Reversible white matter lesions and antithyroid antibodies in the cerebrospinal fluid in Hashimoto's encephalopathy: a case report]

A 71-year-old woman with Hashimoto's disease was admitted to our hospital because of involuntary movement, gait disturbance, and mental decline. Her consciousness was alert but her orientation about time and place was disturbed. She was mentally ill (HDS-R; 12/30, MMSE; 14/30), and could not walk because of truncal ataxia. Myoclonus was present in the upper extremities. Laboratory examinations showed hypothyroidism and very high titers of antithyroid antibodies (ATA) in serum. Head MRI showed no abnormal lesions. On electroencephalogram (EEG), the basic rhythm was slow and bursts of irregular slow waves (4-6 Hz) were present. Her conditions gradually ameliorated so that she was discharged. However, she was hospitalized again because of sudden worsening of the illness: her consciousness got disturbed and the myoclonus became marked. As the result, she got bed-ridden. At the time, thyroid function was almost normal, suggesting that the deterioration could not be attributed to hypothyroidism. The EEG findings were quite different from the former: complex of multiple spikes and slow waves was continuously present. Examination of the cerebrospinal fluid (CSF) revealed an elevated level of protein and IgG (cell 1/m3, protein 101 mg/dl, sugar 60 mg/dl ,Cl 124 mEq/l, IgG 20.4 mg/dl). IgG index was 0.57 and Q albumin (CSF-albumin/serum-albumin ratio) was 15.2 (9.0>) . After the second admission, she recovered from the bed-ridden state but was still unable to walk or communicate. She continued to need complete support for all daily lives. The diagnosis was made as Hashimoto's encephalopathy (HE), from the following points: 1) encephalopathy not due to hypothyroidism, 2) very high titers of ATA, 3) elevated CSF protein. The effectiveness of steroid therapy was so amazing that the neurological problems faded away very soon. Finally she completely recovered. As well as the clinical manifestations, the EEG findings were improved. At the stage in which excellent clinical improvement had achieved, head MRI revealed that T2/FLAIR high lesions without Gd-enhancement temporarily appeared diffusely and extensively in the cerebral white matter. They thereafter almost disappeared on follow-up MRI. Judging from the lesions being non-enhanced and reversible, they supposedly reflected brain edema. It is important to keep it in mind that MRI findings may change abruptly and drastically in HE. ATA was detected not only in serum but also in CSF. Either titer thereafter decreased along with the healing of the illness. Concerning the reason why ATA is present in CSF, we supposed leakage from blood through the disrupted blood-brain barrier, for the following reasons: 1) IgG index was normal, 2) Q albumin was elevated, 3) the change of CSF-ATA titer was almost parallel to that of serum-ATA titer, and 4) the white matter lesions, which presumably reflected brain edema, were observed.     

Transient encephalopathy with reversible white matter lesions in children

We report on two children with acute encephalopathy showing mild clinical manifestations and reversible white matter lesions. In both patients, MRI revealed high intensities on T (2)-weighted imaging and marked reductions of water diffusion in the white matter of the bilateral centrum semiovale and the corpus callosum. These abnormalities disappeared along with the neurological symptoms within a week in both patients. These children represent a characteristic group of patients among childhood acute encephalopathy.     

Hypoglycemic encephalopathy with extensive lesions in the cerebral white matter

Here we report an autopsy case of hypoglycemic encephalopathy with prolonged coma. Laboratory data obtained when the patient lapsed into a coma showed that she had a low level of serum glucose (27 mg/dL). Although the level of glucose returned to within the normal range rapidly after glucose infusion, the patient remained in a coma for 22 months. It was presumed that the state of hypoglycemia persisted for about 4 h. There was no evidence of hypotension or hypoxia. Magnetic resonance imaging was performed 3 h after glucose administration; diffusion‐weighted images revealed hyperintensity in the cerebral white matter and in the boundary zone between the middle and posterior cerebral arteries. Post‐mortem examination revealed superficial laminar necrosis throughout the cerebral cortex. Neuronal necrosis was also found in the hippocampus and dentate gyrus, although the CA3 region appeared normal. In addition to these lesions, which are consistent with hypoglycemia‐induced brain damage, the cerebral white matter exhibited severe loss of myelin and axons with reactive astrocytosis and macrophage infiltration. Old infarcts were also present in the bilateral occipital lobes. Since the cerebral blood flow is reported to be decreased during severe hypoglycemia, the present findings suggest that white matter lesions and boundary‐zone infarctions may develop primarily in uncomplicated hypoglycemia.     

