A case of cerebral infarction presenting as retrosplenial amnesia]

We report a 73-year-old right-handed female who presented with an acute amnesic syndrome. On November 18, 1991, she was admitted to a local hospital complaining of sudden-onset vertigo and nausea, but immediately after the admission she developed an amnesic syndrome. On November 27, she was transferred to our hospital for further assessment of her memory disturbance. Neurologically she was normal except for mild right hemianopsia and increased deep tendon reflexes in the extremities. Neuropsychological assessments were performed over 3 weeks. She was always alert, attentive, and cooperative. She had no confabulation. On the Wechsler Adult Intelligence Scale revised (WAIS-R), her total IQ was 110. Frontal, verbal, and perceptual functions and motor performance were normal. She had no signs of a callosal disconnection. Despite these preserved functions, her memory function was obviously disturbed. Several memory betteries showed that her recent memory for both verbal and visual modalities was impaired, while her immediate memory such as digit span was preserved. For remote memory her retrograde episodic memory concerning both personal and public events was almost intact, although she had a profound anterograde amnesia. In particular she recalled her personal information about just-premorbid events in detail. On the other hand, her semantic memory, for example understanding of proverbs, geography, and scientific law, was preserved. Taken together, her procedural memory on learning tasks, such as "Tower of Hanoi" and mirror drawing, was intact. Computed tomography demonstrated a low-density area medial to the trigon of the left ventricle.(ABSTRACT TRUNCATED AT 250 WORDS)     

A case of bilateral lower pons-medial medullary infarction presenting quadriparesis]

Bilateral medial medullary infarction is rare. Only 18 cases have been reported previously. We experienced a case of the bilateral lower pons-medullary infarction. A 63-year-old woman was admitted to our hospital because of moderate left hemiparesis. Hyperreflexia in left limbs and positive Babinski's reflex in left foot was observed. Sensory disturbance was mild left hemihypesthesia (in light touch, postural sense and vibration) without facial involvement. She also had lateral gaze nystagmus, dysarthria, and bilateral decreased gag reflex. Respiratory failure was not observed. A conservative therapy for cerebral infarction was performed. But the hemiparesis was deteriorated and progressed to complete quadriparesis on the 5th day. The brain MRI (T2-weighted image and FLAIR) demonstrated bilateral lower pons-medial medullary infarction on the 9th day. Cerebral angiography and 3D-CT angiography revealed no stenosis or occlusions in the major cerebral arteries. The anterior spinal artery was not evaluated enough because of the arteriosclerosis. The prognosis of this patient was favorable except for the quadriparesis. The severe quadriparesis has not been improved for about 2 years. The bilateral medial medullary infarction was quite rare in the literature. The prognoses of these cases were unfavorable for the respiratory failure. Our case was not fatal because of no respiratory paralysis.     

A case of midbrain and thalamic infarction showing Collier's sign in one side and blepharoptosis in the other side

A 68-year-old man was admitted to the hospital complaining of aphasia and right hemiparesis. Neurological examination revealed aphasia, right hemiparesis and ataxia in the left upper extremity. He showed striking ocular symptoms: intense retraction of the right eyelid (Collier's sign), complete ptosis of the left eyelid which might conceal Collier's sign, marked upward gaze palsy and slight downward gaze palsy. Vertebral angiography and MRI revealed an infarction in the left medial area and the left crus cerebri in the upper midbrain, the left subthalamic area inferomedially extending from the adjacent area to the posterior commissure and the medial area in the left thalamus resulting from occlusion of the left mesencephalic artery. From these MRI findings and the reports on pathological findings of patients with Collier's sign, we suggest that damage in the posterior commissure or its adjacent area may be responsible for the sign. Upward gaze palsy is often associated with Collier's sign because fibers mediating upward gaze may decussate in the posterior commissure. Third nerve palsy is also associated with the Collier's sign in some patients. The mechanism creating Collier's sign may be a disturbance of inhibitory fibers leading to the motoneuron pool of the levator muscle in the posterior commissure or its adjacent area.     

A case presenting vertical one-and-a-half syndrome and seesaw nystagmus due to thalamomesencephalic infarction]

A 58-year-old woman presented, conjugate upgaze palsy and monocular paresis of downward gaze in the ipsilateral eye (vertical one-and-a-half syndrome; VOHS) as well as seesaw nystagmus (SSN). Vertical oculocephalic response and conjugate horizontal gaze were preserved. Magnetic resonance imaging revealed a right thalamo-mesencephalic infarction including the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF) and the interstitial nucleus of Cajal. On the 22nd hospital day SSN was disappeared, and then on the 32nd day VOHS was improved. The lesions of VOHS may have affected the efferent tracts of riMLF and the descending fibres to the ipsilateral subnucleus of the inferior rectus and contralateral subnucleus of the superior oblique. Furthermore, it was assumed that SSN was caused simultaneously by a lesion in the interstitial nucleus of Cajal existing in the adjacent area of riMLF.     

