Natural history of longitudinally extensive transverse myelitis in 35 Hispanic patients with systemic lupus erythematosus: good short-term functional outcome and paradoxical increase in long-term mortality.

Background and objective Acute transverse myelitis (TM) is an infrequent neurological complication of systemic lupus erythematosus (SLE). Short-term outcome varies widely between cohorts. Little is known about the epidemiology and long-term functional outcome of TM associated to SLE. Methods Patients with SLE and acute TM were identified during hospital admission, visits to the Emergency Room or the Neurology Outpatient Clinic. We evaluated ambispectively those patients with SLE presenting with clinical myelopathy and corroborated with spinal MRI. Cases were divided as partial (non-paralyzing) or complete (paralyzing). We determined long-term functional outcome as well as mortality in those patients with follow-up periods of at least five years. Results We identified 35 patients (partial, n = 15; complete, n = 20) in which complete clinical and imaging data were available (26 with follow-up ≥ 5 years). Patients with complete TM were significantly older than those with partial forms. Positive antiphospholipid antibodies were observed in 80% of patients, suggesting a possible mechanistical role. Surprisingly, functional recovery at one year was in general good; however, we observed a five-year mortality of 31% because of sepsis (in 10 cases) or pulmonary embolism (in one case). Conclusions Short-term outcome of SLE-related TM is generally good, and recurrence rate is low. However, we observed a long-term fatality rate of 31% for reasons unrelated to TM, suggesting that TM is a manifestation of severe immune dysregulation and a predictor of severity and mortality in patients with SLE.

Functional Outcomes 18 Months After Total and Midarm Transplantation: A Case Report.

BACKGROUND: The function reported after arm transplantation is deemed beneficial relative to the marked disability that upper arm amputation causes. OBJECTIVE: We report a 51-year-old man with a Disabilities of the Arm, Shoulder and Hand (DASH) score of 75.83 who underwent bilateral arm transplantation in October 2015. PROCEDURE: The right arm was transplanted at the glenohumeral joint level, including transplantation of the humeral head, joint capsule, and rotator cuff ligaments and tendons. Additionally, neurorrhaphies were performed at the origin of the terminal branches of the brachial plexus, including the axillary and musculocutaneous nerves. Therefore, this was considered a total arm transplantation. The left arm was transplanted at the transhumeral level, with complete transplantation of the biceps and triceps brachii, and terminolateral neurorrhaphy of the donor musculocutaneous nerve to the receptor radial nerve. A maintenance triple immunosuppression scheme was administered, with tacrolimus levels kept at 10 ng/mL. RESULTS: At 18 months post-transplantation, the intrinsic musculature in the left hand showed electrical registry, DASH score was 67.5, Carroll test score was 28 in both extremities, Hand Transplant Score System was 67.5 in the right extremity and 77.5 in the left extremity, and Short Form-36 score was 96.1. The patient was healthy, with restored body integrity. He could lift medium-sized weightless objects, eat and go to the bathroom by himself, drink liquids with bimanual grasp, swim, dress almost independently, and drive. CONCLUSION: The functional evolution of the patient was similar to previously reported transplanted arms, even though the right arm transplant involved the glenohumeral joint and axillary and musculocutaneous nerve repair.

Paraneoplastic syndromes associated with anti-Hu antibodies.

Paraneoplastic syndromes are disorders associated with cancer but without a direct effect of the tumor mass or its metastases on the nervous system. Small cell carcinoma of lung associated with paraneoplastic sensory neuronopathy and/or paraneoplastic encephalomyelitis with the presence of anti-Hu antibodies has been termed "anti-Hu syndrome." Anti-Hu associated PSN-PEM is an immune disorder in which both cell-mediated and humoral mechanisms are involved. Patients are considered affected by Anti-Hu associated PSN-PEM when they develop clinical signs and symptoms of CNS dysfunction and/or sensory neuropathy not caused by metastases or other disorders, and serum or cerebrospinal fluid is positive for Hu abs. SCLC is found in more than 90% of patients with cancer and positive Hu abs. Individual patients with Hu abs associated to SCLC may suffer PSN-PEM, limbic encephalitis, brainstem encephalopathy, opsoclonus-myoclonus, paraneoplastic cerebellar degeneration or myelopathy. Hu abs have a specificity of 99% and sensitivity of 82% in detecting paraneoplastic neurological syndromes. There are two types of treatment: the first is to treat the cancer, the second is to suppress the immune reaction with the use of corticosteroids, cyclophosphamide, azathioprine, plasma exchange, intravenous immunoglobulin and immunoadsorption; however, treatment of paraneoplastic syndromes is generally unsatisfactory.

