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HTLV-I, the human T-cell lymphotropic virus type I, was first identified in rare human T-cell leukemias. Since that time, the virus has been found to be associated with tropical spastic paraparesis (TSP), a spinal cord disorder which results in progressive weakness. TSP associated with HTLV-I infection is most common in parts of the world where the virus is endemic. HTLV-I infection is common in Japan, France, parts of South America, and many Caribbean islands. Curiously, although HTLV-I infection is common on neighboring islands, HTLV-I infection has not been found to be associated with TSP in Puerto Rico - until recently. Two native men, one 58 and one 66, were found to have symptoms consistent with tropical spastic paraparesis. In both cases, antibodies to HTLV-I were found in the patient's blood, and in one case antibodies to this virus could also be confirmed in the patient's cerebrospinal fluid. One of the patients had a history of intravenous drug abuse; the other patient had a medical history which included receiving blood transfusions three years prior to the development of symptoms. These two men are believed to represent the first two cases of tropical spastic paraparesis identified in Puerto Rico. (Consumer Summary produced by Reliance Medical Information, Inc.)

The primary antiphospholipid syndrome and protein S deficiency are known hypercoagulable states predisposing to ischemic strokes. The pathogenesis of those hypercoagulable states has been independently studied and, recently, interaction between them has been proposed. A 48-year-old Hispanic man had generalized seizures 5 months after the acute onset of a left middle cerebral artery infarct. He had a strong family history of strokes. After evaluation for cardiologic, rheumatologic, hematologic and metabolic etiologies for stroke, anticardiolipin antibodies and protein S deficiency were detected. Cerebral angiography was normal. First degree relatives were evaluated and screened for these conditions. Lupus anticoagulant was detected in a sister who reported a transient hemisensory deficit. None of the relatives studied had clinical or laboratory criteria for collagen vascular diseases. Coexistence of the primary antiphospholipid syndrome and protein S deficiency, two known prothrombotic states, has rarely been reported in Hispanic adults in association with ischemic stroke. [ABSTRACT FROM AUTHOR]

Checkpoint inhibitors (CPIs) are increasingly being used in the treatment of malignant melanoma. While showing promise in metastatic melanoma treatment, CPIs are associated with immune-related adverse events in various organ systems. Among these events, checkpoint inhibitor-induced neurotoxicity stands out as a particularly rare yet diagnostically challenging and potentially life-threatening occurrence. We report a unique case of checkpoint inhibitor-induced neurotoxicity in a patient with metastatic melanoma directly after beginning treatment with checkpoint inhibitor encorafenib. The patient presented with an unclear clinical course, with features of Guillain-Barré syndrome, myasthenia gravis, and brainstem encephalitis.  We followed a recently established management algorithm for checkpoint inhibitor-induced neurotoxicity with positive outcomes. This case report highlights the importance of recognizing checkpoint inhibitor-induced neurotoxicity as a potential adverse effect of CPIs when treating metastatic melanoma. J Drugs Dermatol. 2023;22(12):e25-e27.     doi:10.36849/JDD.7991e.

Medical students need to understand core neuroscience principles as a foundation for their required clinical experiences in neurology. In fact, they need a solid neuroscience foundation for their clinical experiences in all other medical disciplines also because the nervous system plays such a critical role in the function of every organ system. Because of the rapid pace of neuroscience discoveries, it is unrealistic to expect students to master the entire field. It is also unnecessary, as students can expect to have ready access to electronic reference sources no matter where they practice. In the preclerkship phase of medical school, the focus should be on providing students with the foundational knowledge to use those resources effectively and interpret them correctly. This article describes an organizational framework for teaching the essential neuroscience background needed by all physicians. This is particularly germane at a time when many medical schools are reassessing traditional practices and instituting curricular changes such as competency-based approaches, earlier clinical immersion, and increased emphasis on active learning. This article reviews factors that should be considered when developing the preclerkship neuroscience curriculum, including goals and objectives for the curriculum, the general topics to include, teaching and assessment methodology, who should direct the course, and the areas of expertise of faculty who might be enlisted as teachers or content experts. These guidelines were developed by a work group of experienced educators appointed by the Undergraduate Education Subcommittee (UES) of the American Academy of Neurology (AAN). They were then successively reviewed, edited, and approved by the entire UES, the AAN Education Committee, and the AAN Board of Directors., (© 2021 American Academy of Neurology.)