A case of HNPP presenting with late onset of symptoms, chronic progressive course and sensory impairment in face and trunk]

A 69-year-old man was admitted with a chief complaint of progressive muscle weakness and tingling sensation in the distal portion of all extremities since the age of 55. On neurological examination, sensory impairment of all modalities was noted in the right side of face, segmental areas of the right Th4-10 and the distal portion of four extremities. Symmetrical muscle weakness and atrophy was also found over the distal portion of all extremities. All deep tendon reflexes except biceps brachii reflexes were absent. Neurophysiological studies, however, rather indicated mononeuritis multiplex in this case. The biopsied specimen of the sural nerve showed a significant decrease in large myelinated fibers and many tomaculous changes. The gene analysis revealed deletion in the CMT 1A locus on chromosome 17p11.2, providing evidence for the diagnosis of hereditary neuropathy with liability to pressure palsies (HNPP). The development of sensory impairment in face or thoracic nerves is quite rare in HNPP, indicating that there exists considerable phenotypic heterogeneity in the disease.     

A case of young adult presenting with cerebral infarction caused by homocystinuria]

Homocystinuria is a congenital metabolic disorder, and has been known as life-threatening risk factor of vascular disease including ischemic stroke. We report a case of cerebral infarction due to homocystinuria. The patient was a 21-year-old woman exhibiting left hemiparesis and a previous history of ectopia lentis. Magnetic resonance imaging showed multiple fresh infarctions in the right frontal and temporal lobes, basal ganglia, corona radiata, and internal capsule. The right common carotid angiogram demonstrated complete occlusion at the origin of the right internal carotid artery. Further investigation clarified increased level of serum methionine and homocysteine and urinary homocystin due to cystathionine beta-synthase deficiency. Homocystinuria was diagnosed as the cause of cerebral infarction. The patient was treated by low methionine diet and administration of folic acid, cobalamin, and aspirin. It should be recognized that some patients with homocystinuria are missed in the neonatal screening for congenital metabolic disorders. Recent studies indicated that the homocysteinemia is one of risk factors of ischemic stroke in the general population as well as in the patients of homocystinuria. We recommend metabolic screening for homocystinuria, when treating a juvenile patient with ischemic stroke of unknown etiology.     

Diffusion-weighted MRI diagnosis of pure motor stroke limited to primarily distal leg weakness.

Pure motor stroke (PMS) manifesting as distal weakness of a single extremity has rarely been described. The authors report a 59-year-old man with PMS who had primarily distal weakness of a single lower extremity, which to the authors' knowledge has not been previously described. Four days after onset, positive diffusion-weighted imaging confirmed that the small subcortical T2-weighted hyper-enhancing and nonenhancing T1-weighted hypo-intensity noted on magnetic resonance imaging (MRI) represented an acute PMS that was the cause of the patient's weakness. Increased awareness of this rare clinical presentation of PMS coupled with the promise of imaging the PMS with diffusion-weighted MRI should lead to earlier stroke intervention in these patients.     

A case of sensory ataxia as the presenting manifestation of neurosarcoidosis

Sarcoidosis rarely selectively affects the cauda equina with characteristic motor and sensory impairments.Using imaging, we report a case of cauda equina polyradiculopathy presenting with progressive sensory ataxia without clinical or electrophysiological evidence of motor involvement. Neurosarcoidosis was diagnosed pathologically by proximal dorsal root biopsy after systemic investigations for inflammatory, infectious, and neoplastic etiologies were found to be negative. There was clinical and radiographic improvement with corticosteroids. In addition, we review previously reported cases of cauda equina sarcoidosis.     