[Alexia without agraphia due to the lesion in the right occipital lobe in a right-handed man. Detection of hemispheric lateralization of handedness and language in a right-handed patient]. / Alexia sin agrafia por lesión occipital derecha en varón diestro. Segregación de la lateralización hemisférica manual y del lenguaje en un paciente diestro.

INTRODUCTION AND CLINICAL CASE: A 65 year-old man, right-handed, without any family history of left handiness, suddenly developed a left homonymous hemianopia and incapacity for reading. Neurological and neuropsychological examinations showed the presence of a profound alexia with preservation of writing to dictation and spontaneously. He was unable to read what he had written. He could spell the words letter by letter but he was unable to read the complete word. MRI showed an extensive infarct in the territory of the right posterior cerebral artery. The infarct extended anteriously to the right thalamus and to the medial temporal fifth or fusiform gyrus. The splenius was spared. Brain SPECT disclosed the area of the infarct and an extensive area of decreased cerebral perfusion over the right parietal and temporal areas. CONCLUSION: Alexia without agraphia has been reported in right-handed patients with left occipital lesions and in right occipital regions in left-handed patients but rarely if ever in right occipital lesions in right-handed patients.

[A case of Hallervorden-Spatz disease with magnetic resonance imaging data]. / Un caso de enfermedad de Hallervorden-Spatz con hallazgos de resonancia magnética.

Hallervorden-Spatz disease (HSD) is an uncommon disorder, progressive and degenerative of the basal ganglia. It begins in the first or second decade of life and is characterized by a dominant extrapiramidal signs, dystonia and progressive dementia. It is autosomic recessive, although sporadic cases are seen in 15%. There is no biological marker for the disease. The post-mortem findings include iron deposits in the globus pallidum and pars reticulata of the substantia nigra. Magnetic resonance imaging (MRI) in T2 shows symmetric hypointense lesions in both globus pallidum with a hyperintense center: giving the "tiger's eye" sign. This is the first case reported in Mexico of sporadic HSD with typical clinical and MRI findings.

[The other genome: the clinical concept of mitochondrial cytopathies or disorders of pxidative phosphorylation]. / El otro genoma: el concepto clínico de las citopatías mitocondriales o enfermedades de la fosforilación oxidativa.

The investigations of mitochondrial DNA abnormalities and its relationship with derangements of oxidative phosphorylation and electron transport system, has yielded description a myriad of syndromes called mitochondrial diseases or cytopathies. The objective of this paper is to review the clinical relevant features of this heterogeneous group of diseases, to understand the board spectrum of signs and symptoms and suggest an algorithm for the diagnosis.

Horizontal appendicular dysmetria in four patients with a typical Wallenberg's syndrome.

During the acute stage of a Wallenberg's syndrome ipsilateral appendicular dysmetria is frequently seen. The dysmetria is more apparent in the ipsilateral upper extremity. These patients also have a peculiar type of dysmetric eye movements that are characterized by hypermetric saccades toward the side of the lesion and hypometric saccades to the opposite side. We examined four patients with acute Wallenberg's syndrome and found horizontal dysmetria of the affected extremity. Hypermetric arm and hand movements were present to the side of the lesion and hypometric movements toward the opposite side. This type of dysmetria is probably related to the same patophysiological mechanism that underlies dysmetric eye movements in the Wallenberg's syndrome. The dysmetria tends to disappear with time although it continues to be present in some patients six months after the ischemic damage.

[Significance of intracranial hypertension management in cryptococcal meningitis in patients with acquired immunodeficiency syndrome. Report of 2 cases]. / Importancia del manejo de la hipertensión endocraneana en meningitis criptocócica en pacientes con síndrome de inmunodeficiencia adquirida. Informe de dos casos.

