Acute paraparesis in HIV-infected patient after initiation of highly active antiretroviral therapy.

Neurological syndromes occur in around 40-70% of HIV-infected people. Direct central nervous system involvement by the virus usually manifests as HIV encephalitis, HIV leucoencephalopathy, vacuolar leucoencephalopathy or vacuolar myelopathy. Indirect involvement is usually associated with neurotropic opportunistic infections which include tuberculosis, toxoplasmosis, cryptococcosis and viral encephalitis such as herpes simplex, varicella-zoster, cytomegalovirus and Human polyomavirus 2. We report a case of transverse myelitis in a recently diagnosed HIV patient who was otherwise asymptomatic initially and developed paraparesis after 1 month of initiation of antiretroviral therapy. After ruling out opportunistic infections and other causes of compressive and non-compressive myelopathy, development of transverse myelitis was attributed to immune reconstitution inflammatory syndrome in view of baseline low CD4 count and their improvement after HAART initiation. Prompt treatment with corticosteroids successfully reversed the symptoms.

Acute myelitis associated with dengue infection.

OBJECTIVES: To present a rare neurological complication of dengue fever. PATIENTS AND METHODS: A 24-year-old female presented with acute myelitis seven days after dengue fever onset. RESULTS: The patient presented with intense fever. The day-7 examination revealed a paraparesis, T2 sensory level, and urinary retention. The patient complained of electric discharges in the four limbs. The sitting and standing positions were impossible. An MRI of the spinal cord performed on day 8 revealed diffuse medullar hyper intense lesions on T2-weighted sequences at the cervical and thoracic levels, with enhancement of the thoracic lesion after gadolinium injection. Laboratory tests revealed positive dengue antigen on day 5 and positive IgM/IgG on day 8. Treatment with intravenous pulse methylprednisolone was initiated. CONCLUSION: Dengue virus has not often been reported as a cause of myelitis. Physicians must be aware of this rare complication in patients living in or coming from endemic areas.

Triptan-induced Reversible Cerebral Vasoconstriction Syndrome Presenting With Thunderclap Headache and Paraparesis.

Reversible cerebral vasoconstriction syndrome is a rare clinical syndrome characterized by sudden thunderclap headache often an under diagnosed neurological emergency. It is often provoked by postpartum state or exposure to provocative drugs. We report a rare case of Rizatriptan-induced reversible cerebral vasoconstriction syndrome presenting with thunderclap headache and paraparesis with complete recovery of neurological and imaging findings.

Improved strength on 5-hydroxytryptophan and carbidopa in spinal cord atrophy.

There is ample evidence of an important role of descending serotonergic projections in modulating spinal motor neuron activation and firing, and experimental studies suggest that 5-HT receptor stimulation can improve motor function after spinal cord injury; however, relevant clinical data is sorely lacking. We describe two sisters with hemiplegic migraine, low CSF and platelet serotonin levels, and progressive spastic paraparesis associated with profound spinal cord atrophy whose lower extremity strength and ambulation responded to a precursor replacement strategy (5-hydroxytryptophan and carbidopa administration), an approach that may have broader applicability in myelopathies of diverse etiology where descending serotonergic projections are compromised.

Vitamin B12 deficiency: an unusual cause of vocal fold palsy.

OBJECTIVE: We report an extremely rare case of vocal fold palsy secondary to vitamin B12 deficiency. METHOD: Case report and English-Language review of the world literature concerning vitamin B12 deficiency and its neurological manifestations. We discuss the physiological role of vitamin B and its specific relationship to the presented patient's symptoms. RESULTS: We describe a rare instance of a patient presenting with bilateral lower limb weakness and unilateral vocal fold palsy, as a manifestation of vitamin B12 deficiency. Quick recognition and treatment resulted in a full recovery. DISCUSSION: To our knowledge, this is the first reported case of unilateral vocal fold palsy secondary to vitamin B12 deficiency. Central and peripheral neuropathies have been described; however, other than the optic nerve, the cranial nerves are very rarely affected. It is important to consider vitamin B12 deficiency as a cause, as speedy identification and treatment can help prevent permanent neurological damage.

