Long-term Sequelae of Pediatric Bickerstaff Brainstem Encephalitis Includes Autonomic and Sleep Dysregulation

2019 ◽  
Vol 34 (3) ◽  
pp. 153-160
Author(s):  
Jonathan D. Santoro

Bickerstaff brainstem encephalitis is a rare, immune-mediated disorder of the brainstem and peripheral nervous system. Published knowledge of pediatric Bickerstaff brainstem encephalitis focuses on the acute phase of the disease process. This study evaluated long-term neurologic and immune sequelae of Bickerstaff brainstem encephalitis in children. A single-center retrospective chart review was performed. Clinical data, neuroimaging, polysomnograms, and serum data were reviewed. Five patients were included in this study. Four patients had no neurologic residua, and 1 patient continued to have mild bulbar dysfunction. There was neither recurrence of symptoms nor development of other neurologic or immunologic disorders at a median of 3 years after diagnosis. Review of systems was largely negative, although 2 patients endorsed symptoms consistent with mild orthostatic hypotension for 1 year after diagnosis, but these findings were not sustained. Four of 5 patients endorsed sleep dysregulation. Three patients met criteria for pediatric obstructive sleep apnea. Prognosis following pediatric Bickerstaff brainstem encephalitis is excellent although posttreatment autonomic and sleep dysregulation may reflect residua from acute phase inflammation in the peripheral nervous system and connections of the reticular activating formation of the brainstem, although this was time limited. Further prospective, multicenter, analysis is warranted.

2021 ◽  
Vol 3 (1) ◽  
Author(s):  
Max Borsche ◽  
Dirk Reichel ◽  
Anja Fellbrich ◽  
Anne S. Lixenfeld ◽  
Johann Rahmöller ◽  
...  

AbstractNeurological long-term sequelae are increasingly considered an important challenge in the recent COVID-19 pandemic. However, most evidence for neurological symptoms after SARS-CoV-2 infection and central nervous system invasion of the virus stems from individuals severely affected in the acute phase of the disease. Here, we report long-lasting cognitive impairment along with persistent cerebrospinal fluid anti-SARS-CoV-2 antibodies in a female patient with unremarkable standard examination 6 months after mild COVID-19, supporting the implementation of neuropsychological testing and specific cerebrospinal fluid investigation also in patients with a relatively mild acute disease phase.


2011 ◽  
Vol 27 (2) ◽  
pp. 229-233
Author(s):  
Katrin Sabine Blum ◽  
Christian Hagel ◽  
Eva Neuen-Jacob ◽  
Peter Herkenrath ◽  
Jens Fiehler ◽  
...  

2004 ◽  
Vol 77 (5) ◽  
pp. 662-669 ◽  
Author(s):  
Ravi J. Tolwani ◽  
Jos� M. Cosgaya ◽  
Sushama Varma ◽  
Reza Jacob ◽  
Lydia E. Kuo ◽  
...  

Author(s):  
O. V. Demydas

Objective — to analyze clinical neurological manifestations in patients suffering from peptic ulcer disease in the acute phase and in remission, based on the findings of a comprehensive clinical neurological, neuropsychological and paraclinical study. Methods and subjects.  84 patients suffering from PUD were comprehensively examined while in the acute phase of the disease and then all 84 were reexamined while in remission. The age range of the patients was from 25 to 60 years. The average age of the patient was 39.90 ± 1.29 years. The examined individuals were destributed into two groups based on whether they presented symptoms of an acute phase or remission of the peptic ulcer disease. The comprehensive examination included: interview and complaint analysis, neurological examination focused on the state of the autonomic nervous system, study of the neuropsychological differences (the trait and state anxiety levels monitoring based on the Spielberg‑Khanin scale, depressive state evaluation using Beck Depression Inventory, self‑perceived health assessment, mood and activity monitoring using the SAN questionnaire, cognitive impairment evaluation using the MMSE scale, assessment of the refocusing speeds and performance distribution using Schulte tables), as well as lab tests and procedures. Results. Most often, subjects complained of headache (74 (88.0 %) in the acute stage and 37 (44.0 %) in remission). The second most common was a complaint of pain in the thoracic spine (69 (82.1 %) and 35 (41.6 %), respectively). Complaints of dizziness, pain in the heart, palpitations, «interruptions» in the heart, paresthesia were often recorded. In 11 (12.4 %) patients with duodenal ulcer in the acute stage experienced episodes of syncopal state, while in the remission stage they were absent. Complaints that indicated the presence of psychoemotional disorders were anxiety, decreased memory and attention, and sleep disturbances. In patients with duodenal ulcer disease we revealed lesions of the central and peripheral nervous system. Central nervous system disorders were manifested in the form of vestibulo‑cerebellar syndrome (in 30 (35.7 %) patients in the acute stage and in 14 (16.6 %) in the remission stage), extrapyramidal disorders (respectively in 10 (11.9 %) and 4 (4.76 %)) and signs of pyramidal dysfunction (37 (44.0 %) and 15 (17.8 %)). Clinical and neurological examination of the peripheral nervous system in 68 (80.9 %) patients with peptic ulcer in the acute stage and in 31 (36.9 %) in the remission stage revealed polyneuropathy syndrome of varying degrees. Signs of polyneuropathy were accompanied by complaints of disorders of the peripheral nervous system (45 (53.5 %) and 15 (17.8 %) cases, respectively). In 27.4 % of patients with peptic ulcer disease in the acute stage of the complaint were absent at all, and only a thorough neurological examination revealed signs of polyneuropathy. In 56 (66.6 %) patients with peptic ulcer disease in the acute stage and 28 (33.3 %) in the remission stage, the morbidity of paravertebral points in the lower thoracic spine was revealed. Conclusions. Having analyzed the data obtained through the interviews, as well as the neurological characteristics of patients with peptic ulcer disease of the duodenum in the acute phase and in remission it was concluded that most of the somatic complaints and neurological manifestations were common in both the acute phase and the remission of the disease. However, all of the identified neurological differences were significantly more common in the acute phase of the disease.  


