SUBACUTE COMBINED DEGENERATION OF UNKNOWN ORIGIN WITH EXTENSIVE INVOLVEMENT OF THE BRAIN

1943 ◽  
Vol 98 (2) ◽  
pp. 216
Author(s):  
R. E. Hemphill ◽  
E. Stengel
1941 ◽  
Vol 87 (366) ◽  
pp. 77-87 ◽  
Author(s):  
R. E. Hemphill ◽  
E. Stengel

The aetiology and pathology, as well as the clinical characteristics of those diseases of the nervous system in which the myelin sheaths and the axis cylinders are unsystematically affected, but the other elements of the nervous tissue spared, are as yet incompletely understood. Of these diseases, the group in which the changes of the nervous system are associated with anaemia and other dyscrasias of the haemopoietic system, generally classified as subacute combined degeneration of the spinal cord, is the most clearly defined. However, even in this group cases occur in which the characteristic degeneration of the spinal cord is not accompanied by gross pathological changes in the blood. There is still, therefore, much research required before these atypical cases can be brought into line with the general pathological conceptions of the subacute combined degeneration. Furthermore, the existence and character of pathological changes in the brain and other parts of the nervous system of cases with subacute combined degeneration, apart from the spinal cord, remain to be investigated more fully. The case which we have to report has various special features, the study of which should contribute in some degree to the elucidation of these complex problems. In this case, which presented the typical picture of a subacute combined degeneration without blood changes, there was an extensive affection of the brain and the peripheral nervous system.


2019 ◽  
Vol 5 (1) ◽  
pp. 205511691983021
Author(s):  
Dylan M Djani ◽  
William E Draper

Case summary A 3-year-old spayed female domestic shorthair cat developed a fever 1 week after starting the anticonvulsant phenobarbital. A diagnostic work-up for seizures and subsequent onset of fever of unknown origin, consisting of MRI of the brain, cerebrospinal fluid analysis and infectious disease testing, was unremarkable. The cat was switched from phenobarbital onto pregabalin with complete resolution of the fever within 24 h, consistent with a drug-induced fever following phenobarbital administration. Relevance and novel information While anticonvulsant hypersensitivities have been reported and studied in veterinary medicine, phenobarbital-induced fever outside of the context of systemic clinical signs has not been documented in the veterinary scientific literature. Drug-induced fever secondary to anticonvulsants should be considered in patients that develop a fever after starting anticonvulsant therapy with an unrewarding diagnostic work-up for fever of unknown origin.


Author(s):  
V.A. Kral

Abstract:The close cooperation of clinical and laboratory research has helped to clarify the etiology of some of the dementing processes of the senium. However, the necessary investigations are complicated, laborious, expensive and can be carried out only in well equipped centres in larger cities. This restricts the number of patients who eventually may benefit from these investigations to a small number. What is needed for the psychogeriatric practice particularly in rural areas and smaller cities are simple diagnostic guidelines for the psychiatrist to answer the question whether the patient suffers from a dementia and if so whether the dementia is in all probability due to a primary degenerative process of the brain parenchyma or of the cerebral vasculature or is it due to another cause.If degeneration of the brain parenchyma seems the prevalent pathogenetic mechanism one would like to establish in a given case which of the known degenerative processes is most probably present in order to avoid mistakes in clinical judgement with their often life threatening consequences.


2012 ◽  
Vol 9 (4) ◽  
pp. 353-360 ◽  
Author(s):  
Edward R. Smith ◽  
R. Michael Scott

Moyamoya is a progressive arteriopathy of unknown origin affecting the branches of the internal carotid artery (ICA). The arteriopathy can present as an isolated medical condition, affecting both sides of the brain (“moyamoya disease”) or can be unilateral or found in association with systemic disorders (“moyamoya syndrome”). The ischemia resulting from luminal narrowing predisposes children to transient ischemic attacks and stroke—the primary presentations of affected patients. Although it is rare—affecting 1 in 1 million children in the US—moyamoya is implicated in 6% of all childhood strokes. Diagnosis is defined by characteristic findings on arteriograms, including stenosis of the branches of the ICA and a pathognomonic spray of small collateral vessels in this region, descriptively likened to a “puff of smoke” (“moyamoya” in Japanese). Treatment is predicated on restoration of cerebral blood flow by surgical revascularization. The rarity of this disorder has limited research and the development of evidence-based clinical management. While acknowledging these limitations, in this article the authors aim to summarize current studies of pediatric moyamoya, with the objective of providing a framework for construction of evidence-based guidelines for treatment. The compilation of current data in these guidelines should serve as a resource to aid pediatric neurosurgeons in their role as advocates for providing appropriate care to affected children.


2021 ◽  
Vol 90 (1) ◽  
pp. 37-43
Author(s):  
H. Haardt ◽  
K. Vanderperren ◽  
C. Bauwens ◽  
L. Lefère ◽  
E. Raes ◽  
...  

