Clinical Reasoning: A 40-Year-Old Woman Presenting With Encephalopathy and Paraparesis.

Patients with acute to subacute multifocal neurologic abnormalities often have a unique presentation, and their diagnosis and management can be challenging. We present the case of a 40-year-old patient who presented with a 4-day history of confusion, bradyphrenia, right facial droop, bilateral lower limb weakness, urinary incontinence, and hypothermia. This case highlights the diagnostic approach to patients with subacute multifocal neurologic abnormalities, the importance of considering coexisting systemic illnesses in the diagnosis, and their management. Readers will explore the diagnostic steps our group has considered to reach our final diagnosis and the importance of management for our leading diagnosis.

Prevention and management of spinal cord ischemia following aortic surgery: A survey of contemporary practice.

OBJECTIVE: Spinal cord ischemia (SCI) is a devastating complication of thoracoabdominal aortic aneurysm repair. We aim to characterize current practices pertaining to SCI prevention and treatment across Canada. METHODS: Two questionnaires were developed by the Canadian Thoracic Aortic Collaborative and the Canadian Cardiovascular Critical Care Society targeting aortic surgeons and intensivists. A list of experts in the management of patients at risk of SCI was developed, with representation from each of the Canadian centers that perform complex aortic surgery. RESULTS: The response rate was 91% for both intensivists (21/23), and from cardiac and vascular surgeons (39/43). Most surgeons agreed that staging is important during endovascular repair of extent II thoracoabdominal aortic aneurysm (60%) but not for open repair (34%). All of the surgeons felt prophylactic lumbar drains were effective in reducing SCI, whereas only 66.7% of intensivists felt that lumbar drains were effective (P < .001). There was consensus among surgeons over when to employ lumbar drains. A majority of surgeons preferred to keep the hemoglobin over 100 g/L if the patient demonstrated loss of lower-extremity function, whereas most intensivists felt a target of 80 g/L was adequate (P < .001). Management of perioperative antihypertensives, use of intraoperative adjuncts, and management of venous thromboembolism prophylaxis in the presence of a lumbar drain, were highly variable. CONCLUSIONS: We observed some consensus but considerable variability in the approach to SCI prevention and management across Canada. Future studies focused on the areas of variability may lead to more consistent and improved care for this high-risk population.

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.

Rosuvastatin-related rhabdomyolysis causing severe proximal paraparesis and acute kidney injury.

We describe the case of a 76-year-old man who presented with bilateral lower limb weakness associated with decreased urine output. His initial blood results showed acute kidney injury (AKI) stage 3 with substantially raised serum creatine kinase concentration of 37 950 IU/L (normal range <171 U/L). He had been on high-dose rosuvastatin for 4 years with a recent brand change occurring 1 week prior to onset of symptoms. There was no history of pre-existing neuromuscular disease. Statin-related rhabdomyolysis was suspected and rosuvastatin was withheld. His muscle strength gradually improved. He required haemodialysis for 10 weeks. He was discharged home after a complicated course of hospitalisation. His renal function improved and he became dialysis-independent; however, he was left with residual chronic kidney disease.

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.

Complete clinical and functional recovery following low-dose methotrexate related paraparesis in a patient with compound c.1298A>C AND c.677C>T MTHFR polymorphism: A case report.