A morbidly obese, diabetic, hypertensive patient, with severe intracranial left middle cerebral artery (MCA) stenosis, suffered recurrent stereotyped transient ischemic attacks over 2 months, despite taking daily antiplatelet agents, high dose statin, insulin and an angiotensin converting enzyme inhibitor. A left MCA (M1) Wingspan stent-assisted angioplasty was performed after standard loading, and daily doses of clopidogrel were given 3 days prior to the procedure. Immediately after the procedure, the patient developed a left hemispheric ischemic stroke syndrome. Urgent re-angiography identified an acute intracranial in-stent thrombosis. This complication was immediately treated successfully with abciximab and balloon angioplasty. The patient had persistent residual stroke despite complete recanalization of the thrombosed stent within 3 h of occlusion. The patient was subsequently found to have incomplete inhibition of platelet activity despite being on clopidogrel 150 mg and aspirin 81 mg daily and having a normal CYP-2C19 genotype, suggesting that suboptimal antiplatelet inhibition, secondary to morbid obesity, contributed to his in-stent thrombosis. Obese patients undergoing coronary artery stenting routinely receive larger loading and maintenance doses of clopidogrel. Our case suggests that obese patients undergoing intracranial stenting may also benefit from higher than conventional clopidogrel doses prior to intracranial stenting, to decrease risk of acute in-stent occlusion.

Background: Acute multiple brain infarction (AMBI) pattern on diffusion-weighted imaging (DWI) is associated with arterial and cardiac sources of embolism. The DWI characteristics of patients with stroke due to vertebrobasilar arterial dissection and atherosclerotic disease have not been reported in detail., Objective: To describe the DWI stroke patterns in patients with posterior circulation occlusive disease to determine mechanisms of ischemia., Design: Retrospective analysis of infarct patterns in patients with symptomatic vertebrobasilar disease., Setting: Large community-based teaching hospital., Patients: Patients admitted with stroke due to vertebrobasilar disease were identified retrospectively. Patients were included if DWI was obtained within 7 days of symptom onset., Main Outcome Measure: Infarct patterns were analyzed according to established templates of vascular territories., Results: Eleven patients with vertebral dissection and 39 patients with atherothrombosis were identified. An AMBI pattern was present in 8 (72%) of 11 patients with arterial dissections and 25 (64%) of 39 patients with atherosclerotic disease (P = .48). Distal embolism to the terminal branches of the basilar artery occurred with equal frequency in both groups and was found in half of all cases. Isolated thalamic infarction did not occur. Pontine infarction was noted in 2 (18%) of 11 patients with dissections and 18 (46%) of 39 patients with atherosclerosis (P = .09). Cerebellar border zone involvement was found in 14 (36%) of 39 patients with atherosclerosis and 4 (37%) of 11 patients with dissections (P = .6)., Conclusions: Large arterial disease is frequently associated with AMBI in the posterior circulation. The incidence of AMBI was comparable to that reported in the anterior circulation. This DWI study supports the importance of embolism as the main mechanism of infarction in patients with vertebrobasilar occlusive disease. On the basis of our experience, large-vessel vertebrobasilar disease rarely causes isolated small-vessel thalamic infarction.

We prospectively evaluated the inflammatory response to acute cerebral ischemia in 57 patients who were seen within 72 hours of ictus. All subjects had cerebrospinal fluid examination, complete blood count, sedimentation rate determination, and body temperature monitoring. Correlation analysis was done between these measurements and infarct volume, which was determined by computed tomography of the brain. We found a positive linear correlation between infarct size and the peripheral white blood cell count, specifically the polymorphonuclear leukocyte count. A relationship was also observed for the cerebrospinal fluid protein level, the gamma globulin level, and the cerebrospinal fluid/serum albumin ratio. The correlations observed presumably reflect the extent of tissue injury and secondary inflammatory response in acute cerebral ischemia.