A case of portal-systemic shunt encephalopathy due to congenital portal vein hypoplasia presenting with abnormal cerebral white matter lesions on the MRI]

A 49-year-old woman, without any past history of liver diseases and blood transfusion, was admitted to our service because of somnolence, and flapping tremor. Neurologically, she was drowsy and disoriented. She had bilateral pyramidal tract signs and flapping tremor. Although the laboratory examination showed marked hyperammonemia (217 micrograms/dl), neither abdominal CT nor liver biopsy showed any evidence of liver cirrhosis. An abdominal angiography showed portal vein hypoplasia associated with the portal-systemic shunt. A T2-weighted MRI showed the high intensity areas in the bilateral deep cerebral white matter, and the posterior limbs of the bilateral internal capsules. This is a rare case of portal-systemic shunt encephalopathy due to congenital portal vein hypoplasia presenting with abnormal cerebral white matter lesions on the MRI.     

Transient encephalopathy with reversible white matter lesions: a case report

We report on a boy with bilateral optic nerve hypoplasia and mild psychomotor retardation. At 1 year and 9 months of age, he was admitted to hospital with a cluster of febrile convulsions and unconsciousness. Magnetic resonance imaging (MRI) revealed widespread areas of high signal intensity on diffusion-weighted imaging of the deep cerebral white matter and corpus callosum. This imaging disappeared at five days of illness. No atrophy or abnormalities were noted on the 6-month follow-up MRI. Despite full recovery after the acute episode, the patient showed retarded developmental progress. We discuss the differential diagnosis for this case.     

Neurologic signs predict periventricular white matter lesions on MRI

OBJECTIVE: Periventricular white matter disease (PVWD) is associated with abnormalities on tests that involve complex cognitive processes, along with an increased risk of cerebrovascular events which are associated with significant morbidity in older patients. This study investigates whether the neurological examination can predict the presence of PVWD on magnetic resonance imaging (MRI). No prior studies have assessed whether the neurological examination can predict the presence of PVWD on MRI. METHODS: A focused neurological examination was performed on a random selection of patients referred for a MRI of the brain. Staff neuroradiologists who were blinded to the results of the physical examination independently read the MRI scans. The MRI interpretations were divided into four categories based on the degree of PVWD: none, mild, moderate, severe. RESULTS: Twenty-three subjects had some degree of PVWD, while 25 subjects had none. The total number of neurologic signs correlated significantly with the severity of PVWD even when adjusting for the effect of age (rho=0.67, p<0.001). Ninety-one percent of subjects with PVWD had three or more abnormal signs, while 76% of subjects without PVWD had fewer than three. Abnormalities with the three step motor sequencing and horizontal visual tracking tests were maximally predictive of PVWD. One or both of these tests were abnormal in 96% of subjects with PVWD, while 64% of subjects without PVWD had no problems with either test. CONCLUSION: Simple neurologic tests can predict the presence or absence of PVWD on MRI.     

CT and MRI rating of white matter lesions

Rating scales play an important role in the evaluation of computed tomography (CT) or magnetic resonance-detected white matter lesions (WML). Unfortunately, this type of visual semiquantitative assessment is not yet an optimal tool because commonly agreed concepts regarding its use are lacking. To generate a discussion platform for further improvement of CT and MRI rating, we will provide some basic definitions, summarize the advantages and disadvantages of scoring schemes and review current efforts towards the improvement of this tool. Future research will have to concentrate on deepening our understanding of the histopathologic substrates of WML and on strategies to document their progression.     

