A case of pure motor monoparesis due to pontine infarction

Pure motor hemiplegia (PMH) is a common manifestation of lacunar infarction. However, pure motor monoparesis (PMM), isolated corticospinal tract deficits confined to one limb, have received little attention. We observed a patients in whom the clinical feature was compatible with PMM and discussed the possible pathomechanism of PMM.     

A case of antiphospholipid antibody syndrome showing a neurological deterioration and infarct development over a month]

A 67-year-old man was admitted to our hospital because of a sudden onset of gait disturbance and behavioral abnormalities. On the admission, he had a moderate consciousness disturbance and right hemiparesis with left internal carotid artery occlusion. Eight days after the stroke, the patient further developed left hemiparesis in association with right internal carotid artery occlusion. Despite anticoagulation therapy and plasma volume loading, neurological symptoms deteriorated over a month, during which CT scan demonstrated a progressive expansion of infarct size. Laboratory tests revealed the presence of lupus anticoagulant. Antiphospholipid antibody syndrome may be associated with a progression of ischemic stroke.     

A case of cortical infarction showing unilateral facial and hypoglossal paresis]

We describe a case presenting with facial and hypoglossal nerve paresis due to cortical cerebral infarction. A 54-year-old man visited our hospital complaining of sudden episode of dysarthria and facial paresis. Neurological findings revealed tongue deviation to the left and left facial paresis with forehead wrinkling while neither limb paralysis nor sensory impairment was observed. Head CT performed on day 3 after onset revealed a cortical infarction in the right prefrontal gyrus. Symptoms gradually improved with medical management. This patient had isolated facial and hypoglossal nerve paresis without other neurological symptoms. Patients with mild paresis of cranial nerves should be diagnosed carefully, because their paresis could be supranuclear type.     

A patient with choreoathetosis of the left upper extremity due to acute cerebral infarction]

A 71-year-old woman was admitted to our hospital because of involuntary movement of the left upper extremity. MR image of the brain 15 days after the onset revealed the low intensity in right posterior limb of internal capsule. The lesion was surrounded by thalamus, subthalamic nucleus, and globus pallidus with enhancement by Gd-DTPA. Surface EMG revealed irregular grouped discharge in short duration and grouped discharge in long duration in the left upper extremity. Those features are compatible with one of choreoathetosis. Choreoathetosis due to cerebral infarction in acute phase is rare. We discussed pathophysiology of this involuntary movement due to lacunar infarction of posterior limb of internal capsule in acute phase.     

A case of slowly progressive aphasia accompanied with auditory agnosia]

A 68-year-old right-handed woman was admitted to our hospital because of difficulty to speak and understand conversation over 10 years. She was able to make herself by writing. No change in her personality or behavioral abnormality was observed so that she could live without help. Although her WAIS score and auditory brain stem response were normal, she could not understand the speech or distinguish the sound. She also spoke plenty of words fluently, resulting in undifferentiated jargon. She did not make any effort in speaking. Her speech was, however, unclear and hard to understand. Brain MRI scan disclosed a moderate atrophy of bilateral temporal lobe and enlargement of Sylvius fissure. A three-dimension reconstructed brain surface image showed enlargement of the perisylvian fissure, and atrophy of the gyrus frontalis inferior, operculum, gyrus temporal superior, bilaterally. Reduced cerebral blood flow was demonstrated on 99mTc-ECD SPECT in the left thalamus and bilateral fronto-temporal lobe. A diagnosis of slowly progressive aphasia with auditory agnosia was made. Our case suggests that bilateral disturbance of neuronal network between the primary auditory area and the secondary auditory area is responsible to the consequence of auditory agnosia.     

A case of chronic pure motor neuropathy associated with anti-GalNAc-GD1a-IgM antibody]

The patient was a 34-year-old male with chronic pure motor neuropathy with such acute onset as seen in Guillain-Barré syndrome. Neurological symptoms were gradually progressive for 4 weeks, and predominantly noted in the left side. Deep reflexes were normal and the distribution of muscle weakness was uneven. Plasma exchange reduced neurological symptoms. Four weeks later, right drop foot was relapsed. High dose intravenous immunoglobulin was effective. Serial electrophysiological studies indicated the asymmetric reduction of CMAP. Repeated assays of anti-GalNAc-GD1a IgM antibody were positive. This is the first report of chronic pure motor neuropathy as multiple mononeuritric type associated with anti-GalNAc-GD1a IgM antibody. This case adds to our knowledge better understanding of the pathogenetic role of anti-GalNAc-GD1a IgM antibody in inflammatory neuropathies.     

