The severity of neurodegeneration and minor differences in the ultrastructure of storage material implied that this was a different disease from other forms of ovine ceroid-lipofuscinosis that accumulate subunit-c of mitochondrial ATP synthase. An autosomal recessive mode of inheritance is considered probable. Although of only minor economic importance, this disease may be important to research into the group of ceroid-lipofuscinoses as a whole.

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A detailed investigation of the neuropathology of a horse affected with stringhalt was performed. Qualitative and quantitative light and electron microscopy, and single teased fibre preparations of peripheral nerve demonstrated predominantly axonal degeneration, the stage of which was appropriate for the duration of clinical signs. There was selective involvement of large myelinated nerve fibres. A proximal to distal increase in the severity of pathological changes was present in the peripheral nerves. The long left recurrent laryngeal nerve was the most severely affected, followed in order by its right counterpart, the hindlimb and forelimb nerves. Neurogenic atrophy of muscles innervated by affected peripheral nerves also showed a distally graded increase in severity. No lesions were observed in the central nervous system. It was concluded that this disease should be classified as a distal axonopathy.

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Ru a kura A ni ma l He a lth La bora tory . Ham ilton a nd Fac ulty o f Vet er inary Scie nce . Massey U nive rs ity .Pa lmc rsto n o rth , New Zeala nd A bstract. A na t ura lly occ urri ng neu ro logical dise ase occ urre d in six dogs fed coo ke d m ea t. Cli nica l sig ns we re ano rex ia . progre ssive s pas tic parap a re sis . recumbe ncy . co nv ulsio ns a nd dea th . T he d iseas e wa s cha racte rize d by b ila te ra lly sy m me trica l spo ngy c ha nge and ne crosis of hrui nstc m n ucle i with a lesion d istri b utio n pa tt e rn sim ila r to th a t in th ia min e def icie nt foxes a nd ca ts . A n assoc ia te d th ia m ine defic ien cy was ev ide nce d by decrea sed th iamin e le vel s in t he blood of o ne d og a nd in th e fo od o f a not her . a nd rapi d rem ission of clinica l sig ns in a dog given t hia m ine hyd ro chl orid e . T he rm a l destructio n of t hiam ine th ro ugh coo king o f th e food s proba b ly ca use d the d iet a ry def icie ncy .Spo nta ne o us le sion s o f poli o cn ccph al om al a cia ha ve been d escrib ed in d ogs with nervo us d isorders of unkn o wn ca use 115 1. cy a n ide 11 5 . 16] a nd le a d 14 4 ] po iso n ing and ca rd iac a rr e st e nc e p ha lo pa t hy 130) . T he les ion s o f th e poli ocn ccph al orn al aci a o f un kn o wn ca use we re co n fine d to t he ce re b ra l co rtex . wh ere as t he lesion s ca use d by cya n ide po iso ni ng a nd ca rd iac a rrest a lso invo lved the b ra inst e m . T he le sio ns ca use d by le ad po iso n ing co m mo n ly in vol ved th e ce re b ra l co rte x 144] .T his pa per describ es a ca n ine e nce p ha lo pa t hy c ha racte rize d by po liom a la cia o f th e b ra instc rn wit h a le sio n di stributi on pa tt ern s im ila r to th at o f th iamin e defi cient foxe s IH] a nd ca ts 12 0 ]. Case Histories and Clinical FindingsThe dogs were cross bre d mal e a nd fe ma le Borde r Co llies') mo nt hs to H yr o ld ( tab le I). Tw o dogs (d ogs 2 a nd 3 ) we re co n fine d in a la borat o ry co lo ny a nd fo ur we re fro m se pa ra te fa rms a nd had been used fo r he rding she e p a nd ca tt le .All dogs ha d neu rol ogica l signs a nd the patt e rn and progression o f th e sig ns wer e fai rly un ifo rm (ta b le I). Five dogs had a n in itia l a nd pe rsist en t a no re xia . Fo ur o f t hese deve lop e d spas tic para pa re sis a bo ut 2 days a fte r o nse t o f a no rex ia . Dogs with spas tic par a pa re sis he ld the ir hind legs stiff a nd st ra ig ht a nd stood a nd walk ed witb a wide base: foreleg . ne ck a nd he ad move me nt s we re norm a l. T he se ve rity of the para par e sis p ro g ressed and th e d ogs be ca m e unable to sta nd: dog 6 wa s ki lled w he n s he co uld ba rel y sta nd . Dogs 2 a nd.' had cro uc hing po stu res a nd ga its wit ho ut spas ticity : th ey becam e rec um be nt. D ur ing recu mbe ncy . five dogs had clo n ic a nd interm itte nt to nic co nv ulsio ns . T hr ee dogs beca me co ma tose a nd d ied . Dogs di ed or were ki lle d .' -7 days af te r ano rex ia was first not iced . Infr...