sensory conduction velocity and the H-reflex latency and H-index of the S1 roots. All patients examined were found to present at least one abnormal nerve conduction parameter. In the present study the side of the arteriovenous shunt had no statistically significant effect on the sensory and motor nerve conduction velocities in the upper extremities. There was a significant correlation between H-reflex latency and H-reflex index, and between H-reflex latency and sural nerve sensory conduction velocity.     

The origins of the subcortical component of the median nerve somatosensory evoked potentials

Short latency median nerve somatosensory evoked potentials (SEPs) were studied in two patients presenting with well localized unilateral pontine lesions producing hemisensory deficits. Both the early subcortical (N11-N13) and the later cortical component (N19-P22) of the SEP were abnormal in the patient whose lesion involved the medial lemniscus, while they remained unaffected in a second case of lateral inferior pontine infarction in which the medial lemniscus was spread. These results suggest that the N11-N13 component of the scalp recorded SEP emanates from the brain stem medial lemniscal pathway.     

Total electrical response to sound in the acoustico-lateral region of the carp, Cyrpinus carpio, medulla oblongata

In order to investigate foci of the electrical activity, studies have been made on the topography and form of total reactions to sound in the acoustico-lateral part of the medulla oblongata in the carp C. carpio. Two foci of the activity were found -- medical and ventral ones. Responses in the medial and ventral foci differ with respect to the form, latency (2.0--2.5 and 1.3--1.5 msec. respectively) and amplitude (200--600 and 50--200 microvolts respectively). It is suggested that total reactions in the medial focus reflect the activity of the nervous elements of the medulla oblongata, whereas the responses of the ventral focus reflect mainly microphonic reactions of sacculus of the inner ear of the carp.     

Localizing value of bilateral cheiro-oral sensory impairment

We present a case of bilateral cheiro-oral syndrome with paresthesia in the right toes following pontine infarction. The causative lesions were situated in the bilateral paramedian pontine tegmentum (PPM). As the lesion on the left side was larger than that on the right, the foot area in the left medial lemniscus was involved, causing paresthesia in the right toes. Bilateral cheiro-oral sensory impairment is rare, and there have been only 4 previous reports. The responsible lesion of complete bilateral cheiro-oral syndrome showing paresthesia in the hand and mouth region on both sides is in the bilateral PPM, while that of incomplete bilateral cheiro-oral syndrome showing paresthesia around both sides of the mouth and in the unilateral hand is in the PPM contralateral to the hand with paresthesia. Bilateral cheiro-oral sensory impairment can occur only in the brainstem.     

A case of pontine infarction with persistent primitive proatlantal artery

A 61-year-old woman was admitted to the hospital on September 18, 1991 because of left hemiparesis, dysphagia, and dysarthria since five days before. She was formerly pointed out diabetes mellitus and hypertension, but she did not receive any treatment. The MRI showed a high signal area in the right paramedian portion of the upper pons on T2 weighted image and proton image. The angiography showed that persistent primitive proatlantal artery originated from the left internal carotid artery and joined to the horizontal portion of the left vertebral artery. The image of carotid-vertebrobasilar system and proatlantal artery showed so severely arteriosclerotic. This is the first report of brainstem infarction with persistent primitive proatlantal artery. In this case, the pontine infarction was thought to occur on the basis of the arteriosclerosis of blood vessels and change of blood flow of carotid-vertebrobasilar system due to persistent primitive proatlantal artery.     

Sac-like pouches in Blastocystis from the house lizard Cosymbotus platyurus

We report a 65-year-old male with progressive supranuclear palsy (PSP) who developed bilateral vocal cord abductor paralysis (VCAP). The patient was admitted to our hospital because of impaired gait. He was well until two years earlier, when he began to walk unsteadily. During the next two years, dysarthria and dysphagia developed and his gait worsened gradually. On admission, neurological examination showed impaired vertical and incomplete lateral gaze. His speech was slow and monotonous. Contractures were found in the neck muscles and elbows. The deep tendon reflexes were increased in the upper and decreased in the lower extremities. Babinski sign was negative. Snout and forced grasping reflexes were elicited. He showed marked bradykinesia. Magnetic resonance imaging revealed a midbrain tectum atrophy. Single photon emission tomography showed severe hypoper-fusion in the frontal cortex. No improvement was provided by the administration of levodopa-carbidopa, bromocriptine, droxydopa and amitriptyline. One month after admission, inspiratory stridor developed at night. The laryngofiberscopic examination demonstrated VCAP. An emergency tracheostomy relieved his respiratory distress. Although VCAP rarely occurs in neurodegenerative disorders other than multiple system atrophy, attention to VCAP should be required in PSP patients.     