RATIONALE: The mechanisms of action of MTX (methotrexate) in the treatment of RA (rheumatoid arthritis) and PsA (psoriatic arthritis) is related to its antifolic activity, due to the high affinity for enzymes that require folate cofactors as dihydrofolate reductase and to the anti-inflammatory activity derivated from the inhibition of thymidylate synthetase that leads to the over-production of adenosine. PATIENT CONCERNS: Our patient was a 41-year-old female, affected by PsA in treatment since 2 years with low-dose methylprednisolone and low-dose subcutaneous MTX. The treatment was effective. The patient subacutely developed a severe paraparesis with impossibility of gait or standing without aid and was admitted to a Neurology Department where the cause of the paraparesis was not clear in spite of accurate radiological neurophysiologic and laboratory tests. Therefore, she was admitted in a rehabilitation unit. DIAGNOSIS AND INTERVENTIONS: Paraparesis in PsA patient in treatment with methotrexate. MTX toxicity was hypothesized; therefore the drug was discontinued while i.m. folic acid and cyanocobalamin were administered for 20 days. The diagnosis was clinical, based on neurological examination (paraparesis) and on the chronic use of MTX (hypothesis of toxicity). OUTCOMES: The patient obtained a complete resolution of paraparesis. Genetic analyses showed associated a compound heterozygosity for the c.1298A>C and c.677C>T variants of methylenetetrahydrofolate reductase (MTHFR) gene. LESSONS: Neurological side effects of MTX are uncommon. In literature no previous case of MTX induced paraparesis in patients treated with low-dose MTX for chronic arthritis has been described. The association between the gene polymorphisms of MTHFR (c.1298A>C and c.677C>T) and MTX toxicity in arthritis patients is confirmed. The case also confirms that folates are a precious antidote of MTX toxicity.

Hereditary ataxias and paraparesias: clinical and genetic update.

PURPOSE OF REVIEW: This review aims at updating the clinical and genetic aspects of hereditary spastic paraplegias (HSPs) and hereditary cerebellar ataxias (HCAs), focusing on the concept of spastic-ataxia phenotypic spectrum and on newly identified clinical overlaps with other neurological and nonneurological diseases. RECENT FINDINGS: Next-generation sequencing (NGS) has allowed the discovery of new genes involved in HSPs and HCAs. They include new HCAs genes such as GRM1 (SCA44), FAT2 (SCA45), PLD3 (SCA46), SCYL1 (SCAR21), UBA5 (SCAR24) and XRCC1 (SCAR26) as well as CAPN1 (SPG76) and CPT1C (SPG73) in HSPs. Furthermore, NGS allowed enriching known genes phenotype, reinforcing the overlap between HSPs and HCAs defining the spastic ataxia spectrum. Clear examples are the expanded phenotypes associated with mutations in SPG7, PNPLA6, GBA2, KIF1C, CYP7B1, FA2H, ATP13A2 and many others. Moreover, other genes not previously linked to HCAs and HSPs have been implicated in spastic or ataxic phenotypes. SUMMARY: The increase of HSPs and HCAs-related phenotypes and the continuous discovery of genes complicate clinical diagnostic in practice but, at the same time, it helps highlighting common pathological pathways, therefore opening new ways to the development of common therapeutic approaches.

Relationship between clinical and radiologic findings of spinal cord injury in decompression sickness.

BACKGROUND: Decompression sickness may involve the central nervous system. The most common site is spinal cord. This study was conducted to determine the relationship between magnetic resonance(MR) imaging findings of spinal damage. METHODS: We conducted a retrospective review of 12 patients (male=10, female=2) who presented with spinal cord symptoms. We investigated their clinical features, neurological findings and radiologic findings. RESULTS: The depth and bottom time of the dive were 34.5 meters (range 22-56) and 22.7 minutes (range 10-55) respectively. Most divers ascended within appropriate time frame as shown by the decompression tables. The most frequent initial symptoms were lower limb weakness (n=12), followed by sensory disturbances (n=10) and bladder dysfuction (n=5). The chief radiologic abnormalities were continuous (n=3), or non-continuous (n=5) high-signal intensity on T2-weighted images at posterior paramedian portion of the spinal cord, mainly thoracic level. There were no abnormal findings in the remaining four (4) patients, and they showed good prognosis. All patients were treated with hyperbaric oxygen therapy and some received high-dose dexamethasone. On discharge, five (5) patients had made a full recovery, seven (7) had some residual neurological sequelae, and all patients except one (1) regained normal bladder function. CONCLUSIONS: Spinal cord decompression sickness is a neurological emergency. Early recognition and treatment may minimize neurological damage. Initial normal finding in MR imaging was a good predictor for prognosis in spinal decompression sickness.

