Case report: Mononeuritis multiplex in the course of dengue fever.

BACKGROUND: Dengue fever usually presents as a self-limiting acute febrile illness with worsening thrombocytopenia, with a small minority of patients developing hemorrhagic or life-threatening complications. Organ specific manifestations like myocarditis, acalculous cholecystitis, encephalitis has been described but are uncommon presentations. Even more rarely, such manifestations are the presenting complaint of Dengue fever. In this case report, we highlight a case of Dengue fever where unrelated neuropathies were the presenting complaint. CASE PRESENTATION: An elderly man presents with 1 day of diplopia and left foot drop, associated with 2 days history of fever. A decreasing white cell count (WBC) and platelet on the 2nd day of admission prompted Dengue virus to be tested and a positive NS-1 antigen was detected, confirming the diagnosis of Dengue fever. He was treated with supportive treatment with a short duration of intravenous fluids recovered uneventfully and was discharged 6 days after admission with almost full resolution of diplopia and partial resolution of left foot drop. Left foot drop recovered completely 2 weeks later. CONCLUSION: Neurological manifestations can be the presenting symptoms in Dengue fever, a diagnosis which should be borne in mind when such symptoms present in patients from endemic areas or in returning travellers from these areas.

Clinicopathological case: progressive cognitive decline with gait disturbance in a steel worker.

A 57-year-old male steel plant worker presented with fatigue and altered liver function tests. Over the next two years, he developed cognitive decline, parkinsonism and seizures. This paper reports the clinicopathological conference at the 37th Edinburgh Advanced Neurology Course 2015 and outlines what we can learn from this case.

Correlating brain volume and callosal thickness with clinical and laboratory indicators of disease severity in children with HIV-related brain disease.

BACKGROUND: Objective MRI markers of central nervous system disease severity may precede subjective features of HIV encephalopathy in children. Previous work in HIV-infected adults shows that brain atrophy was associated with low CD4 and with neuropsychological impairment. Significant thinning of the corpus callosum (CC), predominantly anteriorly, was also found in HIV-infected adults and correlated with CD4 levels. These findings have not been tested in children. PURPOSE: The aim of this study was to determine if brain volume and midsagittal CC linear measurements (thickness and length) on MRI in children with HIV-related brain disease correlate with clinical and laboratory parameters of disease severity. METHODS: Retrospective MRI analysis in children with HIV-related brain disease used a volumetric analysis software and a semi-automated tool to measure brain volume and callosal thickness/length, respectively. Each measure was correlated with clinical parameters of disease severity including Griffiths Mental Development scores (GMDS), absolute CD4 counts (cells/mm(3)), nadir CD4 (the lowest CD4 recorded, excluding baseline), duration of HAART, and decreased brain growth. RESULTS: Thirty-three children with HIV-related brain disease were included. Premotor segment of the CC mean thickness correlated with age (p = 0.394). Motor CC maximum thickness correlated significantly with general developmental quotient (p = 0.0277); CC length correlated with a diagnosis of acquired microcephaly (p = 0.0071) and to CD4 level closest to date of the MRI scan (p = 0.04). CONCLUSIONS: Length of the CC and the "motor CC segment" may represent surrogate clinical biomarkers of central nervous system disease severity and with decreased level of immunity in HIV-infected patients that precede established HIV encephalopathy.

Detection and characterization of a new astrovirus in chicken and turkeys with enteric and locomotion disorders.

In the present paper, we report the unexpected discovery of a new virus in samples from chicken and turkey flocks with clinical disorders such as tenosynovitis, enteric problems, or runting and/or stunting-like conditions. Since 1987, several virus isolation attempts on samples from these flocks resulted in the same macroscopic characteristic lesions in embryonated specific pathogen free eggs, being mortality with bright-red discolouration of legs and wing-tips, a swollen dark-red liver and oedema. Initial work suggested the presence of an agent with characteristics of a non-enveloped RNA virus. Further work, which is described in this paper, showed that the isolated strains formed a new group of avian nephritis viruses, which is genetically and antigenically distinct from known avian astroviruses. Inoculation of a representative strain (isolate 19) of this new group of avian nephritis viruses, provisionally named avian nephritis virus-3, in specific pathogen free layer chicks resulted in diarrhoea, runting and stunting, and even mortality.