Anatomic organization of evoked potentials in rat parietotemporal cortex: somatosensory and auditory responses

1. Two 8 x 8-channel microelectrode arrays were used to map epicortical field potentials from a 3.5 x 3.5-mm2 area in homologous regions of right and left parietotemporal cortex of four rats. Potentials were evoked with bilaterally presented click stimuli and with bilateral tactile stimulation of the 25 major vibrissae. The spatial distribution of temporal components of the somatosensory evoked potential (SEP) and auditory evoked potential (AEP) complex were compared directly with cytochrome oxidase-stained sections of the recorded region. 2. Epicortical responses in both hemispheres to bilateral vibrissal stimuli consisted of a biphasic sharp wave (P1a-N1) constrained to the vibrissa/barrel granular region of primary somatosensory cortex (SmI). A slightly later sharp positive wave (P1b) was localized to secondary somatosensory cortex (SmII) and to perigranular cortex medial to the vibrissa/barrel field. The SEP complex ended with a biphasic slow wave (P2-N2). The P2 was centered on SmI and spread to dysgranular lateral cortex, caudal to but excluding SmII. The N2 was centered on SmII and spread to dysgranular cortex caudal to but excluding SmI. 3. The anatomic organization of the AEP in many ways approximated that of the SEP in the same animals. The timing and morphology of the AEP were nearly identical to the SEP. The AEP consisted of a P1a-N1 sharp wave constrained to the estimated region of primary auditory cortex (AI) in the lateral parietotemporal region, a later P1b localized to secondary auditory cortex (AII), and subsequent slow waves (P2 and N2) that were centered on AI and AII, respectively, and spread to dysgranular regions overlapping the distributions of the P2 and N2 of the SEP complex. 4. These data suggest that the basic neural generators for the SEP and AEP in parietotemporal cortex are quite similar, and provide evidence for the functional anatomy of each temporal component of the sensory evoked potential complex. It is concluded that the early fast waves of the SEP and AEP are modality specific and may represent the parallel activation of primary and secondary sensory cortex through established parallel afferent projections from lateral and medial thalamic nuclei. The later slow waves of the SEP and AEP appear to selectively involve primary and secondary sensory cortex but are more widely distributed, possibly reflecting a less modality-specific level of information processing in dysgranular cortex.     

Chronic hypouricemia by dysfunction of the renal tubule. Observation in a case

A case of hypertensive intracerebellar hematoma surgically treated and cured was reported. The 41-year-old male had two cerebrovascular attacks with headache and vomiting followed by left hemiparesis. Drowsiness and dysarthria appeared the next day. The patient was admitted to a hospital, where right facial palsy, loss of right gag reflex and paralytic hemiplegia on the left side were noted. On the 7th day, the patient's consciousness became clear byt the other neurological evidences did not change. On the 14th day, bradycardia and central hyperventilation appeared and he became drowsy again. The patient was transferred to the authors' clinic. When the patient was admitted, he showed typical cerebellar signs such as nystagmus, ataxia, and slurring speech with pyramidal sign on left side and cranial nerves paralysis on right side, and also showed the changes of vital signs as a medullary syndrome in the late stage of the course. The vertebral angiogram revealed a space taking process in the right cerebellar hemisphere. The old blood (30g) was removed by suboccipital craniectomy. The hematoma cavity had a communication with the IVth ventricle through a small perforation in the medial wall of the hematoma. Spontaneour intracerebellar hematoma including of hypertensive origin is not rare in the reports of autopsy but surgically treated case has only rarely been reported. The main reason of few survivals should be in its fulminate course.     

Shortened PCR cycles in a conventional thermal cycler

Although swallowing difficulties (dysphagia) frequently occur in acute brainstem infarction, physiological studies of dysphagia (videofluoroscopy, manometry) are rarely reported. We present a patient with ipsilateral Horner's syndrome, palatal and laryngeal weakness, aphagia, and ipsilateral face and contralateral extremity pin and temperature loss due to lateral medullary infarction confined to the rostral dorsolateral medulla (RDM). Videofluoroscopy showed that the patient was unable to initiate a swallow. Manometry showed a markedly reduced peak pharyngeal pressure and weak pharyngeal contractions. Within 20 months, the patient's neurological deficits resolved, videofluoroscopy showed a normal swallow, and manometry showed normal peak pharyngeal pressure. Correlation of the clinical, physiological, and imaging evaluations shows that aphagia and severe bilateral pharyngeal paresis can result from unilateral RDM infarction. We suggest that, in man, the bilateral medullary swallowing centers function as one integrated center, and that infarction of a portion of this center is sufficient to cause complete loss of swallowing.     

A case of myelofibrosis that developed polycythemia vera following treatment with ranimustine and then acute myelogenous leukemia (M0)

Short latency somatosensory evoked potentials (SSEP) to median nerve stimulation recorded from post-Rolandic area and the surface of processus spinosus of Cvs at the same record time. To assess electrophysiologically functional status of central nervous system (CNS), SSEP were studied in 43 patients with ischemic heart disease (IHD); 18 cases of angina pectoris (AP group), 25 cases of old myocardial infarction (MI group), and 14 non-IHD controls (NC group). The N13, N20, P25 peak latencies (PLs) and the central conduction time (CCT, the peak latency between N13 and N20) in AP group or MI group were prolonged remarkably compared with NC group. Likewise, MI group showed significant prolongation of N20, P25 PLs and CCT compared to the AP group. Furthermore, in MI group, the N20 peak amplitude was significantly lowered; the subjects percentages of the N20 or P25 amplitude detraction more than 40 percent in one side and the P25 interextremital latency difference over 1 ms were significantly increased than those of the NC group. These results suggested the pathological changes of the somatosensory pathway from superior spinal cord or medulla oblongata to cerebral cortex primary sensory area in CNS in the IHD patients.     

