Correlation between anemia and clinical severity in subacute combined degeneration patients.

OBJECTIVES: Subacute combined degeneration (SCD) is a demyelinating disease commonly caused by vitamin B12 deficiency. Several studies have been reported SCD could be accompanied by anemia. However, the correlation between anemia and clinical severity of SCD patients is unclear. In this study, we aim to analyze the clinical characteristics of SCD concomitant with anemia, and investigate the effect of anemia in predicting the severity of SCD. METHODS: A total 42 patients were included in the study. Clinical, laboratory, radiological findings, and outcomes from the patients were analyzed. All patients were treated with vitamin B12 for no less than 6 months and a functional disability rating scale was used to evaluate severity of neurological impairment at the time of admission and 3 and 6 months after admission in our study. RESULTS: 85.7% patients had macrocytosis. Decreased serum vitamin B12 levels were found in 27 patients (64.3%). MRI showed long-segment abnormality on the spinal cord in 22 patients. No differences in rating score were found in patients grouped by sex, age, clinical course, serum vitamin B12, or MRI manifestations at the time of admission or at the follow-up visits. Negative correlation was seen between hemoglobin levels and the clinical severity scores on admission. CONCLUSION: Not all patients with SCD concomitant with anemia had decreased serum vitamin B12 level. The inverse correlation between hemoglobin level and clinical severity suggests the degree of anemia can help in evaluating the extent of neurologic impairment.

Hypertrophic Pachymeningitis in Chinese Patients: Presentation, Radiological Findings, and Clinical Course.

BACKGROUND: Hypertrophic pachymeningitis (HP) is generally regarded as a rare inflammatory disease, which results in a diffuse thickening of the dura mater. We retrospectively collected data from patients with HP. METHODS: A total of 16 patients with HP were included in our study. The clinical features, laboratory evaluation, imaging findings, treatment, and outcome were reviewed. RESULTS: Of the 16 cases, half were male, with a mean age of 52.6 ± 13.2 years. The mean duration from onset to diagnosis was 8.6 months. The most frequent presenting symptoms in HP cases were a recurrently chronic headache (81.3%) and multiple cranial nerve injury (50%). Antineutrophil cytoplasmic antibody- (ANCA-) related HP was found in 5 cases and IgG4-related HP in 1 case. The intracranial pressure was elevated in 4 cases. The cerebrospinal fluid (CSF) had lymphocytosis in 5 cases and increased protein in 12 cases. Immunoglobulins (IgG, IgA, and IgM) and protein showed linear relationships in the CSF. On magnetic resonance imaging (MRI), localized or diffuse dura maters were thickened in all cases. HP combined with subacute subdural hemorrhage or hypertrophic spinal pachymeningitis was also observed in individual cases. Biopsy of the dura mater in one case showed amounts of inflammatory cells infiltrating, with an increased percentage of IgG4-positive plasma cells. Of all cases referring to glucocorticoid treatment, the symptoms have improved significantly in 10 cases. In other 6 cases, mycophenolate mofetil or azathioprine was added. All patients showed clinical improvement at the follow-up visits. CONCLUSION: The clinical characters of HP are chronic onset, recurrently chronic headache, and multiple cranial nerves paralysis. Inflammatory changes in CSF caused by intrathecal synthesis of immunoglobulin, characteristic dural enhancement on MRI, and pathologic biopsy are all helpful for diagnosis. The addition of immunosuppressant, especially mycophenolate mofetil, is a good choice for steroid-resistance HP.

Isolated brainstem involvement in posterior reversible encephalopathy syndrome: a case report and review of the literature.

Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiological syndrome, reversible vasogenic edema predominantly within parieto-occipital regions. However, isolated brainstem involvement in PRES has been rarely reported, little is known about its clinical manifestations, radiological features and outcomes. We reported a case with PRES with only brainstem involvement and performed a systematic review of published cases. Twenty-four cases, together with our case, were included in the analysis. Mean age was 43 years and 63% were males. Hypertension (50%), nephropathy (25%) and chemotherapy (21%) were the major risk factors. All patients except two had acute hypertension and 50% of patients had renal dysfunction at onset. The most common symptoms were altered consciousness (46%) and headache (46%), Seizure was only presented in 21% of patients. All patients except two were treated with antihypertension. Most patients recovered to their neurological baseline. Clinicians should recognize this unique variant finding in PRES. which always affects males with severe hypertension, especially combined with renal dysfunction. Antihypertensive treatment is the most widely used therapy. Outcome is usually well.

Subacute Combined Degeneration: A Retrospective Study of 68 Cases with Short-Term Follow-Up.

PURPOSE: The study aimed to analyze the clinical characteristics, laboratory test results, neuroimaging findings, and outcomes in patients diagnosed with subacute combined degeneration (SCD). MATERIALS AND METHODS: A total of 68 patients with SCD who had been appropriately treated for no less than 6 months were included in our study. Histories, results of routine blood tests, biochemical indices, serum vitamin B12 levels, and spinal magnetic resonance imaging (MRI) findings from the patients were studied and analyzed. Clinical signs and symptoms, graded using a functional disability rating scale, were scored at the time of admission and 3 and 6 months after admission. RESULTS: Limb numbness, limb weakness, and gait disturbances were the most common symptoms in patients with SCD. All patients showed clinical improvement to different degrees at the follow-up visits after vitamin B12 treatment. No differences in rating score were found in patients grouped by sex, hemoglobin level, serum vitamin B12, or MRI manifestations at the time of admission or at the follow-up visits. Younger patients and those with shorter disease courses had better rating scores at the short-term follow-up visits. CONCLUSION: Anemia, low levels of serum vitamin B12, and MRI abnormalities in the spinal cord are not expected to be associated with worse clinical manifestations. The age of onset and course of disease are important in evaluating the short-term prognosis of patients with SCD.