Herpes zoster-induced trunk muscle paresis presenting with abdominal wall pseudohernia, scoliosis, and gait disturbance and its rehabilitation: a case report.

Herpes zoster (HZ)-induced abdominal wall pseudohernia has been frequently reported, but there has been no report describing HZ-induced trunk muscle paresis leading to functional problems. We describe a 73-year-old man with T12 and L1 segmental paresis caused by HZ presenting with abdominal wall pseudohernia, scoliosis, and standing and gait disturbance who responded well to a systematic rehabilitation approach. He first noticed a right abdominal bulge in the 6th postherpetic week, which was gradually accompanied by right convex thoracolumbar scoliosis, pain, and standing and gait disturbance in the 12th week. Needle electromyography revealed abnormal spontaneous activities at rest in the right T12 myotomal muscles, and motor unit recruitment was markedly decreased. We arranged an outpatient rehabilitation program consisting of using a soft thoracolumbosacral orthosis for pain relief and trunk stability, muscle reeducation of the paretic abdominal muscles, strengthening of the disused trunk and extremity muscles, and gait exercise. Based on electromyographic findings, we instructed him in an effective method of muscle reeducation. After 4 months of rehabilitation, he showed marked improvement and became an outdoor ambulator. We suggest that electromyography is a useful tool to evaluate clinical status and devise an effective rehabilitation program in patients with HZ trunk paresis.

Direct comparison of demyelinating disease induced by the Daniel's strain and BeAn strain of Theiler's murine encephalomyelitis virus.

We compared CNS disease following intracerebral injection of SJL mice with Daniel's (DA) and BeAn 8386 (BeAn) strains of Theiler's murine encephalomyelitis virus (TMEV). In tissue culture, DA was more virulent then BeAn. There was a higher incidence of demyelination in the spinal cords of SJL/J mice infected with DA as compared to BeAn. However, the extent of demyelination was similar between virus strains when comparing those mice that developed demyelination. Even though BeAn infection resulted in lower incidence of demyelination in the spinal cord, these mice showed significant brain disease similar to that observed with DA. There was approximately 100 times more virus specific RNA in the CNS of DA infected mice as compared to BeAn infected mice. This was reflected by more virus antigen positive cells (macrophages/microglia and oligodendrocytes) in the spinal cord white matter of DA infected mice as compared to BeAn. There was no difference in the brain infiltrating immune cells of DA or BeAn infected mice. However, BeAn infected mice showed higher titers of TMEV specific antibody. Functional deficits as measured by Rotarod were more severe in DA infected versus BeAn infected mice. These findings indicate that the diseases induced by DA or BeAn are distinct.

JC polyomavirus granule cell neuronopathy in a patient treated with rituximab.

IMPORTANCE: Progressive multifocal leukoencephalopathy results from lytic infection of the glia by the JC polyomavirus (JCV); JCV granule cell neuronopathy is caused by infection with a mutated form of JCV, leading to a shift in viral tropism from the glia to cerebellar granule cells. This shift results in a clinical syndrome dominated by progressive cerebellar dysfunction that might elude standard diagnostic workup strategies for ataxia. OBSERVATIONS: We present the case report of a patient receiving long-term rituximab therapy who developed progressive cerebellar ataxia and marked isolated cerebellar degeneration. This syndrome resulted from JCV granule cell neuronopathy associated with a novel JCV mutation. CONCLUSIONS AND RELEVANCE: New onset or worsening of isolated cerebellar ataxia in patients being treated with rituximab or natalizumab warrants early assessment for JCV infection.

Muscle strength is only a weak to moderate predictor of gait performance in persons with late effects of polio.

