Hoarseness in pellagra

A 67-year-old woman with chronic alcoholism was referred to our hospital with a 3-month history of progressive weakness in all four extremities and a 5-day history of hoarseness. On admission, she presented with glossitis and laryngitis. Neurological examination revealed weakness in all extremities and nerve conduction studies showed polyneuropathy. She was diagnosed with thiamine deficiency and administered intravenous multivitamins, including thiamine, pyridoxine and mecobalamin. Although the weakness in her extremities was ameliorated after treatment, her glossitis and laryngitis did not improve and psychosis subsequently occurred. After administration of nicotinamide, her psychosis and laryngitis dramatically improved. She was diagnosed with pellagra after detection of decreased serum levels of niacin and thiamine. Weakness may have been due to thiamine-deficient neuropathy or multiple vitamin deficiencies, as it improved with thiamine, pyridoxine and mecobalamin therapy. Because of lack of response to thiamine therapy, we concluded that the psychosis and laryngitis were caused by pellagra. Although stomatitis and glossitis in pellagra has been well documented, laryngitis and hoarseness appear to be very uncommon conditions. Differences in the turnover rates or energy requirements between the gastrointestinal tract and larynges may be a reason for the rarity of laryngeal involvement in pellagra. We should be vigilant to hoarseness, caused by laryngitis, which can be the predominant symptom in pellagra.     

1例Hartnup病临床特点及诊疗分析

1病例介绍 患者,男性,19岁,因“发作性跌倒5年,间断双下肢无力麻木伴皮疹4年”入院。患者5年前跑步时突然跌倒,约2min后自行站起,无意识障碍、肢体抽搐。4年半前再次突然跌倒,症状同前。后逐渐出现发作性行走不稳,每次约持续10min。     

Mitochondrial myopathy,encephalopathy, lactic acidosis,and stroke-like episodes with acanthocytosis: a clinicopathological study of a unique case

Summary A case of a unique combination of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like syndrome (MELAS) with acanthocytosis is reported. Neuropathological examination revealed pellagra-like change in Betz cells, brain-stem neurons and anterior horn cells as well as findings compatible with mitochondrial encephalomyopathies. Abnormal function of nicotinic acid-related enzymes could be the cause of the complicated clinicopathologic findings in this case. This is the first report of MELAS with acanthocytosis.     

Ultrastructure of 6-aminonicotinamide (6-AN) —Induced lesions in the central nervous system of rats

Summary Ultrastructure of neuronal chromatolysis and other lesions in the gray matter of the cervical cord due to 6-aminonicotinamide (6-AN) administration, an antinicotinamide, to rats, were followed up during a 35-days period. Neuronal chromatolysis which was prominent in the anterior horn cells in the acute stage, was completely recovered from via the temprary hyperchromasia of their cytoplasm. Their axons, however, which form the anterior nerve roots, did not show any particular changes throughout the whole period of the experiment. This evidence suggests that neuronal chromatolysis induced by 6-AN might not be the result of axonal damage, but was due to the direct action of 6-AN on the soma of the anterior horn cells.In addition, necrosis of the internuncial cells, various reactions of glial and mesenchymal elements and the spongy state of the neuropil in the laminae VI and VII of Rexed of the cervical gray matter were observed in the acute stage. They were later repaired by glia. These lesions are usually absent in the central nervous system of human cases with pellagra.It is considered that the findings with 6-AN simulate the central nervous lesions of pellagra in human, but some minor differences in pathology such as presence of severer lesions in glia and other elements in the experimental rats would probably be caused by additional factors such as drastic mode of action, probable difference in catabolism of nicotinamide and 6-AN, and so forth.     

Ultrastructure of 6-aminonicotinamide (6-AN)-induced lesions in the central nervous system of rats

Summary Lesions in the CNS induced by 6-aminonicotinamide (6-AN) presented a spongy state of the gray matter and neuronal chromatolysis. With aging of the experimental animals the lesions extended from the phylogenetically early developed structures to those developed later, i.e., from spinal gray matter, dentate nuclei, and brain stem nuclei through limbic structures and striatum to the cerebral cortex. Changes of the neurons were more prominent with aging.Lesions in the CNS of rats at the age, corresponding to the involutional period in the human, were similar to those of Creutzfeldt-Jakob disease (C-J disease) in the presenile age. In recent years, the resemblance between C-J disease and pellagra encephalopathy had been noted by several authors, and they resemble the lesions caused by 6-AN, an antimetabolite of nicotinamide used in our experiment. This evidence, therefore, has led to the hypothesis that dysfunction of NAD(H)- or NADP(H)-dependent enzymes in the CNS of the aged, even if not the primary cause, may be one possible pathogenetic factor of C-J disease.     

Commentary: Predicting aggression in youth

Aggression in youth is common and encompasses a wide range of behaviors that vary along such dimensions as direction (toward self or others), degree of action (ideation, verbal threats, or actual assaults), degree of planning (reactive or impulsive, planned or predatory), system level (biological, psychological, or social), and seriousness of intent. Risk factors vary, depending on the range of behavior being predicted and the developmental level of the youth being assessed. The Brief Rating of Aggression by Children and Adolescents (BRACHA) shows promise for assessing the general risk of aggressiveness in a developmentally diverse child and adolescent inpatient population. Further research may show whether patterns of risk factors also have treatment and prognostic implications that will aid in working with this population.     

Commentary: freedom and function

While the question of whether our actions are determined or are the result of free will is a deep one in philosophy, it does not need to be answered for forensic psychiatrists to give evidence in court. As Stephen Morse has pointed out, the absence of free will is not named as an excusing condition. The insanity defense, for instance, requires proof of functional impairment, to which psychiatrists can usefully testify. Of the approaches available to determinism, my own preference is that of Herbert Hart: until we know that determinism is true, we will continue to prefer a system that requires persons to have made proper choices to act as they did before we hold them responsible. This seems to resemble Dr. Felthous' preferred option, that mentally responsible choices are choices made in the presence of a relatively natural ability to have decided otherwise.     

Commentary: Stalking risk profile

Paul Mullen and his colleagues have developed a Stalking Risk Profile that is the latest model for assessing and managing stalkers. The model includes an assessment of the nature of the relationship between the stalker and his victim; the stalker's motivations; the general psychological, psychopathological, and social realities of the stalker; the circumstances of the victim, and the legal and mental health context. The model is not an actuarial scale, but rather an assessment to be used on a case-by-case basis. It recommends certain standardized tests as part of the assessment. The Stalker Risk Profile is the most thorough risk assessment to date. It includes victim variables and provides specifics for assessment and specifics for treatment of the stalker.