A case of transverse myelitis caused by primary antiphospholipid antibody syndrome]

A 14-year-old man was admitted with sudden onset of paralysis in his lower extremities paralysis and of sensory loss below Th10 level. On admission, linear high intensity signals was seen in the spinal cord from Th 8 to Th 12 level on thoracic MRI T2 weighted image. Laboratory data on admission indicated existence of lupus anticoagrant in the patient's serum. Systemic lupus erythematodes was negative in his past history. The diagnosis of transverse myelitis caused by primary antiphospholipid syndrome was made accordingly. Plasmapheresis was performed to remove the lupus anticoagrant. After plasma pheresis coagulopathy was normalized with disappearance of the lupus anticoagrant. The lesion of the spinal cord is too extensive to be caused by single obstruction of any one blood vessel branch of the spinal cord. The transverse myelitis may be aggravated by the direct invasion of the lupus anticoagrant into nervous tissue from the vasculature whose blood brain barrier had been compromised by intravascular coagulation of this substance.     

An autopsy case of catastrophic antiphospholipid syndrome presenting with recurrent multiple cerebral infarction associated with lung cancer]

We reported an autopsy case of cerebral infarction with primary lung cancer. The patient was a 50-year-old man. Despite having been treated with warfarin potassium and ticlopidine hydrochloride, he relapsed cerebral infarction. His laboratory data on admission showed that lupus anticoagulant was positive, together with a high value of beta-thromboglobulin, thrombin-antithrombin III complex, markers of platelet and coagulation activation, CEA and CA 19-9. The autopsy finding revealed a primary papillary adenocarcinoma in the right lower lung, multiple cerebral infarction, renal infarction, pulmonary infarction and splenic infarction. The atherosclerotic changes were mild in the whole tissues and findings of vasculitis were not observed. Recurrence of cerebral infarction was effectively suppressed with the addition of steroid therapy to antithrombotic therapy. This case was considered as catastrophic antiphospholipid syndrome. It is necessary to differentiate antiphospholipid syndrome in case of the abnormal coagulation and fibrinolytic factors with recurrent cerebral infarction. Moreover, systemic examinations are important, because malignant tumor may exist on the background of the case.     

A case of cerebellar infarction with pure dysarthria]

We report a 69-year-old man with pure ataxic speech. He was admitted to the surgical ward of our hospital because of exercise-induced pain in the right arm. He underwent angiography of the right arm, and was discharged the next day. When he returned home, he exhibited an acute-onset dysarthria. He was admitted to our neurology ward the next day because the dysarthria did not improve. On admission, neurological examinations revealed moderately ataxic speech, but other neurological findings were within normal limits. Cranial MRI revealed an infarct localized from the lobulus simplex to the lobulus quadrangularis in the right cerebellum. Three cases of pure dysarthria due to cerebellar infarction have been reported previously. We compared cerebellar lesions in the 4 cases of pure dysarthria due to cerebellar infarction. Since the lobulus simplex of the upper cerebellar hemisphere was involved in all 4 cases, we speculated that ataxic speech occurred from the impairment of this cerebellar area.     

A case of adult-onset tethered cord syndrome accompanied with slowly progressive muscular atrophy in the lower limbs]

A 70-year-old man developed slowly progressive muscular atrophy in the lower limbs from age 28, followed by urinary disturbance from age 40. Neurological examination revealed bilateral severe muscular atrophy in the lower limbs with hypesthesia and hypalgesia, mild muscle wasting of both hands, urinary incontinence, and constipation. A sacral dimple was also recognized. A diagnosis of tethered cord syndrome with thick filum terminale was made by spinal magnetic resonance imaging (MRI). After the operation for untethering, urinary disturbance has been slightly improved. Although the adult-onset tethered cord syndrome with the thick filum terminale was extremely rare, the presence of congenital dermal abnormality and segmental distribution of the muscular involvement is often helpful for the clinical diagnosis of this disease. Spinal MRI is one of the most diagnostic method for this disease.     

A case of hemimegalencephaly with slowly progressive expansion

We report a case of a male infant with refractory epilepsy, demonstrating hemimegalencephaly with slowly progressive expansion. The patient experienced his first seizure at 4 months of age. Subsequently, tonic seizures occurred very frequently despite extensive antiepileptic medications, and his development deteriorated. Cranial magnetic resonance imaging (MRI) at 4 months of age showed focal cortical dysplasia in the right opercular area. This focal lesion gradually expanded, and became thickened. Five years later, the dysplastic lesion occupied most of the right cerebral hemisphere and the volume of the right hemisphere increased, indicating hemimegalencephaly. He had profound motor and intellectual retardation. In the abnormal cerebral hemisphere, fluorodeoxyglucose-positron emission tomography (FDG-PET) showed marked hypometabolism, and ictal single photon emission computed tomography (SPECT) showed hyperperfusion, more pronounced in the right frontal area. These findings are consistent with a hemimegalencephaly. Hemimegalencephaly with such a progressive expansion has never been described previously. These findings are consistent with a hemimegalencephaly showing progressive expansion, which has never been described previously.     