Two cases of cryptococcal meningitis and increased intracranial pressure in patients with acquired immunodeficiency are described. Both patients presented high intracranial pressure that persisted despite optimal antifungal treatment (amphotericin B, 5-flucytosine initially, and fluconazole posteriorly). The elevated intracranial pressure produced headache, seizures, and reduced visual and auditory acuity. CAT scan demonstrated absence of ventricular dilatation or focal lesions. Both cases were treated with adequate antifungal therapy, as well as with repeated lumbar punctures and placement of a lumboperitoneal shunt due to the persistence of elevated intracranial pressure. One patient presented with unilateral loss of vision due to optic nerve atrophy. After one year of follow-up, one patient died due to progression of his disease, while the other is still alive and without evidence of neurological disease. Intracranial hypertension is a frequent clinical manifestation of cryptococcal meningitis in patients with acquired immunodeficiency syndrome (AIDS) that requires adequate diagnosis and management. Treatment should be directed towards the reduction of intracranial pressure though repeated lumbar punctures and, in some cases, with lumboperitoneal or ventricular-peritoneal shunts.

[Central neurogenic hyperventilation associated with a pontine infarction]. / Hiperventilación central neurogénica en infarto pontino.

Central neurogenic hyperventilation in patients with a normal level of consciousness is uncommon. This condition occurs in bilateral pontine tegmental lesions, particularly tumors such as CNS lymphomas and glioma, as well as traumatic lesions. The physiopathological mechanisms are unknown and no there is no effective treatment for this entity. We report a case of central neurogenic hyperventilation associated to a unilateral basal pontine infarction.

[Hemorrhagic stroke associated to neurocysticercosis]. / Accidente cerebrovascular hemorrágico asociado a neurocisticercosis.

A well-known complication of neurocysticercosis is cerebral arteritis, which is usually manifested by cerebral ischemia. Only anecdotal cases of hemorrhagic stroke associated to this parasitosis have been described. Previously there are only two reported cases of this association. One of these cases had an intracystic hemorrhage confirmed by autopsy without cerebrovascular risk factors. Autopsy revealed an inflammatory arteriopathy adjacent to the cyst intracystic hemorrhage. The second case had a subarachnoidal hemorrhage secondary to the rupture of an aneurysm in the right anteroinferior cerebellar artery. At surgery, the aneurysm was found to be surrounded by a thickened-leptomeninges, which histologically showed the presence of cysticercous with dense inflammation. Our first patient was a 32 year-old female developed a lenticulo-capsular hemorrhage around a cysticercotic lesion. The second patient was a 34 year-old male developed an intracystic hemorrhage. As cerebral angiography was normal in both patients, cerebral hemorrhages were considered to be related to cysticercotic arteritis of small penetrating vessels. We conclude that cysticercosis is associated with differenttypes of intracranial hemorrhage, as documented the present cases. In neurocysticercosis endemic areas, cysticercotic arteritis should be added to the list of causes of intracranial hemorrhage in young people.

The association of myasthenia gravis and connective tissue diseases. Effects of thymectomy in six cases with rheumatoid arthritis and one case with systemic lupus erythematosus.

OBJECTIVES: To describe the effects of thymectomy in a group of patients with myasthenia gravis (MG) with associated connective tissue diseases (CTD). PATIENTS AND METHODS: We analyzed six patients with CTD and myasthenia. They were followed-up for at least 3 years. RESULTS: Records of a cohort of 132 patients with established diagnosis of MG undergoing thymectomy in our institution between 1987-1999 were reviewed. The percentage of patients with CTD was 5 % (6/132). Five patients had rheumatoid arthritis (RA) and one patient systemic lupus erythematosus (SLE). All patients were women, and the mean age was 38.5 years old (SD 13.7). Mean time of MG diagnosis to operation was 16 months (range from 1 to 144 months). Preoperative Osserman classification was the following: stage IIb, four patients; stage III, one patient; and stage IV, one patient. Before surgery all patients were on anticholinesterase agent (pyridostigmine), and four patients were on corticosteroids. An extended transsternal thymectomy was practiced on five patients and a transcervical thymectomy was performed in the remaining patient. Pathologic findings were as follows: thymic hyperplasia in four patients and thymic atrophy in the other two. Good response (remission or improvement) was present in three patients (50 %) and poor response (no change or worse) in the other three (50 %). CONCLUSIONS: A low response to the thymectomy is observed in patients with MG and associated CTD (RA and an SLE).