Steroid-responsive diffuse cerebral white matter lesions in a case of intractable fungal meningoencephalitis]

We report a case of fungal meningoencephalitis with steroid-responsive diffuse cerebral white matter lesions. A 49-year-old male developed auditory hallucination, confusion and fever, on April, 1994. He was diagnosed as having cryptococcal meningoencephalitis based on the detection of cryptococcal antigens in the cerebrospinal fluid (CSF). Intravenous administration of fluconazole resulted in improvement of his neurologic symptoms and CSF findings. For the next seven months, he was treated with oral fluconazole and the neurological status was stable. However, soon after the dose of fluconazole was tapered, he became confused and febrile, which made him admitted to our hospital. Neurological examination on admission showed disturbance of consciousness, disorientation and meningeal irritation. The CSF examination revealed mild pleocytosis (mostly lymphocytes), elevated protein and normal glucose levels, although fungus was not detected. The T2-weighted image of brain MRI demonstrated diffuse hyperintense lesions in the bilateral cerebral white matters. GD-DTPA enhanced MRI showed spotty enhanced lesions in the periventricular white matters. The neurologic symptoms were once relieved after intravenous administration of fluconazole was started, but two months later, he became comatose and needed ventilatory support, despite amphotericine B therapy. Then, a needle brain biopsy targeting the white matter lesion was done. Histopathology of the specimen showed chronic inflammation with granuloma formation and T lymphycyte infiltrate around the small vessels, though fungus was not detected in the tissue. Combined therapy with corticosteroid and antifungal agents remarkably improved the neurological symptoms as well as the MRI findings. In the present case, fungal infection possibly induced an altered immune reactions which resulted in the steroid responsive diffuse cerebral white matter lesions.     

MRI identification of early white matter injury in anoxic-ischemic encephalopathy

BACKGROUND: Anoxic-ischemic encephalopathy (AIE) affects the gray matter more than the white matter. Recent animal experiments suggest that the white matter is more sensitive to ischemia than previously thought. The authors describe the MRI findings in seven patients with AIE who demonstrate early preferential involvement of the white matter. MATERIALS AND METHODS: A retrospective case series study was performed, including seven patients with AIE who underwent MRI of the brain within 7 days of insult. Demographic information, type of insult, clinical examination findings, EEG findings, and clinical outcome were obtained. MRI studies were reviewed with specific attention to the cortex, deep gray matter, and the white matter structures. Mean apparent diffusion coefficient (ADC) was calculated in regions of interest placed in the cerebellar hemispheres, putamen, thalamus, splenium of corpus callosum, centrum semiovale, and medial frontal cortex. RESULTS: The causes of AIE were cardiac arrhythmias in two patients, myocardial infarction in one, drug overdose in two, carbon monoxide poisoning in one, and respiratory failure and sepsis in one. The median time to MRI was 2.5 days. Symmetric areas of restricted diffusion were found in the periventricular white matter tracts (7/7 patients), the corpus callosum (6/7 patients), internal capsule (5/7 patients), and the subcortical association fibers (3/7 patients). ADC maps confirmed the restricted diffusion. Gray matter involvement was seen in three patients, and was more prominent on conventional imaging sequences compared with diffusion-weighted imaging. A subtle decrease in mean ADC was seen in cortex. CONCLUSIONS: Prominent, symmetric restricted diffusion can occur early after AIE in white matter, whereas gray matter involvement may be less prominent. Further studies involving a larger sample and serial imaging are required to confirm these preliminary findings.     

Hashimoto's encephalopathy with bilateral pallidal lesions presenting memory disturbance and executive dysfunction]

A 76-year-old man began to mistake his family for another person, and fall asleep easily while watching TV. He was treated with donepezil but without any effect. He was referred to our hospital on June. On admission, his consciousness was alert. Cranial nerves and motor functions were normal, but pathological reflexes were positive bilaterally. Serological examinations revealed high titers of antibodies against thyroglobulin and TPO, and antibody against alpha-enolase was positive. Total protein level in CSF was 40 mg/dl and cell counts were normal. On MRI, localized symmetrical lesions were observed in bilateral pallidum to genu of internal capsule. SPECT revealed hypoperfusion areas in bilateral striate bodies and frontal lobes. Neuropsychological examinations indicated impairment of executive function and procedural memory. The diagnosis of Hashimoto's encephalopathy was made and we treated the patient with oral prednisolone 60 mg/day followed by gradual tapering. After the treatment, clinical symptom as well as neuropsychological function improved. Neuropsychological impairment in this case was probably due to the disconnection of the thalamo-frontal projection. This case provides interesting suggestions that Hashimoto's encephalopathy may present with vasculitic infarctions in bilateral MCA perforators, and that this disease should be included in one of the differential diagnoses of cerebral infarctions of unknown etiology.     