Treatment of spontaneous cervical spinal subdural hematoma with methylprednisolone pulse therapy.

We report herein a case of hyperacute onset of spontaneous cervical spinal subdural hematoma treated with methylprednisolone pulse therapy that showed good results. A 57-year-old man was admitted for posterior neck pain and paraparesis which occurred an hour ago. MRI revealed a ventral subdural hematoma distributed from the level of C1 down to T3, compressing the spinal cord. Conservative management with methylprednisolone pulse therapy was administered considering the patient's poor general condition. Although emergent surgical decompression is necessary in most cases of spinal subdural hematoma, conservative management with steroid therapy could be effective.

A case of sensory ataxia as the presenting manifestation of neurosarcoidosis.

Sarcoidosis rarely selectively affects the cauda equina with characteristic motor and sensory impairments.Using imaging, we report a case of cauda equina polyradiculopathy presenting with progressive sensory ataxia without clinical or electrophysiological evidence of motor involvement. Neurosarcoidosis was diagnosed pathologically by proximal dorsal root biopsy after systemic investigations for inflammatory, infectious, and neoplastic etiologies were found to be negative. There was clinical and radiographic improvement with corticosteroids. In addition, we review previously reported cases of cauda equina sarcoidosis.

Treatment of Spontaneous Cervical Spinal Subdural Hematoma with Methylprednisolone Pulse Therapy

We report herein a case of hyperacute onset of spontaneous cervical spinal subdural hematoma treated with methylprednisolone pulse therapy that showed good results. A 57-year-old man was admitted for posterior neck pain and paraparesis which occurred an hour ago. MRI revealed a ventral subdural hematoma distributed from the level of C1 down to T3, compressing the spinal cord. Conservative management with methylprednisolone pulse therapy was administered considering the patient's poor general condition. Although emergent surgical decompression is necessary in most cases of spinal subdural hematoma, conservative management with steroid therapy could be effective.

Intravenous immunoglobulin for the treatment of diabetic lumbosacral radiculoplexus neuropathy.

OBJECTIVE: The objective of this study was to evaluate the effect of intravenous immunoglobulin (IVIg) therapy in diabetic lumbosacral radiculoplexus neuropathy (DLRPN) patients who did not respond to analgesic drug therapy and corticosteroids. Background. DLRPN is a rare painful condition that may occur in diabetes mellitus (DM). At the moment, there are limited therapeutic options for DLRPN. METHODS: We recruited five patients affected by type 2 DM and DLRPN. They were selected from a cohort of 13 consecutive DLRPN patients. Inclusion criteria were severe pain (visual analog scale [VAS] > 4/10) and no response to pain symptomatic therapy and corticosteroids. Patients were treated with IVIg (0.4 g/kg/day for 5 days). Outcome measures were VAS, time of onset and duration of pain relief, the Medical Research Council (MRC) scale for lower limb muscle strength, and walking distance. Electrophysiology and needle electromyography (EMG) were retested after IVIg. RESULTS: Four of the patients had positive pain response after IVIg. VAS reduction started 5-10 days after IVIg infusion. Two patients underwent additional IVIg infusions due to pain reappearance after 7-18 months, again with positive response. VAS, MRC scale, and walking distance significantly improved at 1 month (Wilcoxon nonparametric test, two-tailed, P < 0.05). Electrodiagnostic testing was unchanged, but needle EMG showed reduction of denervation signs after IVIg. CONCLUSIONS: IVIg may rapidly reduce pain and improve motor function in DLRPN despite previous negative response to corticosteroids. IVIg may be repeated in those patients who experience disease relapse. Future double-blind trials are needed to evaluate the role of IVIg in DLRPN.

AUY954, a selective S1P(1) modulator, prevents experimental autoimmune neuritis.