A case of slowly progressive unilateral spastic hemiparasis, hand clumsiness, limb ataxia and constructional apraxia]

A 65-year-old woman was seen in our clinic because of a 4-year history of slowly progressive left hand clumsiness and left limping. Neurologically, she had left-side spastic hemiparasis including her tongue, hand clumsiness, limb ataxia, constructional apraxia, and memory impairment. Dementia, parkinsonism and lower motor neuron sign were not confirmed. MRI study showed diffuse cerebral atrophy and shrinkage of the right cerebral peduncle and pontine base. SPECT (99mTc-ECD) study revealed hypoperfusion of the right fronto-parietal cortex. This is a rare case presented with slowly progressive left-side spastic hemiparasis, mimicking Mills' syndrome. In addition this case had other cortical signs, such as ipsilateral hand clumsiness, limb ataxia, constructional apraxia. Neuroimaging study suspects that the right front-parietal cortex is the primary lesion. Etiologically atypical motor neuron disease with adjacent cortical involvement is suspected rather than corticobasal degeneration with severe unilateral pyramidal tract degeneration.     

Lacune in the white matter revealed by MRI in a case of pure motor monoparesis

It has been reported that most of pure motor monoparesis were induced by the superficial mass lesions in the cerebral hemisphere. However, pure motor monoparesis due to lacune is rare. We reported a 81 year-old woman of pure motor monoparesis caused by the lacunar infarction in the parasagittal subcortical white matter. That lesion could not be detected by CT scan performed at onset, and 18 days later after the stroke, but was detected by MRI performed 5 days after the onset. The coronal section of MRI showed the stick-like shaped small infarction in the white matter, which runs along the corticospinal tract, just under the precentral gyrus. The coronal section of MRI is useful for the demonstration of the anatomical localization of lacune.     

A case of brain ischemia presenting spectacular shrinking deficit in adult-onset Still's disease associated with antiphospholipid antibody syndrome]

A 63-year-old man presenting remittent fever and multiple arthralgia was diagnosed as adult-onset Still's disease (AOSD), and started with prednisolone treatment. However, he suddenly developed loss of consciousness, paresis of the right upper extremity and aphasia shortly after the treatment. We detected an increased signal of brain tissue lactate at the branch territory of left middle cerebral artery by MR spectroscopy (MRS), but no lesions by diffusion-weighted nor T2-weighted MRI, suggesting acute brain ischemia of embolic mechanism. Most of the symptoms resolved in a couple of hours after the onset, showing spectacular shrinking deficit (SSD). The patient also revealed complication of antiphospholipid antibody syndrome (APS), which may be associated with ischemic event. This is the first case of acute brain ischemia with SSD, which occurred in AOSD with APS. MRS was superior to diffusion MRI in detection of acute brain ischemia.     

Fluorodeoxyglucose F18 positron emission tomography in a case of slowly progressive pure alexia

We describe a 71-year-old patient with slowly progressive pure alexia in which analysis of her fluorodeoxyglucose FDG-PET scan revealed an area of focal hypometabolism in the visual word form area. She presented with difficulty reading. Examination revealed pure alexia with preservation of other cognitive domains. Brain MRI revealed only slight atrophy. A Fluorodeoxyglucose F18 positron emission tomography scan revealed hypometabolism in the occipital cortex bilaterally, left greater than right, with normal metabolism elsewhere in the brain. This case highlights the utility of FDG-PET scan in evaluating focal neurodegenerative conditions before clear atrophy can be seen on MRI.     

A case of adult moyamoya disease showing progressive angiopathy on cerebral angiography.

In moyamoya disease, progression of carotid occlusive lesion in an adult patient is very rare. We report a case of adult moyamoya disease with acute angiographical stage progression and hemodynamic deterioration. A 56-year-old female complaining of left motor weakness visited our department. On MRI, infarct lesion was found in the right white matter. On cerebral angiography, occlusive lesions in the bilateral internal carotid arterial siphons and moyamoya vessels were found. The right side was stage V and the left side was stage III. On IMP-SPECT, decreased cerebral hemodynamic reserve of the right cerebral hemisphere was found. In this patient, right STA-MCA anastomosis was performed. After operation, she became possible to walk and discharged to home. Five months after operation, good collateral formation and improvement of hemodynamic reserve in the right hemisphere were found. However, on left carotid arteriography, the anterior and middle cerebral arteries were only slightly imaged, and the disease state progressed to stage IV. In addition, decreased blood flow and hemodynamic reserve were appeared in the left hemisphere.     

A case of motor neuron disease with progressive aphasia and dementia]

We report a 71-year-old woman showing rapidly progressive non-fluent aphasia and dementia accompanied by motor neuron disease (MND). There was no family history of dementia or motor neuron disease. There was 10 months history of dysarthria and dysphagia. On examination, she showed profound difficulty in articulation. Her comprehension was impaired in that she was unable to obey three-stage command. Her written language was also impaired with phonological spelling errors, syntactic errors, and perseveration. Neuroradiological investigations showed atrophic changes and hypoperfusion of left temporal and bilateral parietal region revealed by MRI and SPECT, respectively. Her subsequent decline was rapid. It might be likely that aphasia is much more common in dementia with bulbar MND than is currently recognized because bulbar palsy might mask the language disorder.