Spontaneous Escherichia coli meningitis associated with hemophagocytic lymphohistiocytosis.

Spontaneous Escherichia coli meningitis has not been previously reported in association with hemophagocytic lymphohistiocytosis (HLH). A previously healthy 72-year-old woman was admitted due to fever, nuchal rigidity, disturbed consciousness and splenomegaly. Anemia, thrombocytopenia and hyperferritinemia developed on the 8th day of hospitalization. Cultures of cerebrospinal fluid and blood grew E. coli. Abundant macrophages overwhelmed erythrocytes in the bone marrow aspirate, confirming the presence of hemophagocytosis. E. coli meningitis was managed with a 40-day course of antibiotic treatment. However, the severity of anemia and thrombocytopenia progressed despite intensive transfusion therapy. The patient died of HLH on the 60th day of hospitalization.

Avascular necroses inducing limb-girdle syndrome.

Disuse myopathy can be caused by many disorders. However, disuse myopathy with symmetrical weakness of the proximal muscles in the four limbs is rare. Here we report a 45-year-old man who presented with the appearance of myopathy similar to the clinical picture of limb-girdle syndrome with symmetrical weakness of proximal muscles in four limbs. A battery of intensive investigations confirmed that the patient had symmetric disuse myopathy induced by multiple avascular necroses of hip and shoulder joints. After the patient had received bilateral total hip replacement operations he had remarkable improvement in his muscle power in both lower limbs. This report highlights that some myopathies can be reversed if their underlying causes are correctable.

Aseptic meningitis and ischemic stroke in relapsing polychondritis.

Relapsing polychondritis (RP) is a rare disease characterized by recurrent inflammation of cartilaginous structures. Its involvement of central nervous system is uncommon. We report the case of a 71-year-old woman whose initial manifestations were RP and meningitis and whose subsequent attack of RP was associated with a left cerebral infarction. The patient responded to steroid therapy in both attacks of RP.

Intracranial hypertension as an initial manifestation of spinal neuroectodermal tumor.

A 19-year-old girl had headaches, blurred vision and vomiting for 2 weeks. Neurological examination revealed only bilateral papilloedema and left abducens palsy. Neuroimaging of the brain was normal. Cerebrospinal fluid study showed intracranial hypertension (IH), hypoglycorrhachia, hyperproteinorrhachia, and a negative cytology study. Eight months after the onset, paraparesis occurred. Spinal magnetic resonance imaging showed intramedullary masses at the cervical and thoracic cords with extensive seeding. Biopsy of the mass showed primitive neuroectodermal tumor (PNET). IH rarely occurs in patients with spinal cord neoplasms. Its incidence is low and the condition is always associated with signs of myelopathy. We report a patient whose initial manifestation of spinal PNET was IH only. Spinal tumor should be considered in IH patients whose intracranial examinations are negative.

Sensory neuropathy as the initial manifestation of multiple system atrophy.

Peripheral neuropathy is usually subtle or subclinical in patients with multiple system atrophy (MSA). A 54-year-old man who initially presented with sensory polyneuropathy developed extrapyramidal and cerebellar signs and autonomic failure typical of MSA 7 years later. Sural nerve biopsy disclosed a prominent loss of large myelinated fibers, whereas the unmyelinated fibers were totally spared. These findings imply that sensory neuropathy can be part of the multiple-site degeneration in MSA, and that predominant involvement of large myelinated fibers of the sensory nerves can be a presenting feature.

Markedly asymmetrical parkinsonism as a leading feature of adult-onset Huntington's disease.

We report on a 28-year-old man who presented with right hand tremor, bradykinesia, and rigidity of his right side extremities. Our case report emphasizes that markedly asymmetrical parkinsonism can be an initial presentation of adult-onset Huntington's disease (HD), and different clinical presentations can be observed in members of an individual HD family with the same CAG repeat length.

Cardiovascular autonomic function and sympathetic skin response in chronic inflammatory demyelinating polyradiculoneuropathy.

Quantitative cardiovascular autonomic function tests (AFTs) and sympathetic skin response (SSR) were performed in 12 patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Parasympathetic autonomic function was examined by Valsalva ratio and R-R interval variation during rest and deep breathing. Sympathetic autonomic function was evaluated by blood pressure responses to sustained handgrip and active standing. Using quantitative AFTs, subclinical autonomic dysfunction was demonstrated in three (25%) patients. Abnormal SSR was seen in six (50%) patients. Thus, mild autonomic dysfunction is present in many CIDP patients. The autonomic abnormalities involve parasympathetic or sympathetic components; in the sympathetic nervous system, both vasomotor and sudomotor fibers may be involved.

Spinal epidural abscess successfully treated with percutaneous, computed tomography-guided, needle aspiration and parenteral antibiotic therapy: case report and review of the literature.

OBJECTIVE AND IMPORTANCE: We describe a case of a posterior spinal epidural abscess that was successfully treated with percutaneous, computed tomography-guided, needle aspiration and parenteral antibiotic therapy. CLINICAL PRESENTATION: A 48-year-old man experienced acute pain in the paralumbar region for 1 week, followed by gait disturbance and micturition difficulty. Laboratory studies demonstrated leukocytosis and hyperglycemia. Blood cultures yielded Staphylococcus aureus. Magnetic resonance imaging scans of the thoracolumbar spine revealed a posterior spinal epidural abscess located between L2 and the lower cervical spine. INTERVENTION: The patient's low back pain persisted despite 10 days of antibiotic therapy. Therefore, percutaneous, computed tomography-guided, needle aspiration was performed. The low back pain was relieved dramatically and immediately after the procedure. The spinal epidural abscess completely resolved after 6 weeks of antibiotic treatment. CONCLUSION: Surgical decompression and antibiotic therapy are the treatments of choice for patients with spinal epidural abscesses. Selected patients may be treated nonsurgically. Rarely, percutaneous drainage of the abscess has been reported to be helpful. Our case suggests that percutaneous, computed tomography-guided, needle aspiration might be a rational alternative to surgical decompression for treatment of spinal epidural abscesses.

A longitudinal cardiovascular autonomic function study in mild Guillain-Barré syndrome.

To identify subclinical autonomic dysfunction in mild Guillain-Barré syndrome (GBS), a set of autonomic function tests was serially performed for up to 6 months in 5 GBS patients with mild disability at the nadir. Parasympathetic autonomic function tests consisted of Valsalva ratio and R-R interval variation during rest and deep breathing. Sympathetic autonomic function was evaluated by blood pressure responses to sustained handgrip, hand immersion in ice water, and active standing. The results showed that abnormal parasympathetic and sympathetic function was frequently encountered in all 5 patients during the acute stage of the illness. Autonomic dysfunction occurred both in axonal and demyelinating types of GBS. There was a trend of improvement in most autonomic function tests after 3 months, comparable to the recovery of motor function. In conclusion, subclinical autonomic dysfunction was present in mild GBS. It was temporary and would resolve spontaneously.