Motor exam of patients with spinal cord injury: a terminological imbroglio.

The description of the motor deficit of patients with spinal cord injury (SCI) varies significantly, leading to confusion within the neurological terminology. This paper proposes a concise and easy to use terminology to describe the motor deficit of patients with SCI. A broad review of the origin of the nomenclature used to describe the motor deficit of patients with SCI was performed and discussed. The prefix: "hemi" should be used to describe paralysis of one half of the body; "mono" for one limb; "para" for lower limbs, di" for two symmetrical segments and/or parts in both sides of the body; "tri" for three limbs, or two limbs and one side of the face; and "tetra" for four limbs. The suffix: "plegia" should be used for total paralysis of a limb or part of the body, and "paresis" for partial paralysis. The term "brachial" refers to the upper limbs; and "podal" to the lower limbs. According to the spinal cord origin of the main key muscles for the limbs, patients with complete injury affecting spinal cord segments C1-5 usually presents with "tetraplegia"; C6-T1 presents with "paraplegia and brachial diparesis"; T2-L2 with "paraplegia"; and L3-S1 with "paraparesis".

Massive Lumbar Disk Herniation Following "Therapeutic" Water Boiling of the Lower Extremities: Case Report and Literature Review.

BACKGROUND: Legs burning for treating lumbar radicular pain are still in use nowadays in low socioeconomical environments. They are dangerous as the case we report shows clearly. CLINICAL CASE: A 49-year-old man came to our attention with severe flaccid paraparesis occurred 10 days before, almost immediately after he had immersed his legs in boiling water to treat his severe left lumbocrural pain. This was known to be due to a right L3/4 herniated disk diagnosed by magnetic resonance imaging. At the examination he showed severe motor paresis and absent reflexes of his lower limbs, while crural pain was mild and sensation and urinary function were unaffected. The results of his neurologic examination led us to suspect an acute motor axon degeneration related to thermal shock. Lumbar magnetic resonance imaging, performed before the planned electromyogram as an exception to the established routine, showed instead a giant 5- × 5.5-cm, herniated disk compressing the dural sac at L3. CONCLUSIONS: Prompt surgical decompression led to rapid improvement. We discuss here the pathophysiology of this unusual case and point out how medieval practices for treating sciatica-like pain are not only unjustified from a medical viewpoint but also potentially dangerous.

Warfarin-associated Intraspinal Hematoma.

Intracerebral hemorrhage is a well-known complication resulting from warfarin use; however, warfarin-associated intraspinal hematoma is very rare. Warfarin-associated intraspinal hematoma may exhibit delayed progression, and patients may present with atypical symptoms, occasionally resulting in delayed diagnosis. We report the case of a 65-year-old man who visited our emergency department (ED) with acute urinary retention. He had been previously diagnosed with non-valvular atrial fibrillation, arterial hypertension, and benign prostatic hyperplasia, and he used warfarin for the prevention of systemic embolism. The patient was initially diagnosed with worsening of the prostatic hyperplasia. After 2 days, he revisited the ED with painless paraparesis. Magnetic resonance imaging of the thoracic spine revealed an intraspinal hematoma at Th7-8, and blood coagulation tests indicated a prothrombin time-international normalized ratio of 3.33. Despite attempts to reverse the effects of warfarin with vitamin K administration, the paraparesis progressed to paraplegia, necessitating urgent surgical removal of the hematoma. Partial recovery of motor function was evident after surgery. From the present case, we learned that intraspinal hematoma should be included in the differential diagnosis of patients using warfarin who present with acute urinary retention. Although there are no evidence-based treatment guidelines for warfarin-associated intraspinal hematoma, surgical treatment may be warranted for those who exhibit neurological deterioration.