A case of effective antithrombotic therapy for cerebral infarction presenting long-lasting fluctuation of symptoms]

We report a 78-year-old female presenting fluctuation of cerebral ischemic symptoms for a long time. Three weeks after transient ischemic attack of speech disturbance, she suffered right hemiparesis and motor dominant aphasia. The symptoms gradually progressed. Magnetic resonance angiography (MRA) showed a stenosis of branches of the left middle cerebral artery and single photon emission computed tomography (SPECT) did hypoperfusion of the same artery territory. Though antiplatelet and anticoagulant therapy was continued, the symptoms fluctuated. After 5 months, she recovered to mild deficit and MRI finally showed no responsible infarcted area. The pathophysiology of this course was considered to be progressing stroke based on a stenosis of the middle cerebral artery and the atrial fibrillation. Early diagnosis and treatment for cerebral ischemia may lead to the recovery from fluctuating symptoms.     

A case of pontine infarction presenting with conjugate deviation and unilateral conjugate gaze palsy]

We reported a case of pontine infarction presenting with conjugate deviation and unilateral conjugate gaze palsy. A 75-year-old man was admitted to our hospital because of vomiting. On the day of admission, his neurological findings showed only conjugate deviation to the right without consciousness disturbance, hemispatial neglect, and hemiparesis. On day 5 after admission, the conjugate deviation to the right disappeared, but he could not gaze left with either of his eyes. The pathological lesions, therefore, were considered to be a unilateral paramedian pontine reticular formation (PPRF) and abducens nucleus. Diffusion-weighted imaging (DWI) on day 2 revealed a small high intensity lesion in the left paramedian pontine tegmentum. A diagnosis of brain infarction was made. A Holter electrocardiogram and electroencephalogram were normal. Transesophageal echocardiography disclosed ulcerated plaque in the aortic arch. This is the first case of pontine infarction presenting with conjugate deviation in which unilateral conjugate gaze palsy and DWI could confirm the pathological lesion.     

A case of brainstem infarction presenting with paralytic pontine exotropia and non-paralytic pontine exotropia]

We reported a patient with brainstem infarction who presented paralytic pontine exotropia (PPE) in acute phase and non-paralytic pontine exotropia (NPPE) during the recovery phase. On March 10, 1991, a 60-year-old man was brought to our hospital with chief complaints of double vision and right hemiparesis. The ocular position of the left eye on forward gaze was fixed at the midline, while the right eye was abducted, and skew deviation was observed. On leftward gaze, neither eye could pass the midline, and on rightward gaze the right eye was abducted and monocular nystagmus was noted in the abducted right eye, which indicated paralytic pontine exotropia. T2-weighted MRI showed high signal intensity lesions in the left paramedian portion of the mid-pontine tegmentum beneath the fourth ventricle, and in the midline of the pontine base extending to the left side. The next day, similar disturbance of ocular movement was seen on rightward gaze, but the limitation of leftward gaze had improved and NPPE was noted to have developed. On the third day, there was no abduction of the right eye on forward gaze, but left MLF syndrome was still present. Seven days later, the disturbances of ocular movement disappeared. The lesion was considered to be due to partial impairment of the left paramedian pontine reticular formation in addition to the MLF. PPE caused by ischemia due to the perforating arteries of the brainstem, different from that due to basilar artery occlusion may recover after progression through NPPE and MLF syndromes. Hence, one-and-a-half syndrome, PPE, NPPE, and MLF syndromes are considered to be analogous to one another.     

A case of cerebellar infarction presenting as thunderclap headache

Thunderclap headache (TCH) refers to a sudden-onset, severe headache that features in subarachnoid hemorrhage, unruptured intracranial aneurysm, cerebral venous thrombosis, pituitary apoplexy, cervical artery dissection, and hypertensive reversible posterior leukoencephalopathy. TCH is a rare manifestation in cerebral or cerebellar infarctions. Herein, we report on a 60-year-old woman with a thunderclap headache as the first symptom of cerebellar infarction, in the absence of abnormal findings in the brain computed tomography (CT), CT angiography, and lumbar puncture. An urgent brain MRI showed an acute infarction of the right cerebellar hemisphere. The next day, the patient presented with right side ataxia. In emergency cases presenting with thunderclap headache, one should consider an expanded evaluation and/or close observation, with frequent neurological examinations, even though the findings are normal on the initial neurological examination, cerebrospinal fluid analysis, and brain CT.     