OBJECTIVE: To assess muscle strength in the knee extensors, knee flexors and ankle dorsiflexors in persons with late effects of polio, and determine how much muscle strength, gender, age and BMI are related to gait performance. METHODS: Ninety community-dwelling ambulant persons (47 men and 43 women; mean age 64 years SD 8) with late effects of polio participated. Isokinetic concentric knee extensor and flexor muscle strength was measured at 60°/s and ankle dorsiflexor muscle strength at 30°/s. Gait performance was assessed by the Timed "Up & Go", the Comfortable and Fast Gait Speed tests, and the 6-Minute Walk test. RESULTS: There were significant correlations between knee extensor and flexor muscle strength and gait performance (p < 0.01), and between ankle dorsiflexor muscle strength and gait performance (p < 0.05), for both lower limbs. Muscle strength in the knee extensors and flexors explained 7% to 37% and 9% to 47%, respectively, of the variance in gait performance. Strength in the ankle dorsiflexors explained 4% to 24%, whereas gender, age and BMI contributed at most an additional 9%. CONCLUSION: Knee muscle strength, and to some extent ankle dorsiflexor muscle strength, are predictors of gait performance in persons with late effects of polio, but the strength of the relationships indicates that other factors are also important.

Motor radiculopathy.

A 48-year-old immunosuppressed woman presented to a rheumatology follow-up clinic after suffering from herpes zoster infection. She had manifestations of foot drop 3 months after the initial infection. She was diagnosed with motor radiculopathy following herpes zoster infection that was effectively managed by physiotherapy and amitriptyline.

[Primary Epstein-Barr virus infections with neurological complications in two middle-aged men]. / Primær Epstein-Barr-virus-infektion med neurologiske komplikationer hos to midaldrende mænd.

Primary infections with Epstein-Barr virus (EBV) often lead to infectious mononucleosis with sore throat, lymphadenopathy and hepatitis, especially in youngsters. However, neurological complications can occur even in immunocompetent individuals. We report two case stories of two middle-aged men with primary EBV infections who presented severe neurological manifestations of the disease, but both fully recovered. Hepatitis was present in both cases, but not the classical mononucleosis. The cases stress that clinicians should be aware of these rare courses of primary EBV infections.

Herpes zoster virus: an unusual but potentially treatable cause of sciatica and foot drop.

The herpes zoster virus is a rare but potential cause of acute motor weakness. This article describes 2 patients with drop foot secondary to an infection of varicella zoster who were incorrectly referred to an orthopedic clinic from their general practitioners. The first patient was a 74-year-old man who presented with weakness in the right foot and a vesicular rash. The pattern of disease supported the clinical diagnosis of shingles affecting the L5 motor and sensory division. No investigation was required, and the patient was treated with a foot drop splint. The second patient was a 71-year-old man who presented with right leg and foot weakness and a vesicular rash affecting his right buttock and posterior right thigh. Lumbar magnetic resonance excluded a stenotic lesion; electrophysiological studies supported the diagnosis of a lower motor neuron lesion. The patient was treated with a 1-week course of acyclovir and a foot drop splint. The correct diagnosis will aid in correct referral and will prompt management, which will potentially provide a faster and better outcome for the patient.

Acute wrist and foot drop associated with hepatitis C virus related mixed cryoglobulinemia: rapid response to treatment with rituximab.

The nature of the B-cell subsets associated with chronic hepatitis C virus related type II mixed cryoglobulinemia (HCV-MC) is unclear. We report the case of a 64-year-male with acute onset wrist drop and foot drop, secondary to HCV-MC related mononeuritis multiplex, who was treated with rituximab, an anti-CD20(+) antibody directed against B cells. We monitored the frequency of B-cell subsets in peripheral blood before and after rituximab, and correlated B-cell subset changes with clinical response. Significant improvements in his wrist and foot drop, as well as his vasculitic rash, depression and erectile dysfunction were evident within six days of starting rituximab and have persisted several months after B-cell recovery. More than 95% of CD20(+) B cells had disappeared from peripheral blood within 1 week, returning to baseline by week 21. CD20(+)CXCR3(+) frequency at baseline was similar to that at week 21. CD20(+)CD5(+), the human equivalent of B1 B cells and CD20(+)IgM(+)IgD(+), naïve B cells were increased. By contrast, CD20(+)CD27(+) memory cell frequency was reduced. These data suggest that CD27(+) memory B cells, but not CD5(+) and IgM(+)IgD(+) B cells may play a role in the clinical manifestations of cryoglobulinemia.