偏头痛患者脑白质损害的MRI特征

目的探讨偏头痛患者脑白质损害的MRI特征。方法对80例偏头痛患者(偏头痛组)及80例健康体检者(对照组)进行MRI检查。对结果进行比较分析。结果 MRI检查显示偏头痛组中有42例脑白质损害,表现为皮质下脑白质内等T1、长T2信号影,T2Flair高信号;对照组中有9例脑白质损害。偏头痛组脑白质损害的比例(52.5%)明显高于对照组(11.3%)(χ2=31.34,P0.01)。偏头痛组中有先兆偏头痛患者脑白质损害的比例(68.6%,24/35)明显高于无先兆偏头痛患者(40.0%,18/45)(χ2=15.58,P0.01)。结论偏头痛患者脑白质损害的MRI表现与其它原因引起的脑白质病变类似。偏头痛患者脑白质损害的发生率较高,且有先兆偏头痛者较无先兆偏头痛者更高。     

Migraine with aura and white matter lesions: an MRI study

Several studies report the presence of white matter lesions on brain magnetic resonance imaging in patients with migraine. The aim of our study was to detect the entity of white matter T2-hyperintensities in 90 high selected patients affected by migraine with aura, compared to a group of 90 healthy controls. We found no significant difference of incidence of white matter alterations comparing these two groups.     

Pathologic findings of silent hyperintense white matter lesions on MRI.

The pathologic features of silent hyperintense white matter lesions in T2-weighted images on MRI were studied in patients with no neurologic signs or symptoms. The small patchy hyperintense white matter lesions represented myelin pallor associated with vessels showing hypertension and arteriosclerotic changes. 'Caps' also showed myelin pallor with dilated perivascular spaces. There were no lacunar infarcts in these lesions. Some of 'caps' was shown to be elongated normal lateral ventricle. 'Rims' of early stage revealed subependymal gliosis that was a part of normal aging processes.     

A case of isolated CNS sarcoidosis with diffuse confluent high intensity lesions at bilateral deep white matter]

We reported a 60-year-old woman who suffered from isolated neurosarcodosis. She was presenting comprehensive dysfunction and intermittent high fever. In several months she gradually developed dysorientation, amnesia, dementia. However, no focal sign such as paralysis or sensory disturbance was demonstrated. Her blood chemistry showed normal including ACE (angiotensin converting enzyme) and lysozyme. Cerebrospinal fluid revealed elevated mononuclear cells, protein, and decreased glucose level. At first we treated with antibiotics including antiviral drugs in suspect of the infectious encephalomeningitis. But no improvement was observed. The elevation of ACE in spinal fluid made us suspect of neurosarcoidosis. So intravenous predonizoron (1,000 mg) was given, improving. Her high fever and mental disturbance improved. Second spinal fluid showed improvement. During the course her brain MRI revealed new bilateral diffuse confluent high intensity lesions at the deep white matters. Brain biopsy of deep matter at the right anterior lobe showed noncaseating granuloma. Since systemic work-up to detect sarcoidosis did not reveal lesions other than CNS, we considered this patient as having isolated CNS sarcoidosis.     

Clinical significance of MRI white matter lesions in the elderly

The clinical relevance of white matter hyperintensities (WMH) seen on MRIs of elderly individuals is controversial. To resolve this issue, we performed MRI and neuropsychological testing on 46 healthy participants in the longitudinal Aging Process Study at the University of New Mexico. We graded the MRIs for severity of WMH using a scale tested on an elderly patient population. We found that 22% of normal subjects had moderate lesions and 9% had severe lesions. All subjects had normal neurologic examination findings and were within normal limits on a battery of neuropsychological tests. Neuropsychological performance decreased and the severity of WMH increased with age. However, when the data were corrected for age, there was no correlation between neuropsychological function and the presence of WMH. We conclude that white matter changes in the elderly by themselves are of doubtful clinical significance.