Experimental autoimmune neuritis (EAN) is a T cell-mediated autoimmune inflammatory demyelinating disease of the peripheral nervous system and an animal model of human inflammatory demyelinating polyradiculoneuropathy. AUY954, which targets selectively the sphingosine-1-phosphate receptor 1 (S1P(1)), is known to sequester lymphocytes into secondary lymphoid tissues. In EAN rats, AUY954 greatly prevented paraparesis if administrated from the day of immunization. T cell, B cell, and macrophage infiltration, inflammatory demyelination, and local expression of interleukine-17 and matrix metalloproteinase-9 in sciatic nerves of EAN rats were significantly decreased by AUY954 treatment. Therefore, S1P(1) modulation might be a potential treatment option for inflammatory neuropathies.

Improved functional recovery with oxandrolone after spinal cord injury in rats.

At present, only the corticosteroid, methylprednisolone, is used for acute spinal cord injury to improve function. However, improvements are modest, and are associated with myopathy and immunosuppression so that alternative treatments are needed. Oxandrolone is an androgenic steroid with potential neuroprotective properties that is used to prevent muscle loss and is not immunosuppressive. Oxandrolone increased locomotor recovery concomitant with reduced loss of cord tissue in a standard weight drop model of spinal cord contusion injury indicating oxandrolone as a possible alternative to methylprednisolone. Oxandrolone also increased axonal sprouting within the ventral horns distal to the injury consistent with formation of relay circuits mediating locomotor recovery.

Mycophenolate mofetil and intravenous dexamethasone in the treatment of persistent lupus myelitis.

Methylprednisolone and cyclophosphamide pulse therapies are the most commonly used for transverse myelopathy in neuropsychiatric lupus. Little is known about the efficacy of other immunosuppressors. We describe the case of a 33-year-old woman with systemic lupus erythematosus, who developed a tranverse myelopathy, beginning with a hiccup due to involvement of the medulla oblongata; despite pulses of methylprednisolone plus azathioprine and cyclosporine therapy, she developed paraparesis with involvement of the cervical spine cord. After oral cyclophosphamide, the lesion remained active. Subsequent therapy with mycophenolate mofetil and continuous intravenous infusions of dexamethasone resulted in reduction of the lesion's size, disappearance of magnetic resonance imaging enhancement, and a complete recovery.

Spinal cord ganglioglioma presenting as acute paraparesis.

A 17-year-old male presented with acute onset paraparesis in the lower limbs. Urinary retention was present and the patient required catheterisation. Clinical examination confirmed severe bilateral lower limb weakness and a sensory level at T8. Magnetic resonance imaging (MRI) revealed a haemorrhagic intramedullary tumour extending from T8 to the conus. Microsurgical excision of the tumour was performed and the patient made a good functional recovery. The histology of the tumour demonstrated a ganglioglioma of the spinal cord. Acute paraparesis has not previously been reported with a spinal cord ganglioglioma. We discuss the clinical, diagnostic and pathological features of spinal cord gangliogliomas.

Hydroxyurea therapy in paraparesis and cauda equina syndrome due to extramedullary haematopoiesis in thalassaemia: improvement of clinical and haematological parameters.

Patients with beta-globin disorders show amelioration of clinical condition by sustained synthesis of fetal haemoglobin in adult life. We report data on a patient with beta(o)-thalassaemia genotype and thalassaemia intermedia clinical phenotype. He received therapy with hydroxyurea (20 mg/kg/d) because of the presence of extramedullary masses causing paraparesis, neurogenic bladder and impotence. During therapy, the patient showed an improved clinical picture and a significant increase in total Hb (from 71.8 to 103.2 g/L) and a gamma/alpha globin synthetic ratio (from 0.39 to 0.68). The myelosuppressive effect of hydroxyurea was revealed by a decrease in CFU-GEMM, BFU-E, and CFU-GM. Therefore hydroxyurea can be effective in the treatment of patients with extramedullary haematopoiesis (EMH) who are not transfusion-dependent and cannot be treated with radiotherapy.