Background and Purpose: We undertook this study to investigate the relationship between outcome, hematoma volume, and admission peripheral white blood cell count and body temperature in acute hypertensive intracerebral hemorrhage., Methods: Eighty-two consecutive patients who presented with hypertensive intracerebral hemorrhage within 72 hours of onset were retrospectively assessed. The peripheral white blood cell count, polymorphonuclear leukocytes, and the body temperature on admission were measured. The outcome at 30 days after ictus was determined with a modified Glasgow Outcome Scale. Correlation analysis was performed between these measurements and hematoma volume, which was calculated by brain computed tomography. We also looked at the presence or absence of intraventricular extension., Results: The mean hematoma volume was significantly greater in those patients who died compared with those with a good, moderate, and severe outcome (79.6 cm3 versus 10.7, 18.3, and 19.9 cm3, respectively; P < .0005). The mean peripheral white blood cell count was higher in those who died than in the other three groups (12.580 +/- 0.521 versus 8.160 +/- 0.543, 8.565 +/- 0.543, and 7.427 +/- 0.786 x 10(9)/L, respectively; P < .0005). The mean body temperature of those who died tended to be higher than those in the good-outcome group (99.12 +/- 0.21 versus 98.18 +/- 0.21 degrees F, P < .05). A positive linear correlation was observed between hematoma volume and white blood cell count (r = .506, df = 77, P < .001), as well as the polymorphonuclear leukocyte count (r = .561, df = 76, P < .001). A trend was also observed for admission temperature (r = .265, df = 74, P < .05). The leukocyte response was enhanced by the presence of intraventricular extension., Conclusions: There is a relationship between the size of the hematoma and the degree of leukocytosis in hypertensive intracerebral hemorrhage. This relationship appears to most likely represent a stress-induced reaction of the white blood cell count.

A patient with an acute ischemic stroke had an interatrial septal aneurysm shown by transesophageal echocardiography. Interatrial shunting, compatible with a patent foramen ovale, was observed on a follow-up study after a second stroke. This was seen in association with a right atrial thrombus. This case illustrates that an interatrial septal aneurysm serves as a marker for potential interatrial shunting, which can lead to paradoxical cerebral embolism.

Transcranial Doppler ultrasonography of the middle cerebral arteries was performed during two tasks: sorting of mah-jongg tiles and vibratory stimulation. These tasks selectively increase cerebral blood flow by positron emission tomography. The purpose of this study was to determine if analogous increases in cerebral blood flow velocity could be detected. We measured flow velocity during right hand manipulation followed by left hand manipulation, or vice versa, with resting studies in between. The average increase in the mean velocity, by paired t-test, was significant for the right middle cerebral artery with both left hand (p < .0005) and right hand (p < .005) tile sorting. For the left middle cerebral artery, there was an increase in the mean velocity with right hand (p < .005) but not for left hand sorting (p = .13). These findings support the importance of the right hemisphere in the performance of this type of spatial task. No significant flow velocity increase occurred during vibratory stimulation.

A patient with hemiballism-hemichorea as the initial manifestation of the acquired immunodeficiency syndrome is described. Movement disorders in AIDS have not been frequently reported. Possible etiologies and therapeutic trials are presented. To our knowledge this is the first case report of hemiballism-hemichorea as the initial presenting feature of an AIDS patient in Puerto Rico.

The development of neurologic deficits confined to the corticospinal tracts has been referred as Primary Lateral Sclerosis (PLS). Through the years, this diagnosis has remained uncertain. In this study we describe seven patients with chronic involvement of the pyramidal system. Two of these patients had serologic evidence of human T-lymphotrophic virus type I infection, one patient had multiple sclerosis and in four patients the clinical diagnosis of PLS was made. The clinical characteristics and diagnostic studies of these patients are presented. A review of the literature with emphasis in the differential diagnosis and a proposed workup for patients with chronic spastic paraparesis are made. This study provides supporting evidence in favor of the clinical entity of PLS.