Medullary infarction due to vertebral dissecting aneurysm in a patient with Beh?et's disease]

A 65-year-old man with Beh?et's disease was admitted to our hospital because of an acute left occipital headache and truncal ataxia. He had been treated with prednisolone since the age of 40. MRI showed a hyperintense lesion on T2-weighted images on the left of the medulla oblongata. Left vertebral angiogram showed findings consistent with a dissection at the V4 segment of the artery. After admission, the patient showed marked improvement and was discharged 1 month later without neurological deficits. This is a very rare case of medullary infarction due to vertebral dissecting aneurysm in a patient with Beh?et's disease.     

Risk factors for deep venous thrombosis of the upper extremities

BACKGROUND: Hypercoagulable states and triggering factors (surgery, trauma, immobilization, pregnancy, and use of oral contraceptives) are associated with an increased risk for deep venous thrombosis of the lower extremities. In contrast, risk factors for deep venous thrombosis of the upper extremities have not been identified. OBJECTIVE: To evaluate the prevalence of hypercoagulable states and triggering factors in patients with primary deep venous thrombosis of the upper extremities. DESIGN: Frequency-matched case-control study. SETTING: Hemophilia and thrombosis center at a university hospital. PATIENTS: 36 patients who had primary deep venous thrombosis of the upper extremities, 121 patients who had primary deep venous thrombosis of the lower extremities, and 108 healthy controls. Patients who had deep venous thrombosis of the lower extremities and study controls were frequency-matched by age, sex, geographic origin, and social status with patients who had deep venous thrombosis of the upper extremities. MEASUREMENTS: Resistance to activated protein C was evaluated by a clotting method based on the activated partial thromboplastin time. If test results were abnormal or borderline, DNA analysis for substitution in coagulation factor V gene was done. Antithrombin, protein C, protein S, antiphospholipid antibodies, and total plasma homocysteine levels were also measured. RESULTS: Prevalences of abnormalities of the natural anticoagulant system (9%) and hyperhomocysteinemia (6%) in patients who had deep venous thrombosis of the upper extremities were similar to prevalences of both factors in controls (6% and 7%, respectively) but lower than in patients who had deep venous thrombosis of the lower extremities (31% and 14%, respectively). Antiphospholipid antibodies were found only in patients who had venous thrombosis of the lower extremities (7%). The overall prevalence of hypercoagulable states in patients who had thrombosis of the upper extremities (15%) was similar to that in controls (12%) but was significantly lower than that in patients who had thrombosis of the lower extremities (56%). A recent history of strenuous exercise of muscles in the affected extremity was the most frequent triggering factor for patients who had deep venous thrombosis in the upper extremities (33%). CONCLUSIONS: This preliminary study indicates that the prevalence of hypercoagulable states is low in patients who have primary deep venous thrombosis of the upper extremities.     

A case of adult type adrenoleukodystrophy with an acute onset and repeated episodes of ataxic dysarthria

We report a 30-year-old man with adult type adrenoleukodystrophy (ALD) who manifested an acute onset and repeated episodes of ataxic dysarthria. He noticed a moderate dysarthria after a high grade fever in February of 1995; however, two weeks later his symptom disappeared completely. Three months later, he noticed the dysarthria again and he was referred to our hospital for further examination. General physical findings on admission revealed a dark skin color, pigmentation of gingivae and reduced body hair. Neurologically he was normal except for a moderate ataxic dysarthria. Cranial T2-weighted MRI showed multiple high intensity lesions in the subcortical white matter of frontal lobe, bilateral peritrigonal white matter, splenium of the corpus callosum and bilateral cerebellar white matter. Only cerebellar lesions responsible for his symptom were enhanced on MRI after gadolinium administration. Initially we diagnosed him with multiple sclerosis (MS) based upon the clinical course and MRI findings, and then started corticosteroid treatment. His dysarthria was slightly improved after the treatment and bilateral gadolinium-enhanced lesions of cerebellar white matter on MRI disappeared. Multimodality evoked potentials such as short latency somatosensory evoked potentials, brainstem auditory evoked potentials and pattern-reversal visual evoked potentials, disclosed a prolonged central conduction time associated with bilaterally symmetric individual interpeak latencies. These findings, which supported diffuse and bilateral subclinical demyelinating lesions in the central nervous system, were unusual for MS; therefore his plasma very-long-chain fatty acids (VLCFA) were assayed for ALD. Finally, he was diagnosed with adult type ALD because of the high ratio of C26: 0/C22: 0 (0.075; normal 0.033). It is very difficult to clinically distinguish the early stage of adult type ALD especially in patients like this from MS. Therefore it is useful and important to evaluate not only the level of plasma VLCFA, but also to evaluate multimodality evoked potentials.