2006 ◽  
Vol 110 (2) ◽  
pp. 175-191 ◽  
Author(s):  
Shelley J. Allen ◽  
David Dawbarn

The neurotrophins are growth factors required by discrete neuronal cell types for survival and maintenance, with a broad range of activities in the central and peripheral nervous system in the developing and adult mammal. This review examines their role in diverse disease states, including Alzheimer's disease, depression, pain and asthma. In addition, the role of BDNF (brain-derived neurotrophic factor) in synaptic plasticity and memory formation is discussed. Unlike the other neurotrophins, BDNF is secreted in an activity-dependent manner that allows the highly controlled release required for synaptic regulation. Evidence is discussed which shows that sequestration of NGF (nerve growth factor) is able to reverse symptoms of inflammatory pain and asthma in animal models. Both pain and asthma show an underlying pathophysiology linked to increases in endogenous NGF and subsequent NGF-dependent increase in BDNF. Conversely, in Alzheimer's disease, there is a role for NGF in the treatment of the disease and a recent clinical trial has shown benefit from its exogenous application. In addition, reductions in BDNF, and changes in the processing and usage of NGF, are evident and it is possible that both NGF and BDNF play a part in the aetiology of the disease process. This highly selective choice of functions and disease states related to neurotrophin function, although in no way comprehensive, illustrates the importance of the neurotrophins in the brain, the peripheral nervous system and in non-neuronal tissues. Ways in which the neurotrophins, their receptors or agonists/antagonists may act therapeutically are discussed.


Diabetologia ◽  
1970 ◽  
Vol 6 (2) ◽  
pp. 98-103 ◽  
Author(s):  
Edith Reske-Nielsen ◽  
Knud Lundbaek ◽  
Gunnar Gregersen ◽  
Aage Harmsen

Author(s):  
Tim Godel ◽  
Philipp Bäumer ◽  
Said Farschtschi ◽  
Klaus Püschel ◽  
Barbara Hofstadler ◽  
...  

Abstract Purpose To examine long-term alterations of the dorsal root ganglia (DRG) and the peripheral nerve in patients with neurofibromatosis type 2 (NF2) by in vivo high-resolution magnetic resonance neurography (MRN) and their correlation to histology. Methods In this prospective study the lumbosacral DRG, the right sciatic, tibial, and peroneal nerves were examined in 6 patients diagnosed with NF2 and associated polyneuropathy (PNP) by a standardized MRN protocol at 3 T. Volumes of DRG L3–S2 as well as peripheral nerve lesions were assessed and compared to follow-up examinations after 14–100 months. In one patient, imaging findings were further correlated to histology. Results Follow-up MRN examination showed a non-significant increase of volume for the DRG L3: +0.41% (p = 0.10), L4: +22.41% (p = 0.23), L5: +3.38% (p = 0.09), S1: +10.63% (p = 0.05) and S2: +1.17% (p = 0.57). Likewise, peripheral nerve lesions were not significantly increased regarding size (2.18 mm2 vs. 2.15 mm2, p = 0.89) and number (9.00 vs. 9.33, p = 0.36). Histological analyses identified schwannomas as the major correlate of both DRG hyperplasia and peripheral nerve lesions. For peripheral nerve microlesions additionally clusters of onion-bulb formations were identified. Conclusion Peripheral nervous system alterations seem to be constant or show only a minor increase in adult NF2. Thus, symptoms of PNP may not primarily attributed to the initial schwannoma growth but to secondary long-term processes, with symptoms only occurring if a certain threshold is exceeded. Histology identified grouped areas of Schwann cell proliferations as the correlate of DRG hyperplasia, while for peripheral nerve lesions different patterns could be found.


1993 ◽  
Vol 15 (1) ◽  
pp. 23-27 ◽  
Author(s):  
T. J. Postma ◽  
B. A. Benard ◽  
P. C. Huijgens ◽  
G. J. Ossenkoppele ◽  
J. J. Heimans

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