A fourteen-day-old warmblood colt presented with severe neurological signs of unknown origin. The foal was recumbent and comatose, and showed spastic motion of the head. Computed tomography (CT) examination revealed multiple impression fractures to the left parietal and temporal bones, as well as traumatic brain injury. Three fragments of the temporal bone were dislocated into the cranial vault. The foal was surgically treated within 24 hours of presentation: the fragments were elevated and the largest fragment was fixated with an L-shaped titanium MatrixMidface 0.8 mm reconstruction plate. The brain injury resolved with medical treatment and supportive care. Apart from a localized wound abscess that was drained, postoperative healing was uneventful and the foal regained the ability to ambulate 18 days after surgery. Follow-up CT imaging documented good fracture healing and a favorable development of the soft tissue injuries. Seven months after surgery, the foal was fully recovered and showed no neurological signs.


2017 ◽  
Vol 30 (6) ◽  
pp. 578-582 ◽  
Author(s):  
Shaista Siddiqui ◽  
Shazia Naaz ◽  
Mehtab Ahmad ◽  
Zafar Ahmad Khan ◽  
Shagufta Wahab ◽  
...  

Encephalocraniocutaneous lipomatosis (ECCL) or Haberland syndrome is an uncommon sporadic neurocutaneous syndrome of unknown origin. The rarity and common ignorance of the condition often makes diagnosis difficult. The hallmark of this syndrome is the triad of skin, ocular and central nervous system (CNS) involvement and includes a long list of combination of conditions. Herein we report a case of a 5-month-old male child who presented to our centre with complaint of seizure. The patient had various cutaneous and ocular stigmatas of the disease in the form of patchy alopecia of the scalp, right-sided limbal dermoid and a nodular skin tag near the lateral canthus of the right eye. MRI of the brain was conducted which revealed intracranial lipoma and arachnoid cyst. The constellation of signs and symptoms along with the skin, ocular and CNS findings led to the diagnosis of ECCL.


2019 ◽  
Vol 7 (2) ◽  
pp. e000759
Author(s):  
Ian Keith Hawkins ◽  
Daniel R Rissi ◽  
Anibal G Armién ◽  
Rebecca Penrose Wilkes ◽  
Eman Anis

A four-year-old intact female pointer dog from a hunting plantation in South Georgia, USA, developed acute neurological signs. The referring veterinarian suspected rabies or pseudorabies; the dog was euthanased and submitted to the Tifton Veterinary Diagnostic and Investigational Laboratory for necropsy. No gross anatomical changes were present. Portions of brain were submitted for rabies virus fluorescent antibody test with a negative result. Histopathology revealed a marked lymphoplasmacytic meningoencephalitis with numerous intracytoplasmic eosinophilic inclusions within neurons, still raising concerns for rabies meningoencephalitis. Rabies immunohistochemistry was then performed on the brain, yielding a negative result. Brain samples were also negative for canine distemper virus and herpesvirus (pan-herpesvirus primers) PCR. Electron microscopy did not reveal viral particles within the inclusions. Similar intraneuronal pseudoinclusions have been previously reported in non-rabid dogs and cats. Such inclusions are a diagnostic challenge, especially in animals with central neurological signs and/or meningoencephalitis.


1999 ◽  
Vol 29 (6) ◽  
pp. 441-443 ◽  
Author(s):  
E. Balaci ◽  
Thomas E. Sumner ◽  
S. T. Auringer ◽  
T. David Cox

Author(s):  
Marta Sophie S Stęga ◽  
Wojciech Choiński ◽  
Michał Puliński

ntroduction Anisocoria is a condition characterized by unequal pupils. There are two described types: physiologic and pathologic. Pathologic anisocoria is caused by interruption in reflex arc. Reason of anisocoria can be difficult to define due to variety of possible diseases that may be causing it. Aim Case presentation of anisocoria caused by congenital cystic lesions of the brain. Case Study 12-year-old girl after head trauma was admitted to the Emergency Department due to anisocoria noticed by family doctor. Patient had headache, sleepiness, dizziness and lacrimation. After looking at patients’ childhood photo it was stated, that anisocoria was present before the injury. In physical examination decreased mobility of left eyeball, left-sided ptosis and left-sided decreased reaction to the light (both, direct and consensual) were noted. Head CT scan was performed, which revealed bilateral cystic lesions in basal ganglia, thalamus and in mesencephalon. The biggest lesions were located in left thalamus – 29x15mm. Biopsy of the biggest cyst was performed. No neoplastic tissue was found. Final diagnosis was stated: diencephalic and mesencephalic polycyclic lesions of unknown origin. Surgical approach was departed and conservative treatment was applied (regular head MRI scans) Results and discussion Described case report was an example of diagnostic difficulties which can be caused by anisocoria. In this case pathologic anisocoria was caused by congenital cystic lesions of the brain that were detected in the age of 12. Conclusions Anisocoria can be a sign of many different pathologies, but also can be physiologic. If pathologic anisocoria is suspected, imaging diagnostics should always be performed.


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