Herpes zoster radiculopathy treated with fluoroscopically-guided selective nerve root injection.

BACKGROUND: Varicella-zoster virus, a member of the herpes virus family, is a neurotrophic virus that primarily affects afferent sensory neurons. Reactivation of latent virus within the dorsal root ganglion and axoplasmic transport to epithelial nerve terminals causes the segmental cutaneous rash and neuralgic pain characteristic of herpes zoster. SETTING: Outpatient orthopedic practice. CASE DESCRIPTION: A 75-year-old male developed a herpetic rash followed by burning pain in the right L5 distribution. The pain was exacerbated by standing or walking. Six weeks later, the rash had improved, but the patient developed a right foot drop requiring use of a molded ankle-foot orthosis. MRI of the lumbar spine revealed mild degenerative changes without evidence of significant spinal stenosis or disc disease. Electrodiagnostic studies confirmed the diagnosis of right L5 radiculopathy. RESULTS: The patient had dramatic improvement of pain and weakness after undergoing a fluoroscopically guided right L5 selective nerve root block with Depo-Medrol and Lidocaine. He then began a course of physical therapy and, 6 weeks later, had only trace weakness of the ankle dorsiflexor group on the right side. The patient has continued without significant weakness or pain since the procedure and has returned to normal functioning. DISCUSSION: This case demonstrates apparent treatment of a relatively uncommon phenomenon, herpes zoster radiculopathy, using selective nerve root block. LIMITATIONS: There is a limited amount of data regarding this disorder presently available regarding Herpes Zoster Radiculopathy. A second limitation would be an inability to exclude spinal pathology as an alternative etiology of this patient's condition. CONCLUSION: Cases of herpes zoster-induced radiculopathy may become more frequent, as evidenced by the increasing number of cases of herpes zoster in the United States noted epidemiologically.

HTLV-I proviral load correlates with progression of motor disability in HAM/TSP: analysis of 239 HAM/TSP patients including 64 patients followed up for 10 years.

To clarify clinical and laboratory findings that may be related to the pathomechanism of HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP), we analyzed these findings in 239 patients with HAM/TSP, including 64 patients followed up for 10 years after their first examinations, with special interest in the HTLV-I proviral load in peripheral blood mononuclear cells (PBMCs). The proviral load in PBMCs did not differ in terms of modes of HTLV-I transmission. However, the proviral load in patients with age of disease onset greater than 65 years tended to be higher than those with a younger age of onset. In the 64 patients followed up for 10 years, the clinical symptoms deteriorated in 36 patients (56%), unchanged in 26 patients (41%), and improved in 2 patients (3%). HTLV-I proviral load also appeared to be related to the deterioration of motor disability in these patients. To our knowledge, the present study is the first longitudinal study concerning the relationship between the clinical course of HAM/TSP and HTLV-I proviral load. It is suggested that HTLV-I proviral load is related to the progression of motor disability and is an important factor to predict prognosis of patients with HAM/TSP.

Subacute sensory neuropathy associated with Epstein-Barr virus.

A 35-year-old man experienced severe sensory loss, pseudoathetosis, and areflexia during recovery from a severe viral illness. Sensory nerve action potentials were absent, motor conduction velocities were mildly slowed, and blink reflexes were normal. Magnetic resonance imaging (MRI) revealed abnormal signal within the central and dorsal aspects of the thoracic cord. Acute and convalescent Epstein-Barr virus (EBV) titers suggested EBV as the etiology. Subacute sensory neuropathy, with peripheral and central nervous system involvement, is a rare complication of EBV infection.