A case presenting with trochlear nerve palsy and segmental sensory disturbance due to circumscribed midbrain and upper pontine hemorrhage]

We describe a patient presenting with trochlear nerve palsy and segmental sensory disturbance due to circumscribed mesencephalic hemorrhage. A 36-year-old man with no past illness visited our hospital complaining of sudden onset of diplopia, dysesthesia of the left face and upper extremity, and acuphenes of the left ear. Neurological examination revealed left trochlear nerve palsy and segmental sensory disturbance of the left side almost above T11 level. Pain and temperature sensation were disturbed, but vibration, joint position, graphesthesia, kinesthesia, and discrimination sensation were spared. Magnetic resonance imaging of the head, performed 7 days after onset, revealed acute to subacute phase hemorrhage at the right inferior colliculus. No abnormalities were identified on cerebral angiography. Symptoms gradually improved with conservative therapy. After about ten weeks, diplopia disappeared and area of sensory disturbance was reduced (disturbance of pain sensation reduced to about T4 level, temperature sensation to about T9). Segmental sensory disturbance usually accompanies spinal cord lesion. However, several cases of similar symptoms following cerebrovascular disease of the brainstem have been reported. Conversely, some reports have indicated that trochlear nerve palsy due to midbrain hemorrhage accompanies sensory disturbance contralateral to the lesion. The nature of sensory disturbance is thus variable. The present case suggests that segmental sensory disturbance might accompany trochlear nerve palsy caused by hemorrhage of the inferior colliculus, as intramedullary fibers of the trochlear nerve and spinothalamic tract are located nearby and somatotopy of the spinothalamic tract is preserved even at the level of the midbrain.     

Somatosensory and skin temperature disturbances caused by infarction of the postcentral gyrus: a case report

Somatosensory functions are subdivided into 2 large groups: the elementary somatosensory functions, which consist of light touch, pain, thermal sensation, joint position sense, and vibration sense, and the intermediate somatosensory functions, which include 2-point discrimination, tactile localization, weight, texture, and shape perception. In this report, we describe a patient with somatosensory dysfunction after infarction of the postcentral gyrus. On physical examination a month after the onset of the infarction, voluntary movements were skillful, and both the elementary and intermediate somatosensory functions were disturbed in the right hand. The patient also displayed a decrease in the skin temperature of the right hand. The sensory-evoked potential in response to electrical stimulation of the right median nerve was normal, and brain MRI showed that the infarction was located in the posterior half of the left postcentral gyrus. These findings suggested that the lesion was situated at areas 1 and 2, and that area 3b was preserved. Thermography revealed that the skin temperature of the right hand was decreased predominantly on the ulnar side, and that recovery from cooling with ice water was delayed. By comparing the results of our patient with a case report that showed no disturbance of the elementary somatosensory functions with a localized lesion in the postcentral gyrus, we suggest that area 1 participates in the elementary somatosensory functions and that skin temperature may be controlled somatotopically in the somatosensory cortex.     

A case of carcinomatous autonomic and sensory neuropathy]

A 61-year-old male with carcinomatous autonomic and sensory neuropathy was presented. The disease started with numbness in his lower extremities and loss of sensation gradually developed over the upper extremities within two months. Finally he became unable to walk because of orthostatic hypotension and dystaxia due to loss of deep sensation. Physical examination on admission revealed mydriasis with negative light reflex, orthostatic hypotension, sensory loss of below C-4, areflexia, positive Babinski's sign and sexual impotence. Laboratory examinations revealed serum antineural antibody, elevation of protein and oligoclonal IgG band in the CSF. Sural nerve biopsy specimen showed severe loss of myelinated fibers, especially in the large-sized fibers. The pharmacological functional tests of the autonomic nerves disclosed disturbance of parasympathetic nervous system as well as the postsynaptic fibers of the sympathetic nerves. An instillation test of 2.5% mecholyl solution demonstrated contraction of both pupils. Chest X-ray showed abnormal shadow at the right hilus and transbronchial biopsy revealed small cell carcinoma (oat cell type). Plasma exchange improved orthostatic hypotension and decreased the titer of the antineural antibody. In this case, the autonomic ganglion including the ciliary ganglia and the dorsal root ganglia cells were predominantly involved and such cases have been described as a variant of paraneoplastic syndrome in the literature.     

A case of sensory perineuritis with Bowen disease]

A 60-year-old man was admitted because of subacutely progressive sensory disturbance characterized by fluctuating dysesthesia and light pain on the skin of his hands and feet. He exhibited mononeuritis multiplex, and a plaque with sloughing was observed on the left side of his back. Sural nerve biopsy revealed marked thickening of the perineurium with vascularization, and no necrotizing vasculitis. The pathological features of the nerve biopsy were compatible with sensory perineuritis. A biopsy from his skin lesion revealed Bowen's disease. There have been several reported cases of sensory perineuritis accompanied by malignant tumors. In these cases, immune dysfunctions were considered a common underlying cause in both diseases. This is the first reported case of sensory perineuritis associated with Bowen's disease.