Accidente cerebrovascular hemorrágico asociado a neurocisticercosis / Hemorrhagic stroke associated to neurocysticercosis

Una complicación bien reconocida de la neurocisticercosis es la arteritis cerebral, la cual puede manifestarse como isquemia cerebral o hemorragia. Existen reportes anecdóticos de evento vascular hemorrágico en pacientes con neurocisticercosis. En la literatura sólo hay dos reportes de esta manifestación. Uno de los casos descritos en la literatura tuvo una hemorragia intraquística en un paciente sin factores de riesgo cardiovascular. La autopsia demostró arteriopatía inflamatoria alrededor de la hemorragia intraquística. El segundo caso reportado en la literatura tuvo una hemorragia subaracnoidea secundaria a la ruptura de un aneurisma en la arteria cerebral anterior derecha. En la cirugía se encontró engrosamiento de las meninges y presencia de cisticercos con inflamación intensa alrededor del aneurisma. Nuestro primer caso se trató de una paciente de 32 años que desarrolló una hemorragia alrededor de una lesión cisticercosa. El segundo caso se trató de una mujer de 34 años que desarrolló una hemorragia intraquística. La angiografía cerebral fue normal en ambos casos y se consideró que las hemorragias se relacionaron con arteritis cisticercosa de vasos de pequeño calibre. Concluimos que la cisticercosis se asocia con diferentes tipos de hemorragia intracraneal, como los casos que mostramos en este artículo. En áreas endémicas de neurocisticercosis la arteritis cisticercosa debe ser incluida en la lista de causas de hemorragia intracraneal en pacientes jóvenes (AU)

La asociación de miastenia gravis y enfermedades del tejido conjuntivo. Efectos de timectomía en seis casos con artritisreumatoidea y un caso con lupuseritematoso sistémico / The association of myasthenia gravis and connective tissue diseases. Effects of thymectomy in six cases

Objetivos: Describir los efectos de la timectomía en un grupo de pacientes con miastenia gravis (MG) con enfermedades del tejido conectivo asociado (ETC). Pacientes y métodos: Analizamos seis pacientes con ETC y miastenia. Se les hizo un seguimiento de al menos 3 años. Resultados: Se realizó una revisión entre los años 1987-1999 en nuestra institución de los ficheros de una cohorte de 132 pacientes con diagnosis de MG establecida que sufren de timectomía. El porcentaje de pacientes con ETC fue del 5 por ciento (6/132). Cinco pacientes tuvieron artritis reumatoidea (AR) y uno lupus eritematoso sistémico (LES). Todos los pacientes eran mujeres y la media de edad fue de 38,5 años (DE 13,7). El tiempo medio desde el diagnóstico de MG hasta la intervención fue de 16 meses (límites 1 a 144 meses). La clasificación preoperatoria de Osserman fue la siguiente: estadío IIb, cuatro pacientes; estadío III, un paciente, y estadío IV, un paciente. Antes de la intervención todos los pacientes estaban tomando anticolinesterásicos (piridostigmina) y cuatro pacientes tomaban corticoides. A cinco pacientes se les practicó una timectomía transesternal extendida y al paciente restante se le efectuó una timectomía transcervical. Los hallazgos patológicos fueron los siguientes: hiperplasia tímica n cuatro pacientes y atrofia tímica en los otros dos. Tres pacientes (50 por ciento) presentaron buena respuesta (remisión o mejora), y los otros tres pacientes (50 por ciento) pobre respuesta (ningún cambio o empeoramiento).Conclusiones: Se observó una baja respuesta a la timectomía en pacientes con MG en asociación con ETC (AR y un LES) (AU)